800:. If ADAMTS13 is ≥20 IU/dL, consider to stop caplacizumab and seek other diagnoses. If ADAMTS13 activity is borderline, use clinical judgment; 2) for low or intermediate pretest probability, still consider TPE and corticosteroids, but withhold caplacizumab until plasma ADAMTS13 test results are available; if ADAMTS13 activity is <10 IU/dL, consider adding caplacizumab and rituximab; if ADAMTS13 activity is ≥20 IU/dL, no caplacizumab should be used and other diagnoses should be sought; if ADAMTS13 activity falls borderline, consider other diagnoses.
520:
539:(also spelled Upshaw–Schülman). People with this inherited ADAMTS13 deficiency have a surprisingly mild phenotype, but develop TTP in clinical situations with increased von Willebrand factor levels, e.g. infection. Reportedly, less than 5% of all TTP cases are due to Upshaw–Schulman syndrome. People with this syndrome generally have 5–10% of normal ADAMTS-13 activity.
44:
907:); the procedure must be repeated daily to eliminate the inhibitor and abate the symptoms. If apheresis is not available, fresh frozen plasma can be infused, but the volume that can be given safely is limited due to the danger of fluid overload. Plasma infusion alone is not as beneficial as plasma exchange.
1091:
microvascular thrombi. In 1966, a review of 16 new cases and 255 previously reported cases led to the formulation of the classical pentad of symptoms and findings (i.e., thrombocytopenia, microangiopathic hemolytic anemia, neurological symptoms, kidney failure, fever); in this series, mortality rates
373:
As TTP progresses, blood clots form within small blood vessels (microvasculature), and platelets (clotting cells) are consumed. As a result, bruising, and rarely bleeding can occur. The bruising often takes the form of purpura, while the most common site of bleeding, if it occurs, is from the nose or
3726:
Scully M, Antun A, Cataland SR, Coppo P, Dossier C, Biebuyck N, Hassenpflug WA, Kentouche K, Knöbl P, Kremer
Hovinga JA, López-Fernández MF, Matsumoto M, Ortel TL, Windyga J, Bhattacharya I, Cronin M, Li H, Mellgård B, Patel M, Patwari P, Xiao S, Zhang P, Wang LT (May 2024). "Recombinant ADAMTS13 in
542:
A 2024 study suggested that hereditary TTP is underdiagnosed and should be considered in cases of unexplained stroke, neonatal jaundice, and severe pre-eclampsia. The study estimated the global prevalence of hereditary TTP at 40 per million, in contrast to previously reported estimates of 0.5 to 2.0
672:
The mechanism of secondary TTP is poorly understood, as ADAMTS13 activity is generally not as depressed as in idiopathic TTP, and inhibitors cannot be detected. Probable etiology may involve, at least in some cases, endothelial damage, although the formation of thrombi resulting in vessel occlusion
768:
abnormalities. Notably, the complete classic pentad of TTP symptoms—microangiopathic hemolytic anemia, thrombocytopenia, renal abnormalities, fever, and neurologic abnormalities—is only seen in about 10% of acute cases at initial presentation. Clinical suspicion of TTP is often established with an
1095:
While a response to blood transfusion had been noted before, a 1978 report and subsequent studies showed blood plasma was highly effective in improving the disease process. In 1991, plasma exchange was reported to provide better response rates compared to plasma infusion. In 1982, the disease had
2582:
Bendapudi PK, Hurwitz S, Fry A, Marques MB, Waldo SW, Li A, Sun L, Upadhyay V, Hamdan A, Brunner AM, Gansner JM, Viswanathan S, Kaufman RM, Uhl L, Stowell CP, Dzik WH, Makar RS (April 2017). "Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic
1003:
receive prophylactic plasma every two to three weeks; this maintains adequate levels of functioning ADAMTS13. Some tolerate longer intervals between plasma infusions. Additional plasma infusions may be necessary for triggering events, such as surgery; alternatively, the platelet count may be
3917:
Martino, Suella; Jamme, Mathieu; Deligny, Christophe; Busson, Marc; Loiseau, Pascale; Azoulay, Elie; Galicier, Lionel; Pène, Frédéric; Provôt, François; Dossier, Antoine; Saheb, Samir; Veyradier, Agnès; Coppo, Paul; Microangiopathies, French
Reference Center for Thrombotic (2016-07-06).
2653:
Coppo P, Schwarzinger M, Buffet M, Wynckel A, Clabault K, Presne C, Poullin P, Malot S, Vanhille P, Azoulay E, Galicier L, Lemiale V, Mira JP, Ridel C, Rondeau E, Pourrat J, Girault S, Bordessoule D, Saheb S, Ramakers M, Hamidou M, Vernant JP, Guidet B, Wolf M, Veyradier A (April 2010).
791:
guidelines suggested diagnostic and early management strategy. Suggestions included 1) for high pretest probability based on PLASMIC score or French score, start TPE and corticosteroids, and collect plasma samples for ADAMTS13 testing before therapy. Consider
507:. Very large vWF multimers are more prone to lead to coagulation. Hence, without proper cleavage of vWF by ADAMTS13, coagulation occurs at a higher rate, especially in the microvasculature, part of the blood vessel system where vWF is most active due to high
709:. As platelets are used up in the formation of thrombi, this then leads to a decrease in the number of overall circulating platelets, which may then cause life-threatening bleeds. Red blood cells passing the microscopic clots are subjected to
4330:
4310:
4447:
2089:
Tsai HM (2023). "Thrombotic
Thrombocytopenic Purpura, Hemolytic-Uremic Syndrome, and Related Disorders". In Means RT Jr, Arber DA, Glader BE, Appelbaum FR, Rodgers GM, Dispenzieri A, Fehniger TA, Michaelis LC, Leonard JP (eds.).
935:, may be used on diagnosis; this is thought to kill the B cells and thereby reduce the production of the inhibitor. A stronger recommendation for rituximab exists where TTP does not respond to corticosteroids and plasmapheresis.
972:
outside of pregnancy, but prophylactic TPE during pregnancy. For asymptomatic congenital TTP, offer prophylactic plasma infusion during pregnancy, and consider plasma infusion or a wait and watch approach outside of pregnancy.
673:
may not be essential in the pathogenesis of secondary TTP. These factors may also be considered a form of secondary aHUS; people presenting with these features are, therefore, potential candidates for anticomplement therapy.
783:
A definitive diagnosis of TTP may be established when a laboratory assay of ADAMTS13 identifies under 10% of normal enzyme function. Borderline or normal ADAMTS13 activity suggests a different diagnosis is more likely.
851:
Unlike HUS and aHUS, TTP is known to be caused by a defect in the ADAMTS13 protein, so a lab test showing ≤5% of normal ADAMTS13 levels is indicative of TTP. ADAMTS13 levels above 5%, coupled with a positive test for
2115:
Levy GG, Nichols WC, Lian EC, Foroud T, McClintick JN, McGee BM, Yang AY, Siemieniak DR, Stark KR, Gruppo R, Sarode R, Shurin SB, Chandrasekaran V, Stabler SP, Sabio H (October 2001). "Mutations in a member of the
3705:"Takeda's Adzynma (ADAMTS13, recombinant-krhn) Approved by U.S. FDA as the First and Only Recombinant ADAMTS13 Enzyme Replacement Therapy for the Treatment of Congenital Thrombotic Thrombocytopenic Purpura (cTTP)"
4440:
2707:"Performance Validation of Three Scoring Systems for the Prediction of Thrombotic Microangiopathy Due to Severe ADAMTS13 Deficiency and the Response to Therapeutic Plasma Exchange: First Study in Korea"
2557:
Konkle, Barbara (2022). "Chapter 115: Disorders of
Platelets and Vessel Wall". In Loscalzo, Joseph; Fauci, Anthony S.; Kasper, Dennis L.; Hauser, Stephen L.; Longo, Dan L.; Jameson, J. Larry (eds.).
1045:
The incidence of TTP is about 4–5 cases per million people per year. Idiopathic TTP occurs more often in women as well as people of
African descent, and TTP secondary to autoimmune disorders such as
1019:
are used to monitor disease progression or remission. ADAMTS13 activity and inhibitor levels may be measured during follow-up, but in those without symptoms the use of rituximab is not recommended.
288:, such that ADAMTS13 dysfunction is present from birth. Diagnosis is typically based on symptoms and blood tests. It may be supported by measuring activity of or antibodies against ADAMTS13.
4433:
5528:
2618:
Bentley MJ, Lehman CM, Blaylock RC, Wilson AR, Rodgers GM (August 2010). "The utility of patient characteristics in predicting severe ADAMTS13 deficiency and response to plasma exchange".
314:
About 1 per 100,000 people are affected. Onset is typically in adulthood and women are more often affected. About 10% of cases begin in childhood. The condition was first described by
551:
Secondary TTP is diagnosed when the person's history mentions one of the known features associated with TTP. It comprises about 40% of all cases of TTP. Predisposing factors are:
1033:
The mortality rate is around 95% for untreated cases, but the prognosis is reasonably favorable (80–90% survival) for people with idiopathic TTP diagnosed and treated early with
1053:
are at an increased risk for TTP, its presentation in Black people does not have any distinguishable features compared to those of other races. Pregnant women and women in the
3493:
for "Caplacizumab and
Immunosuppressive Therapy Without Firstline Therapeutic Plasma Exchange in Adults With Immune-mediated Thrombotic Thrombocytopenic Purpura (MAYARI)" at
1563:"Thrombotic Microangiopathy in Malignant Hypertension and Hemolytic Uremic Syndrome (HUS)/Thrombotic Thrombocytopenic Purpura (TTP): Can We Differentiate One from the Other?"
1198:
748:
Thrombotic thrombocytopenic purpura (TTP) initially presents with a range of symptoms that may include severe thrombocytopenia (platelet count usually < 30,000/mmÂł),
960:
guidelines recommended for first acute episode and relapses of immune-mediated TTP (iTTP), add corticosteroids to therapeutic plasma exchange (TPE) and consider adding
945:. However, the use of caplacizumab was associated with increase bleeding tendencies in some studied subjects. Its cost-effectiveness has also been questioned. Use of
3506:
Zheng XL, Vesely SK, Cataland SR, Coppo P, Geldziler B, Iorio A, Matsumoto M, Mustafa RA, Pai M, Rock G, Russell L, Tarawneh R, Valdes J, Peyvandi F (October 2020).
2754:
Zheng XL, Vesely SK, Cataland SR, Coppo P, Geldziler B, Iorio A, Matsumoto M, Mustafa RA, Pai M, Rock G, Russell L, Tarawneh R, Valdes J, Peyvandi F (October 2020).
3678:
6158:
3813:"The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency"
5521:
4940:
451:
2529:
Hardin, J. Matthew (2021). "13-07: Thrombotic
Thrombocytopenic Purpura". In Knoop, Kevin J.; Stack, Lawrence B.; Storrow, Alan B.; Thurman, R. Jason (eds.).
1203:
1025:(Adzynma) was approved for medical use in the United States in November 2023. The use of ADAMTS13 is well established for the treatment of congenital TTP.
848:
symptoms. Generally, TTP has higher rates of neurological symptoms (≤80%) and lower rates of renal symptoms (9%) than HUS (10–20% and 90%, respectively).
1079:
in 1924. Moschcowitz ascribed the disease (incorrectly, as now known) to a toxic cause. Moschcowitz noted his patient, a 16-year-old girl, had anemia,
2863:
1864:"A Severe Case of Congenital Thrombotic Thrombocytopenia Purpura Resulting From Compound Heterozygosity Involving a Novel ADAMTS13 Pathogenic Variant"
1546:
430:. These platelet-vWF complexes form small blood clots which circulate in the blood vessels and cause shearing of red blood cells, resulting in their
330:
or diarrheal illness before developing TTP. Neurological symptoms are very common and vary greatly in severity. Frequently reported symptoms include
5514:
2482:"Depression and cognitive impairment following recovery from thrombotic thrombocytopenic purpura: Depression and Cognitive Impairment Following TTP"
4158:
2656:"Predictive features of severe acquired ADAMTS13 deficiency in idiopathic thrombotic microangiopathies: the French TMA reference center experience"
2980:
Complement dysregulation leads to atypical hemolytic uremic syndrome (aHUS), while ADAMTS13 deficiency causes thrombotic thrombocytopenic purpura.
5645:
5247:
2189:
Kokame K, Matsumoto M, Soejima K, Yagi H, Ishizashi H, Funato M, Tamai H, Konno M, Kamide K, Kawano Y, Miyata T, Fujimura Y (September 2002).
2566:
2538:
2322:
2099:
1438:
941:
is an adjunct option in treating TTP as it has been shown that it induces a faster disease resolution compared with those people who were on
4252:"Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group"
2993:
3335:"Evidence-Based Minireview: Should caplacizumab be used routinely in unselected patients with immune thrombotic thrombocytopenic purpura?"
1863:
883:
are seen, and therapy is started. Transfusion is contraindicated in thrombotic TTP, as it fuels the coagulopathy. Since the early 1990s,
5803:
4345:
2340:"von Willebrand Factor Propeptide in Vascular Disorders: A Tool to Distinguish Between Acute and Chronic Endothelial Cell Perturbation"
740:
is common in those recovering from TTP; 59% of recovered TTP patients screened positive for depression within 11 years after recovery.
6163:
5029:
2913:
825:
378:) may also develop. The classic presentation of TTP, which occurs in less than 10% of people, includes five medical signs. These are:
138:
4930:
876:
813:
749:
478:
of TTP is credited to two independent groups of researchers (Furlan and Tsai) who published their research in the same issue of the
415:
402:
2313:
Menkes, John H.; Sarnat, Harvey B.; Maria, Bernard L. (2006). "Thrombotic
Thrombocytopenic Purpura and Hemolytic-Uremic Syndrome".
5882:
5834:
5039:
4116:"An Acute Febrile Pleiochromic Anemia with Hyaline Thrombosis of the Terminal Arterioles and Capillaries: An Undescribed Disease"
3266:
Peyvandi F, Scully M, Kremer
Hovinga JA, Cataland S, Knöbl P, Wu H, Artoni A, Westwood JP, Mansouri Taleghani M (February 2016).
1208:
480:
17:
5601:
4998:
4624:
1919:"Acquired thrombotic thrombocytopenic purpura: ADAMTS13 activity, anti-ADAMTS13 autoantibodies and risk of recurrent disease"
5888:
1049:
occurs more frequently in people of
African descent, although other secondary forms do not show this distribution. Although
3441:"Treatment of acquired thrombotic thrombocytopenic purpura without plasma exchange in selected patients under caplacizumab"
2994:"How I treat: the clinical differentiation and initial treatment of adult patients with atypical hemolytic uremic syndrome"
1059:
period accounted for a notable portion (12–31%) of the cases in some studies; TTP affects about one in 25,000 pregnancies.
5874:
5191:
4882:
4719:
4120:
5862:
5825:
4734:
2480:
Han B, Page EE, Stewart LM, Deford CC, Scott JG, Schwartz LH, Perdue JJ, Terrell DR, Vesely SK, George JN (August 2018).
1000:
536:
455:
285:
245:
In about half of cases a trigger is identified, while in the remainder the cause remains unknown. Known triggers include
6148:
5695:
5358:
5113:
4804:
1524:
1046:
254:
3862:"Different disparities of gender and race among the thrombotic thrombocytopenic purpura and hemolytic-uremic syndromes"
828:(aHUS). Consequently, differential diagnosis of these TMA diseases is essential. Both TTP and HUS are characterized by
6015:
5591:
5108:
4639:
4594:
2871:
2191:"Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity"
657:
560:
3042:
5970:
5815:
5461:
5181:
1072:
809:
3439:
Völker LA, Brinkkoetter PT, Knöbl PN, Krstic M, Kaufeld J, Menne J, Buxhofer-Ausch V, Miesbach W (November 2020).
1516:
953:
has been reported in select patients. The MAYARI study was designed to evaluate the effectiveness of this option.
6086:
5657:
5638:
5383:
857:
821:
134:
863:(EHEC), are more likely indicative of HUS, whereas absence of shiga-toxin/EHEC can confirm a diagnosis of aHUS.
6056:
5799:
5628:
5226:
4872:
4809:
4704:
4356:
3920:"Thrombotic Thrombocytopenic Purpura in Black People: Impact of Ethnicity on Survival and Genetic Risk Factors"
942:
693:(vWF) into smaller units. The increase in circulating multimers of vWF increases platelet adhesion to areas of
5839:
1100:
in people with TTP was made in 1998. The location of ADAMTS13 within the human genome was identified in 2001.
2893:
780:
The PLASMIC score, Bentley score, and French TMA score have been used to assess clinical probability of TTP.
6143:
6046:
5491:
5186:
4905:
4877:
4507:
2338:
van Mourik JA, Boertjes R, Huisveld IA, Fijnvandraat K, Pajkrt D, van Genderen PJ, Fijnheer R (1999-07-01).
796:
if ADAMTS13 test results are expected within 72 hours. If ADAMTS13 <10 IU/dL, continue caplacizumab and
443:
6153:
5960:
5935:
5893:
5466:
5378:
4694:
2386:
1842:
327:
129:
5955:
5945:
5763:
5368:
5295:
4983:
4517:
4497:
1008:
872:
690:
492:
427:
277:
3167:"The role of rituximab in the management of patients with acquired thrombotic thrombocytopenic purpura"
1541:
2339:
1462:
1381:
5711:
5606:
5596:
4714:
4512:
4487:
3931:
2202:
2129:
1096:
been linked with abnormally large von Willebrand factor multimers. The identification of a deficient
1088:
888:
774:
725:
formation. The presence of the thrombi reduces blood flow to organs resulting in cellular injury and
308:
4425:
3812:
2266:"Global prevalence of hereditary thrombotic thrombocytopenic purpura determined by genetic analysis"
5950:
5940:
5736:
5616:
5436:
5373:
5348:
5322:
5176:
5154:
4749:
4709:
4629:
4619:
4334:
3219:"Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias"
2932:
Feng S, Eyler SJ, Zhang Y, Maga T, Nester CM, Kroll MH, Smith RJ, Afshar-Kharghan V (August 2013).
924:
900:
761:
367:
359:
246:
231:
104:
81:
812:(TMA), the formation of blood clots in small blood vessels throughout the body, which can lead to
6076:
5758:
5563:
5285:
5149:
5034:
4993:
4867:
4862:
4857:
4684:
4180:
4098:
3899:
3842:
3660:
3592:
3494:
3315:
3248:
3078:
2246:
2171:
2071:
2014:
1956:
1899:
1725:
1665:
1600:
1498:
1359:
1299:
1167:
250:
108:
68:
5716:
5690:
4006:
326:
The signs and symptoms of TTP may at first be subtle and nonspecific. Many people experience an
4099:"Hyaline Thrombosis of the Terminal Arterioles and Capillaries: A Hitherto Undescribed Disease"
3615:"Thrombotic thrombocytopenic purpura: pathogenesis, diagnosis and potential novel therapeutics"
5965:
5741:
5650:
5343:
5049:
4925:
4920:
4799:
4773:
4699:
4689:
4527:
4502:
4396:
4367:
4273:
4232:
4137:
4079:
4061:
4020:
3967:
3949:
3891:
3834:
3793:
3744:
3652:
3634:
3584:
3576:
3537:
3470:
3421:
3372:
3354:
3307:
3299:
3240:
3196:
3138:
3070:
3062:
3023:
3015:
2971:
2953:
2909:
2845:
2827:
2785:
2736:
2687:
2635:
2600:
2562:
2534:
2511:
2462:
2444:
2400:
2367:
2359:
2318:
2295:
2238:
2220:
2163:
2155:
2095:
2063:
2055:
2006:
1998:
1948:
1940:
1891:
1883:
1804:
1796:
1757:
1717:
1709:
1657:
1639:
1592:
1584:
1490:
1482:
1434:
1409:
1401:
1351:
1343:
1291:
1283:
1159:
1151:
1055:
977:
770:
737:
488:
296:
153:
119:
56:
5791:
5731:
5726:
5586:
5441:
5290:
5196:
5144:
5014:
4789:
4724:
4263:
4222:
4214:
4170:
4129:
4069:
4051:
4010:
4002:
3957:
3939:
3881:
3873:
3824:
3783:
3775:
3736:
3704:
3642:
3626:
3568:
3527:
3519:
3460:
3452:
3411:
3403:
3362:
3346:
3289:
3279:
3230:
3186:
3178:
3128:
3120:
3112:
3054:
3005:
2961:
2945:
2901:
2835:
2819:
2775:
2767:
2726:
2718:
2677:
2667:
2627:
2592:
2501:
2493:
2452:
2436:
2351:
2285:
2277:
2228:
2210:
2145:
2137:
2045:
1990:
1930:
1875:
1788:
1701:
1647:
1631:
1574:
1474:
1393:
1333:
1273:
1143:
985:
880:
837:
817:
726:
718:
398:
390:
203:
199:
2355:
681:
The underlying mechanism typically involves autoantibody-mediated inhibition of the enzyme
6071:
5905:
5611:
5426:
5353:
5024:
4739:
4584:
4522:
1776:
1068:
1022:
904:
686:
519:
355:
315:
300:
292:
149:
5746:
4133:
3779:
1004:
monitored closely around these events with plasma being administered if the count drops.
3935:
2731:
2706:
2290:
2265:
2206:
2133:
1130:
Kremer Hovinga JA, Coppo P, Lämmle B, Moake JL, Miyata T, Vanhoorelbeke K (April 2017).
6096:
5808:
5751:
5633:
5486:
5476:
5393:
4935:
4763:
4227:
4202:
4115:
4074:
4039:
4015:
3990:
3986:
3962:
3919:
3886:
3861:
3788:
3763:
3679:"FDA Approves First Treatment for Patients with Rare Inherited Blood Clotting Disorder"
3647:
3557:"Thrombotic thrombocytopenic purpura related to severe ADAMTS13 deficiency in children"
3532:
3507:
3465:
3440:
3416:
3391:
3367:
3191:
3166:
3133:
3101:"Interventions for haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura"
3100:
2966:
2933:
2905:
2840:
2780:
2755:
2682:
2655:
2506:
2481:
2457:
2387:"An autopsy-based retrospective study of secondary thrombotic thrombocytopenic purpura"
1652:
1397:
1034:
989:
950:
908:
884:
466:
In 1998, the majority of cases were shown to be caused by the inhibition of the enzyme
426:
in the small blood vessels. Platelets are consumed in the aggregation process and bind
187:
4339:
4159:"Thrombotic thrombocytopenic purpura: report of 16 cases and review of the literature"
2596:
2233:
2190:
1862:
Conboy E, Partain PI, Warad D, Kluge ML, Arndt C, Chen D, Rodriguez V (January 2018).
6137:
6106:
5928:
5923:
5918:
5675:
5506:
5471:
5446:
5401:
5363:
5231:
5098:
4958:
4910:
4842:
4814:
4175:
3829:
3235:
3218:
2631:
2391:
2281:
2018:
1604:
1076:
841:
195:
4407:
4184:
3903:
3846:
3664:
3596:
3392:"Cost effectiveness of caplacizumab in acquired thrombotic thrombocytopenic purpura"
3319:
3252:
2250:
2075:
1960:
1729:
1669:
1635:
1502:
1363:
1171:
6116:
5558:
5416:
4988:
4968:
4783:
4778:
4768:
4609:
3390:
Goshua G, Sinha P, Hendrickson JE, Tormey C, Bendapudi PK, Lee AI (February 2021).
3116:
3082:
2175:
1903:
1689:
1303:
1050:
965:
946:
938:
916:
892:
845:
793:
752:(evidenced by schistocytes in the blood smear), and various clinical signs such as
710:
584:
508:
475:
439:
435:
3489:
3350:
2705:
Park SH, Kim HK, Jeong J, Lee SH, Lee YJ, Kim YJ, Jo JC, Lim JH (September 2023).
2034:"Thrombotic Microangiopathies, Thrombotic Thrombocytopenic Purpura, and ADAMTS-13"
1619:
523:
Thrombotic thrombocytopenic purpura is inherited in an autosomal recessive manner.
4361:
4218:
4040:"Thrombotic Thrombocytopenic Purpura: Pathophysiology, Diagnosis, and Management"
3944:
3811:
Terrell DR, Williams LA, Vesely SK, Lämmle B, Hovinga JA, George JN (July 2005).
3182:
3010:
2949:
2672:
1879:
1428:
1338:
1321:
1278:
1261:
5913:
5721:
5553:
5542:
5451:
5276:
4978:
4644:
4410:
4391:
4268:
4251:
3334:
2864:"MAHA, TTP, HUS, DIC... Oh My! Understanding Microangiopathic Hemolytic Anemias"
1978:
1792:
1749:
1016:
993:
981:
853:
722:
694:
638:
627:
623:
612:
602:
592:
588:
504:
423:
4413:
4372:
3860:
Terrell DR, Vesely SK, Kremer Hovinga JA, Lämmle B, George JN (November 2010).
1478:
6101:
6066:
6041:
5685:
5667:
5411:
5252:
5129:
4847:
4819:
4589:
4319:
3572:
2808:"Thrombotic microangiopathies: a general approach to diagnosis and management"
2722:
2440:
2317:(7th ed.). Philadelphia: Lippincott Williams & Wilkins. p. 525.
1918:
912:
650:
646:
607:
570:
528:
500:
375:
219:
73:
61:
4141:
4065:
3953:
3638:
3580:
3407:
3358:
3303:
3066:
3019:
2957:
2831:
2448:
2363:
2224:
2159:
2059:
2002:
1944:
1887:
1800:
1713:
1643:
1588:
1486:
1405:
1347:
1287:
1155:
1131:
6091:
6036:
5623:
5456:
5406:
5170:
4757:
4729:
4634:
4469:
4402:
3556:
2424:
1084:
1080:
969:
961:
920:
896:
797:
714:
705:
meet, which in turn results in the formation of small platelet clots called
702:
698:
642:
631:
617:
577:
565:
431:
304:
258:
235:
160:
112:
4236:
4203:"Von Willerbrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura"
4083:
4024:
3971:
3895:
3838:
3797:
3748:
3656:
3588:
3541:
3474:
3425:
3376:
3311:
3244:
3200:
3142:
3099:
Michael, M; Elliott, EJ; Ridley, GF; Hodson, EM; Craig, JC (January 2009).
3074:
3058:
3027:
2975:
2849:
2789:
2740:
2691:
2639:
2604:
2515:
2466:
2404:
2371:
2299:
2242:
2215:
2167:
2067:
2050:
2033:
2010:
1952:
1935:
1895:
1761:
1721:
1661:
1596:
1494:
1413:
1382:"Von Willebrand factor, ADAMTS-13, and thrombotic thrombocytopenic purpura"
1355:
1295:
1163:
4277:
3740:
3284:
3267:
2756:"ISTH guidelines for the diagnosis of thrombotic thrombocytopenic purpura"
1994:
1808:
6005:
6000:
5990:
5849:
5680:
5538:
5481:
5103:
5083:
5019:
4973:
4915:
4852:
4794:
4614:
4563:
4492:
2807:
2150:
1847:
1705:
1579:
1562:
1147:
1097:
1012:
753:
706:
682:
532:
496:
471:
467:
447:
419:
363:
351:
339:
335:
318:
in 1924. The underlying mechanism was determined in the 1980s and 1990s.
281:
273:
269:
262:
239:
227:
85:
4302:
4056:
3555:
Loirat C, Girma JP, Desconclois C, Coppo P, Veyradier A (January 2009).
2823:
6051:
6010:
5280:
5134:
5044:
4963:
4950:
4900:
4829:
4553:
4548:
4543:
3294:
3124:
2264:
Seidizadeh O, Cairo A, Mancini I, George JN, Peyvandi F (August 2024).
757:
597:
343:
331:
191:
3877:
3630:
3523:
3508:"ISTH guidelines for treatment of thrombotic thrombocytopenic purpura"
3456:
2771:
2497:
43:
5995:
4892:
4837:
4604:
4599:
4573:
4482:
4314:
2141:
1322:"How I treat patients with thrombotic thrombocytopenic purpura: 2010"
932:
833:
765:
555:
347:
295:
the risk of death has decreased from more than 90% to less than 20%.
266:
207:
3614:
2385:
Iwata H, Kami M, Hori A, Hamaki T, Takeuchi K, Mutou Y (June 2001).
1822:
968:. For asymptomatic iTTP with low plasma ADAMTS13 activity, consider
37:
Moschcowitz syndrome, idiopathic thrombotic thrombocytopenic purpura
2934:"Partial ADAMTS13 deficiency in atypical hemolytic uremic syndrome"
4558:
2920:
In contrast to TTP, HUS is not caused by a deficiency of ADAMTS13.
1620:"Clinical and laboratory diagnosis of TTP: an integrated approach"
829:
665:
518:
474:. Knowledge of this relationship between reduced ADAMTS13 and the
382:
223:
215:
211:
77:
350:
can also be seen. Other symptoms include, but are not limited to
5139:
4658:
4477:
4350:
4323:
1827:
1542:"Platelet Disorders — Thrombotic Thrombocytopenic Purpura (TTP)"
1207:. U.S. Department of Health & Human Services. Archived from
957:
928:
788:
5510:
4429:
3268:"Caplacizumab for Acquired Thrombotic Thrombocytopenic Purpura"
3043:"Treatment of typical (enteropathic) hemolytic uremic syndrome"
976:
People with refractory or relapsing TTP may receive additional
717:
within blood vessels, which in turn leads to microangiopathic
1977:
Kremer Hovinga JA, George JN (October 2019). Longo DL (ed.).
51:
Spontaneous bruising in a woman with critically low platelets
4250:
Rock GA, Shumak KH, Buskard NA, et al. (August 1991).
2900:. Vol. 1 (24th ed.). Elsevier. pp. 1124–31.
820:. This characteristic is shared by two related syndromes,
3764:"Current Concepts in Thrombotic Thrombocytopenic Purpura"
3707:(Press release). Takeda Pharmaceuticals. 9 November 2023
3217:
Allford SL, Hunt BJ, Rose P, Machin SJ (February 2003).
2425:"Pathophysiology of thrombotic thrombocytopenic purpura"
2120:
gene family cause thrombotic thrombocytopenic purpura".
887:
has become the treatment of choice for TTP. This is an
1823:"THROMBOTIC THROMBOCYTOPENIC PURPURA, HEREDITARY; TTP"
1199:"Immune-mediated thrombotic thrombocytopenic purpura"
4292:
1748:
Stanley M, Killeen RB, Michalski JM (January 2023).
503:, and the blood vessel wall in the process of blood
454:: an inherited deficiency of ADAMTS13 (known as the
6026:
5983:
5904:
5848:
5790:
5783:
5776:
5704:
5666:
5579:
5572:
5425:
5392:
5336:
5321:
5312:
5269:
5240:
5219:
5210:
5163:
5122:
5091:
5080:
5073:
5062:
5007:
4949:
4891:
4828:
4748:
4677:
4666:
4657:
4572:
4536:
4468:
4461:
4382:
4296:
773:presentation of one or more classic symptoms and a
656:Hormone altering drugs (estrogens, contraceptives,
438:. The two best understood causes of TTP are due to
169:
159:
145:
128:
118:
99:
91:
67:
55:
33:
28:
3041:Bitzan M, Schaefer F, Reymond D (September 2010).
4456:Diseases of the skin and appendages by morphology
4038:Sukumar S, Lämmle B, Cataland SR (January 2021).
3727:Congenital Thrombotic Thrombocytopenic Purpura".
2992:Cataland, Spero R.; Wu, Haifeng M. (April 2014).
2032:Chapman K, Seldon M, Richards R (February 2012).
1917:Lämmle B, Hovinga JA, George JN (February 2008).
4103:Proceedings of the New York Pathological Society
2806:Arnold DM, Patriquin CJ, Nazy I (January 2017).
1979:"Hereditary Thrombotic Thrombocytopenic Purpura"
535:gene. This hereditary form of TTP is called the
272:. This results in decreased break down of large
3094:
3092:
2195:Proceedings of the National Academy of Sciences
2094:. Vol. 2 (15th ed.). Wolters Kluwer.
531:. Such cases may be caused by mutations in the
280:(vWF) into smaller units. Less commonly TTP is
3991:"Pathogenesis of Thrombotic Microangiopathies"
3212:
3210:
871:Due to the high mortality of untreated TTP, a
261:. The underlying mechanism typically involves
5522:
4941:Pityriasis lichenoides et varioliformis acuta
4441:
4196:
4194:
1777:"Moschcowitz, Multimers, and Metalloprotease"
1456:
1454:
1452:
1450:
8:
4595:Dermatofibroma (benign fibrous histiocytoma)
3613:Saha M, McDaniel J, Zheng X (October 2010).
3160:
3158:
3156:
3154:
3152:
1683:
1681:
1679:
1204:Genetic and Rare Diseases Information Center
760:, neurologic symptoms, myocardial ischemia,
713:, which damages their membranes, leading to
689:responsible for cleaving large multimers of
4007:10.1146/annurev.pathmechdis.3.121806.154311
3105:The Cochrane Database of Systematic Reviews
1972:
1970:
1427:Wood, Marie E.; Philips, George K. (2003).
1375:
1373:
5787:
5780:
5576:
5529:
5515:
5507:
5318:
5216:
5088:
5077:
5070:
4674:
4663:
4465:
4448:
4434:
4426:
4293:
3165:Lim W, Vesely SK, George JN (March 2015).
2559:Harrison's principles of internal medicine
1260:Joly BS, Coppo P, Veyradier A (May 2017).
1255:
1253:
1251:
1249:
1247:
1245:
1193:
1191:
1189:
1187:
1185:
1183:
1181:
204:low red blood cells due to their breakdown
42:
25:
4267:
4226:
4174:
4073:
4055:
4014:
3961:
3943:
3885:
3828:
3787:
3646:
3531:
3464:
3415:
3366:
3293:
3283:
3234:
3190:
3132:
3009:
2965:
2839:
2779:
2730:
2681:
2671:
2505:
2456:
2289:
2232:
2214:
2149:
2049:
1934:
1651:
1578:
1547:National Heart, Lung, and Blood Institute
1463:"Syndromes of Thrombotic Microangiopathy"
1337:
1315:
1313:
1277:
1243:
1241:
1239:
1237:
1235:
1233:
1231:
1229:
1227:
1225:
1125:
1123:
1121:
1119:
1117:
1115:
1113:
446:TTP), caused by autoantibodies targeting
3333:Goshua G, Bendapudi PK (December 2022).
1868:Journal of Pediatric Hematology/Oncology
2533:(5th ed.). McGraw-Hill Education.
1109:
198:throughout the body. This results in a
5248:Disseminated intravascular coagulation
2356:10.1182/blood.V94.1.179.413k18_179_185
1618:Chiasakul T, Cuker A (November 2018).
1561:Shibagaki Y, Fujita T (January 2005).
1433:(3rd ed.). Elsevier. p. 68.
6159:Vascular-related cutaneous conditions
4157:Amorosi EL, Ultmann JE (March 1966).
3817:Journal of Thrombosis and Haemostasis
3619:Journal of Thrombosis and Haemostasis
3608:
3606:
3047:Seminars in Thrombosis and Hemostasis
2801:
2799:
2552:
2550:
2418:
2416:
2414:
2038:Seminars in Thrombosis and Hemostasis
1843:"Thrombotic thrombocytopenic purpura"
1750:"Thrombotic Thrombocytopenic Purpura"
1743:
1741:
1739:
1517:"Thrombotic thrombocytopenic purpura"
1262:"Thrombotic thrombocytopenic purpura"
1132:"Thrombotic thrombocytopenic purpura"
370:has also been observed as a symptom.
7:
4134:10.1001/archinte.1925.00120130092009
3780:10.1146/annurev.med.57.061804.084505
2583:microangiopathies: a cohort study".
1536:
1534:
1461:Shatzel JJ, Taylor JA (March 2017).
777:revealing severe thrombocytopenia.
2896:. In Goldman L, Schafer AI (eds.).
2429:International Journal of Hematology
899:and replacement with donor plasma (
346:and symptoms similar to those of a
180:Thrombotic thrombocytopenic purpura
29:Thrombotic thrombocytopenic purpura
5030:Postinflammatory hyperpigmentation
2906:10.1016/B978-1-4377-1604-7.00175-5
1398:10.1053/j.seminhematol.2003.10.003
1092:were found to be very high (90%).
891:involving removal of the person's
826:atypical hemolytic uremic syndrome
418:(MAHAs), is caused by spontaneous
416:microangiopathic hemolytic anemias
218:dysfunction. Symptoms may include
139:atypical hemolytic uremic syndrome
14:
4931:Transient acantholytic dermatosis
3683:U.S. Food and Drug Administration
877:microangiopathic hemolytic anemia
814:microangiopathic hemolytic anemia
750:microangiopathic hemolytic anemia
622:Platelet aggregation inhibitors (
491:responsible for the breakdown of
403:microangiopathic hemolytic anemia
124:Based on symptoms and blood tests
5883:platelet storage pool deficiency
5835:Heparin-induced thrombocytopenia
5040:Idiopathic guttate hypomelanosis
4176:10.1097/00005792-196603000-00003
3830:10.1111/j.1538-7836.2005.01436.x
3685:(Press release). 9 November 2023
3236:10.1046/j.1365-2141.2003.04049.x
2632:10.1111/j.1537-2995.2010.02653.x
2282:10.1182/bloodadvances.2024013421
1467:Medical Clinics of North America
1007:Measurements of blood levels of
5050:Hypopigmented mycosis fungoides
4256:New England Journal of Medicine
3272:New England Journal of Medicine
2531:The atlas of emergency medicine
1983:New England Journal of Medicine
1781:New England Journal of Medicine
1694:New England Journal of Medicine
1636:10.1182/asheducation-2018.1.530
1083:and large bruises, microscopic
1067:TTP was initially described by
481:New England Journal of Medicine
422:of platelets and activation of
362:, or dots on the skin known as
311:are generally not recommended.
242:. Repeated episodes may occur.
18:Immune thrombocytopenic purpura
5602:Activated protein C resistance
5291:Lichen sclerosis et atrophicus
4999:Subcorneal pustular dermatosis
4625:Infantile digital fibromatosis
3866:American Journal of Hematology
3223:British Journal of Haematology
3117:10.1002/14651858.CD003595.pub2
2561:(21st ed.). McGraw Hill.
2486:American Journal of Hematology
2092:Wintrobe's Clinical Hematology
1690:"Thrombotic Microangiopathies"
1136:Nature Reviews Disease Primers
875:of TTP is made even when only
1:
4883:Epidermolysis bullosa simplex
4720:Langerhans cell histiocytosis
4121:Archives of Internal Medicine
3351:10.1182/hematology.2022000412
2868:Tampa Emergency Medicine Blog
2597:10.1016/S2352-3026(17)30026-1
5696:Trousseau sign of malignancy
5359:Systemic lupus erythematosus
5114:Systemic lupus erythematosus
4805:Systemic lupus erythematosus
4219:10.1182/blood-2008-02-078170
4044:Journal of Clinical Medicine
3945:10.1371/journal.pone.0156679
3183:10.1182/blood-2014-10-559211
3011:10.1182/blood-2013-11-516237
2950:10.1182/blood-2013-03-492421
2673:10.1371/journal.pone.0010208
1880:10.1097/MPH.0000000000000895
1525:National Library of Medicine
1339:10.1182/blood-2010-07-271445
1279:10.1182/blood-2016-10-709857
1047:systemic lupus erythematosus
495:(vWF), a protein that links
6016:Nonthrombocytopenic purpura
5592:Antithrombin III deficiency
4640:Lymphangioma circumscriptum
4269:10.1056/NEJM199108083250604
3111:(1): CD003595–CD003595–63.
2872:University of South Florida
1793:10.1056/NEJM199811263392210
1430:Hematology/Oncology Secrets
1320:George JN (November 2010).
697:injury, particularly where
658:hormone replacement therapy
561:Bone marrow transplantation
354:or paleness of the skin, a
6180:
5971:Congenital afibrinogenemia
5875:Glanzmann's thrombasthenia
5462:Mucous membrane pemphigoid
3995:Annual Review of Pathology
3715:– via Business Wire.
1775:Moake JL (November 1998).
1479:10.1016/j.mcna.2016.09.010
810:thrombotic microangiopathy
715:rupture of red blood cells
15:
6164:Syndromes affecting blood
6087:Gastrointestinal bleeding
5889:Hermansky–Pudlak syndrome
5658:Antiphospholipid syndrome
5639:Essential thrombocythemia
5549:
3768:Annual Review of Medicine
3762:Tsai HM (February 2006).
3573:10.1007/s00467-008-0863-5
2723:10.3343/alm.2023.43.5.485
2441:10.1007/s12185-009-0476-1
1756:. StatPearls Publishing.
1380:Moake JL (January 2004).
822:hemolytic-uremic syndrome
135:Hemolytic-uremic syndrome
50:
41:
6057:Subconjunctival bleeding
5863:Bernard–Soulier syndrome
5826:Upshaw–Schulman syndrome
5800:Thrombocytopenic purpura
5629:Sticky platelet syndrome
5227:Thrombocytopenic purpura
4873:Dermatitis herpetiformis
4858:Acute contact dermatitis
4810:Pityriasis rubra pilaris
4735:Wiskott–Aldrich syndrome
4705:Lichen simplex chronicus
4585:Epidermal inclusion cyst
3408:10.1182/blood.2020006052
2898:Goldman's Cecil Medicine
2423:Tsai HM (January 2010).
1688:Moake JL (August 2022).
1001:Upshaw-Schulman syndrome
537:Upshaw–Schulman syndrome
456:Upshaw–Schulman syndrome
387:Changes in mental status
286:Upshaw–Schulman syndrome
16:Not to be confused with
6047:Intracranial hemorrhage
5492:Squamous-cell carcinoma
5487:Systemic histoplasmosis
4878:Porphyria cutanea tarda
4508:Squamous-cell carcinoma
4114:—— (1925).
395:Reduced kidney function
249:, certain medications,
107:, certain medications,
5961:Factor XIII deficiency
5941:Hypoprothrombinemia/II
5936:von Willebrand disease
5894:Gray platelet syndrome
5467:Cicatricial pemphigoid
5384:Acne keloidalis nuchae
5379:Folliculitis decalvans
4097:Moschcowitz E (1924).
3487:Clinical trial number
3059:10.1055/s-0030-1262881
2216:10.1073/pnas.172277399
2051:10.1055/s-0031-1300951
1936:10.3324/haematol.12701
1386:Seminars in Hematology
804:Differential diagnosis
524:
374:gums. Larger bruises (
165:< 20% risk of death
130:Differential diagnosis
5956:Factor XII deficiency
5946:Factor VII deficiency
5764:Renal vein thrombosis
5369:Loose anagen syndrome
5296:Necrobiosis lipoidica
5232:Actinic/solar purpura
4906:Insect bite reactions
4695:Seborrheic dermatitis
4518:Merkel-cell carcinoma
4498:Molluscum contagiosum
3741:10.1056/NEJMoa2314793
3285:10.1056/NEJMoa1505533
1995:10.1056/NEJMra1813013
1567:Hypertension Research
1009:lactate dehydrogenase
919:) are usually given.
873:presumptive diagnosis
691:von Willebrand factor
522:
493:von Willebrand factor
309:Platelet transfusions
278:von Willebrand factor
5712:Deep vein thrombosis
5607:Protein S deficiency
5597:Protein C deficiency
5192:Annulare centrifugum
4715:Glucagonoma syndrome
4513:Basal-cell carcinoma
4488:Seborrheic keratosis
3561:Pediatric Nephrology
1706:10.1056/NEJMra020528
1580:10.1291/hypres.28.89
1521:MedlinePlus Genetics
1148:10.1038/nrdp.2017.20
1073:Beth Israel Hospital
889:exchange transfusion
775:complete blood count
637:Immunosuppressants (
587:medications such as
247:bacterial infections
105:bacterial infections
6149:Autoimmune diseases
5951:Factor X deficiency
5840:May–Hegglin anomaly
5617:Prothrombin G20210A
5437:Aphthous stomatitis
5374:Lichen planopilaris
5349:Androgenic alopecia
5155:Fixed drug eruption
4730:Pemphigus foliaceus
4630:Granular cell tumor
4201:Sadler, JE (2008).
4057:10.3390/jcm10030536
3936:2016PLoSO..1156679M
2824:10.1503/cmaj.160142
2207:2002PNAS...9911902K
2134:2001Natur.413..488L
1087:, and, at autopsy,
925:monoclonal antibody
901:fresh frozen plasma
762:mesenteric ischemia
414:TTP, as with other
368:High blood pressure
360:shortness of breath
251:autoimmune diseases
232:shortness of breath
173:1 in 100,000 people
109:autoimmune diseases
82:shortness of breath
6077:Pulmonary hematoma
5984:Signs and symptoms
5759:Pulmonary embolism
5564:Bleeding diathesis
5457:Pemphigus vulgaris
5286:Granuloma annulare
5150:Carcinoid syndrome
5035:Tuberous sclerosis
4994:Coccidioidomycosis
4868:Bullous pemphigoid
4863:Pemphigus vulgaris
4795:Secondary syphilis
4685:Contact dermatitis
4383:External resources
3495:ClinicalTrials.gov
2894:"Thrombocytopenia"
2892:Abrams CS (2012).
858:enterohemorrhagic
525:
332:feeling very tired
322:Signs and symptoms
307:may also be used.
297:Immunosuppressants
200:low platelet count
154:immunosuppressants
6131:
6130:
6127:
6126:
5979:
5978:
5966:Dysfibrinogenemia
5850:Platelet function
5772:
5771:
5651:Purpura fulminans
5504:
5503:
5500:
5499:
5344:Telogen effluvium
5308:
5307:
5304:
5303:
5265:
5264:
5261:
5260:
5206:
5205:
5058:
5057:
4926:Lichen spinulosus
4921:Keratosis pilaris
4800:Mycosis fungoides
4700:Stasis dermatitis
4690:Atopic dermatitis
4653:
4652:
4528:Trichoepithelioma
4503:Actinic keratosis
4423:
4422:
3878:10.1002/ajh.21833
3631:10.1111/jth.13764
3625:(10): 1889–1900.
3524:10.1111/jth.15010
3518:(10): 2496–2502.
3457:10.1111/jth.15045
2772:10.1111/jth.15006
2568:978-1-264-26851-1
2540:978-1-260-13495-7
2498:10.1002/ajh.24060
2324:978-0-7817-5104-9
2128:(6855): 488–494.
2101:978-1-975184-69-8
1550:. 24 March 2022 .
1440:978-1-56053-516-4
978:immunosuppressive
808:TTP is a form of
576:Antiviral drugs (
489:metalloproteinase
434:and formation of
194:forming in small
177:
176:
120:Diagnostic method
23:Medical condition
6171:
5792:Thrombocytopenia
5788:
5781:
5737:Lowenberg's sign
5587:Clotting factors
5577:
5531:
5524:
5517:
5508:
5442:Oral candidiasis
5319:
5217:
5145:Erythema nodosum
5089:
5078:
5071:
5015:Tinea versicolor
4853:Bullous impetigo
4790:Pityriasis rosea
4725:Lichen sclerosus
4710:Darier's disease
4675:
4664:
4620:Kaposi's sarcoma
4466:
4450:
4443:
4436:
4427:
4294:
4282:
4281:
4271:
4247:
4241:
4240:
4230:
4198:
4189:
4188:
4178:
4154:
4148:
4145:
4110:
4094:
4088:
4087:
4077:
4059:
4035:
4029:
4028:
4018:
3982:
3976:
3975:
3965:
3947:
3914:
3908:
3907:
3889:
3857:
3851:
3850:
3832:
3808:
3802:
3801:
3791:
3759:
3753:
3752:
3723:
3717:
3716:
3714:
3712:
3701:
3695:
3694:
3692:
3690:
3675:
3669:
3668:
3650:
3610:
3601:
3600:
3552:
3546:
3545:
3535:
3512:J Thromb Haemost
3503:
3497:
3485:
3479:
3478:
3468:
3445:J Thromb Haemost
3436:
3430:
3429:
3419:
3387:
3381:
3380:
3370:
3330:
3324:
3323:
3297:
3287:
3263:
3257:
3256:
3238:
3214:
3205:
3204:
3194:
3162:
3147:
3146:
3136:
3096:
3087:
3086:
3038:
3032:
3031:
3013:
2989:
2983:
2982:
2969:
2929:
2923:
2922:
2889:
2883:
2882:
2880:
2879:
2860:
2854:
2853:
2843:
2818:(4): E153–E159.
2803:
2794:
2793:
2783:
2760:J Thromb Haemost
2751:
2745:
2744:
2734:
2702:
2696:
2695:
2685:
2675:
2650:
2644:
2643:
2615:
2609:
2608:
2591:(4): e157–e164.
2579:
2573:
2572:
2554:
2545:
2544:
2526:
2520:
2519:
2509:
2477:
2471:
2470:
2460:
2420:
2409:
2408:
2382:
2376:
2375:
2335:
2329:
2328:
2310:
2304:
2303:
2293:
2261:
2255:
2254:
2236:
2218:
2186:
2180:
2179:
2153:
2142:10.1038/35097008
2112:
2106:
2105:
2086:
2080:
2079:
2053:
2029:
2023:
2022:
1974:
1965:
1964:
1938:
1914:
1908:
1907:
1859:
1853:
1852:
1839:
1833:
1832:
1819:
1813:
1812:
1772:
1766:
1765:
1745:
1734:
1733:
1685:
1674:
1673:
1655:
1615:
1609:
1608:
1582:
1558:
1552:
1551:
1538:
1529:
1528:
1513:
1507:
1506:
1458:
1445:
1444:
1424:
1418:
1417:
1377:
1368:
1367:
1341:
1317:
1308:
1307:
1281:
1257:
1220:
1219:
1217:
1216:
1195:
1176:
1175:
1127:
986:cyclophosphamide
881:thrombocytopenia
838:thrombocytopenia
818:thrombocytopenia
727:end organ damage
719:hemolytic anemia
527:TTP may also be
399:Hemolytic anemia
391:Thrombocytopenia
190:that results in
46:
26:
6179:
6178:
6174:
6173:
6172:
6170:
6169:
6168:
6134:
6133:
6132:
6123:
6072:Hemopericardium
6022:
5975:
5906:Clotting factor
5900:
5844:
5768:
5717:Bancroft's sign
5700:
5691:Virchow's triad
5662:
5612:Factor V Leiden
5568:
5545:
5535:
5505:
5496:
5428:
5421:
5388:
5354:Alopecia areata
5332:
5314:
5300:
5257:
5236:
5212:
5202:
5159:
5118:
5104:Viral exanthems
5082:
5066:
5064:
5054:
5025:Pityriasis alba
5003:
4945:
4887:
4824:
4744:
4740:Zinc deficiency
4670:
4668:
4649:
4577:
4568:
4532:
4523:Nevus sebaceous
4457:
4454:
4424:
4419:
4418:
4378:
4377:
4305:
4291:
4286:
4285:
4249:
4248:
4244:
4200:
4199:
4192:
4156:
4155:
4151:
4113:
4096:
4095:
4091:
4037:
4036:
4032:
3984:
3983:
3979:
3930:(7): e0156679.
3916:
3915:
3911:
3859:
3858:
3854:
3810:
3809:
3805:
3761:
3760:
3756:
3735:(17): 1584–96.
3725:
3724:
3720:
3710:
3708:
3703:
3702:
3698:
3688:
3686:
3677:
3676:
3672:
3612:
3611:
3604:
3554:
3553:
3549:
3505:
3504:
3500:
3486:
3482:
3438:
3437:
3433:
3389:
3388:
3384:
3332:
3331:
3327:
3265:
3264:
3260:
3216:
3215:
3208:
3177:(10): 1526–31.
3164:
3163:
3150:
3098:
3097:
3090:
3040:
3039:
3035:
3004:(16): 2478–84.
2991:
2990:
2986:
2931:
2930:
2926:
2916:
2891:
2890:
2886:
2877:
2875:
2862:
2861:
2857:
2805:
2804:
2797:
2766:(10): 2486–95.
2753:
2752:
2748:
2704:
2703:
2699:
2652:
2651:
2647:
2617:
2616:
2612:
2585:Lancet Haematol
2581:
2580:
2576:
2569:
2556:
2555:
2548:
2541:
2528:
2527:
2523:
2479:
2478:
2474:
2422:
2421:
2412:
2384:
2383:
2379:
2337:
2336:
2332:
2325:
2315:Child Neurology
2312:
2311:
2307:
2276:(16): 4386–96.
2263:
2262:
2258:
2201:(18): 11902–7.
2188:
2187:
2183:
2114:
2113:
2109:
2102:
2088:
2087:
2083:
2031:
2030:
2026:
1989:(17): 1653–62.
1976:
1975:
1968:
1916:
1915:
1911:
1861:
1860:
1856:
1841:
1840:
1836:
1821:
1820:
1816:
1787:(22): 1629–31.
1774:
1773:
1769:
1747:
1746:
1737:
1687:
1686:
1677:
1617:
1616:
1612:
1560:
1559:
1555:
1540:
1539:
1532:
1515:
1514:
1510:
1460:
1459:
1448:
1441:
1426:
1425:
1421:
1379:
1378:
1371:
1319:
1318:
1311:
1272:(21): 2836–46.
1259:
1258:
1223:
1214:
1212:
1197:
1196:
1179:
1129:
1128:
1111:
1106:
1069:Eli Moschcowitz
1065:
1043:
1031:
1023:Apadamtase alfa
909:Corticosteroids
905:cryosupernatant
869:
806:
746:
735:
687:metalloprotease
679:
677:Pathophysiology
549:
517:
464:
412:
356:fast heart rate
324:
316:Eli Moschcowitz
301:glucocorticoids
293:plasma exchange
265:inhibiting the
150:Plasma exchange
24:
21:
12:
11:
5:
6177:
6175:
6167:
6166:
6161:
6156:
6151:
6146:
6144:Coagulopathies
6136:
6135:
6129:
6128:
6125:
6124:
6122:
6121:
6120:
6119:
6111:
6110:
6109:
6104:
6099:
6097:Hemoperitoneum
6094:
6089:
6081:
6080:
6079:
6074:
6069:
6061:
6060:
6059:
6054:
6049:
6044:
6039:
6030:
6028:
6024:
6023:
6021:
6020:
6019:
6018:
6008:
6003:
5998:
5993:
5987:
5985:
5981:
5980:
5977:
5976:
5974:
5973:
5968:
5963:
5958:
5953:
5948:
5943:
5938:
5933:
5932:
5931:
5926:
5921:
5910:
5908:
5902:
5901:
5899:
5898:
5897:
5896:
5891:
5879:
5878:
5877:
5867:
5866:
5865:
5854:
5852:
5846:
5845:
5843:
5842:
5837:
5831:
5830:
5829:
5828:
5823:
5813:
5812:
5811:
5809:Evans syndrome
5796:
5794:
5785:
5778:
5774:
5773:
5770:
5769:
5767:
5766:
5761:
5756:
5755:
5754:
5749:
5744:
5742:Peabody's sign
5739:
5734:
5729:
5724:
5719:
5708:
5706:
5702:
5701:
5699:
5698:
5693:
5688:
5683:
5678:
5672:
5670:
5664:
5663:
5661:
5660:
5655:
5654:
5653:
5643:
5642:
5641:
5636:
5634:Thrombocytosis
5631:
5621:
5620:
5619:
5614:
5609:
5604:
5599:
5594:
5583:
5581:
5574:
5570:
5569:
5567:
5566:
5561:
5556:
5550:
5547:
5546:
5536:
5534:
5533:
5526:
5519:
5511:
5502:
5501:
5498:
5497:
5495:
5494:
5489:
5484:
5479:
5477:Coxsackievirus
5474:
5469:
5464:
5459:
5454:
5449:
5444:
5439:
5433:
5431:
5423:
5422:
5420:
5419:
5414:
5409:
5404:
5398:
5396:
5390:
5389:
5387:
5386:
5381:
5376:
5371:
5366:
5361:
5356:
5351:
5346:
5340:
5338:
5334:
5333:
5331:
5330:
5327:
5325:
5316:
5310:
5309:
5306:
5305:
5302:
5301:
5299:
5298:
5293:
5288:
5283:
5273:
5271:
5267:
5266:
5263:
5262:
5259:
5258:
5256:
5255:
5250:
5244:
5242:
5238:
5237:
5235:
5234:
5229:
5223:
5221:
5214:
5208:
5207:
5204:
5203:
5201:
5200:
5194:
5189:
5187:Gyratum repens
5184:
5179:
5173:
5167:
5165:
5161:
5160:
5158:
5157:
5152:
5147:
5142:
5137:
5132:
5126:
5124:
5120:
5119:
5117:
5116:
5111:
5109:Toxic erythema
5106:
5101:
5099:Drug eruptions
5095:
5093:
5086:
5075:
5068:
5060:
5059:
5056:
5055:
5053:
5052:
5047:
5042:
5037:
5032:
5027:
5022:
5017:
5011:
5009:
5005:
5004:
5002:
5001:
4996:
4991:
4986:
4981:
4976:
4971:
4966:
4961:
4955:
4953:
4947:
4946:
4944:
4943:
4938:
4936:Lichen nitidus
4933:
4928:
4923:
4918:
4913:
4908:
4903:
4897:
4895:
4889:
4888:
4886:
4885:
4880:
4875:
4870:
4865:
4860:
4855:
4850:
4845:
4840:
4838:Herpes simplex
4834:
4832:
4826:
4825:
4823:
4822:
4817:
4812:
4807:
4802:
4797:
4792:
4787:
4781:
4776:
4771:
4766:
4760:
4754:
4752:
4746:
4745:
4743:
4742:
4737:
4732:
4727:
4722:
4717:
4712:
4707:
4702:
4697:
4692:
4687:
4681:
4679:
4672:
4661:
4655:
4654:
4651:
4650:
4648:
4647:
4642:
4637:
4632:
4627:
4622:
4617:
4612:
4607:
4602:
4597:
4592:
4587:
4581:
4579:
4570:
4569:
4567:
4566:
4561:
4556:
4551:
4546:
4540:
4538:
4534:
4533:
4531:
4530:
4525:
4520:
4515:
4510:
4505:
4500:
4495:
4490:
4485:
4480:
4474:
4472:
4463:
4459:
4458:
4455:
4453:
4452:
4445:
4438:
4430:
4421:
4420:
4417:
4416:
4399:
4387:
4386:
4384:
4380:
4379:
4376:
4375:
4364:
4353:
4342:
4327:
4306:
4301:
4300:
4298:
4297:Classification
4290:
4289:External links
4287:
4284:
4283:
4242:
4190:
4149:
4147:
4146:
4089:
4030:
3977:
3909:
3852:
3803:
3754:
3718:
3696:
3670:
3602:
3547:
3498:
3480:
3451:(11): 3061–6.
3431:
3402:(7): 969–976.
3382:
3325:
3278:(6): 511–522.
3258:
3206:
3148:
3088:
3053:(6): 594–610.
3033:
2984:
2944:(8): 1487–93.
2924:
2915:978-1437716047
2914:
2884:
2855:
2795:
2746:
2717:(5): 485–492.
2697:
2645:
2626:(8): 1654–64.
2610:
2574:
2567:
2546:
2539:
2521:
2492:(8): 709–714.
2472:
2410:
2377:
2350:(1): 179–185.
2330:
2323:
2305:
2256:
2181:
2107:
2100:
2081:
2024:
1966:
1909:
1854:
1834:
1814:
1767:
1735:
1700:(8): 589–600.
1675:
1610:
1553:
1530:
1508:
1473:(2): 395–415.
1446:
1439:
1419:
1369:
1332:(20): 4060–9.
1309:
1221:
1177:
1142:17020: 17020.
1108:
1107:
1105:
1102:
1064:
1061:
1042:
1039:
1035:plasmapheresis
1030:
1027:
999:Children with
990:cyclosporine A
980:therapy, e.g.
951:plasmapheresis
885:plasmapheresis
868:
865:
805:
802:
745:
742:
734:
731:
678:
675:
670:
669:
663:
662:
661:
654:
635:
620:
615:
610:
605:
600:
595:
581:
568:
563:
558:
548:
545:
516:
513:
487:ADAMTS13 is a
463:
460:
452:congenital TTP
411:
408:
407:
406:
396:
393:
388:
385:
328:influenza-like
323:
320:
188:blood disorder
175:
174:
171:
167:
166:
163:
157:
156:
147:
143:
142:
132:
126:
125:
122:
116:
115:
101:
97:
96:
93:
89:
88:
71:
65:
64:
59:
53:
52:
48:
47:
39:
38:
35:
31:
30:
22:
13:
10:
9:
6:
4:
3:
2:
6176:
6165:
6162:
6160:
6157:
6155:
6154:Rare diseases
6152:
6150:
6147:
6145:
6142:
6141:
6139:
6118:
6115:
6114:
6112:
6108:
6107:Hematosalpinx
6105:
6103:
6100:
6098:
6095:
6093:
6090:
6088:
6085:
6084:
6082:
6078:
6075:
6073:
6070:
6068:
6065:
6064:
6062:
6058:
6055:
6053:
6050:
6048:
6045:
6043:
6040:
6038:
6035:
6034:
6032:
6031:
6029:
6025:
6017:
6014:
6013:
6012:
6009:
6007:
6004:
6002:
5999:
5997:
5994:
5992:
5989:
5988:
5986:
5982:
5972:
5969:
5967:
5964:
5962:
5959:
5957:
5954:
5952:
5949:
5947:
5944:
5942:
5939:
5937:
5934:
5930:
5927:
5925:
5922:
5920:
5917:
5916:
5915:
5912:
5911:
5909:
5907:
5903:
5895:
5892:
5890:
5887:
5886:
5885:
5884:
5880:
5876:
5873:
5872:
5871:
5868:
5864:
5861:
5860:
5859:
5856:
5855:
5853:
5851:
5847:
5841:
5838:
5836:
5833:
5832:
5827:
5824:
5822:
5819:
5818:
5817:
5814:
5810:
5807:
5806:
5805:
5801:
5798:
5797:
5795:
5793:
5789:
5786:
5782:
5779:
5775:
5765:
5762:
5760:
5757:
5753:
5750:
5748:
5745:
5743:
5740:
5738:
5735:
5733:
5732:Louvel's sign
5730:
5728:
5727:Lisker's sign
5725:
5723:
5720:
5718:
5715:
5714:
5713:
5710:
5709:
5707:
5703:
5697:
5694:
5692:
5689:
5687:
5684:
5682:
5679:
5677:
5676:Thrombophilia
5674:
5673:
5671:
5669:
5665:
5659:
5656:
5652:
5649:
5648:
5647:
5644:
5640:
5637:
5635:
5632:
5630:
5627:
5626:
5625:
5622:
5618:
5615:
5613:
5610:
5608:
5605:
5603:
5600:
5598:
5595:
5593:
5590:
5589:
5588:
5585:
5584:
5582:
5578:
5575:
5571:
5565:
5562:
5560:
5557:
5555:
5552:
5551:
5548:
5544:
5540:
5537:Disorders of
5532:
5527:
5525:
5520:
5518:
5513:
5512:
5509:
5493:
5490:
5488:
5485:
5483:
5480:
5478:
5475:
5473:
5470:
5468:
5465:
5463:
5460:
5458:
5455:
5453:
5450:
5448:
5447:Lichen planus
5445:
5443:
5440:
5438:
5435:
5434:
5432:
5430:
5424:
5418:
5415:
5413:
5410:
5408:
5405:
5403:
5402:Onychomycosis
5400:
5399:
5397:
5395:
5391:
5385:
5382:
5380:
5377:
5375:
5372:
5370:
5367:
5365:
5364:Tinea capitis
5362:
5360:
5357:
5355:
5352:
5350:
5347:
5345:
5342:
5341:
5339:
5335:
5329:
5328:
5326:
5324:
5320:
5317:
5313:Miscellaneous
5311:
5297:
5294:
5292:
5289:
5287:
5284:
5282:
5278:
5275:
5274:
5272:
5268:
5254:
5251:
5249:
5246:
5245:
5243:
5239:
5233:
5230:
5228:
5225:
5224:
5222:
5218:
5215:
5211:Nonblanchable
5209:
5198:
5195:
5193:
5190:
5188:
5185:
5183:
5180:
5178:
5174:
5172:
5169:
5168:
5166:
5162:
5156:
5153:
5151:
5148:
5146:
5143:
5141:
5138:
5136:
5133:
5131:
5128:
5127:
5125:
5121:
5115:
5112:
5110:
5107:
5105:
5102:
5100:
5097:
5096:
5094:
5090:
5087:
5085:
5079:
5076:
5072:
5069:
5061:
5051:
5048:
5046:
5043:
5041:
5038:
5036:
5033:
5031:
5028:
5026:
5023:
5021:
5018:
5016:
5013:
5012:
5010:
5008:Hypopigmented
5006:
5000:
4997:
4995:
4992:
4990:
4987:
4985:
4982:
4980:
4977:
4975:
4972:
4970:
4967:
4965:
4962:
4960:
4959:Acne vulgaris
4957:
4956:
4954:
4952:
4948:
4942:
4939:
4937:
4934:
4932:
4929:
4927:
4924:
4922:
4919:
4917:
4914:
4912:
4911:Lichen planus
4909:
4907:
4904:
4902:
4899:
4898:
4896:
4894:
4890:
4884:
4881:
4879:
4876:
4874:
4871:
4869:
4866:
4864:
4861:
4859:
4856:
4854:
4851:
4849:
4846:
4844:
4843:Herpes zoster
4841:
4839:
4836:
4835:
4833:
4831:
4827:
4821:
4818:
4816:
4815:Parapsoriasis
4813:
4811:
4808:
4806:
4803:
4801:
4798:
4796:
4793:
4791:
4788:
4785:
4782:
4780:
4777:
4775:
4772:
4770:
4767:
4765:
4761:
4759:
4756:
4755:
4753:
4751:
4747:
4741:
4738:
4736:
4733:
4731:
4728:
4726:
4723:
4721:
4718:
4716:
4713:
4711:
4708:
4706:
4703:
4701:
4698:
4696:
4693:
4691:
4688:
4686:
4683:
4682:
4680:
4676:
4673:
4665:
4662:
4660:
4656:
4646:
4643:
4641:
4638:
4636:
4633:
4631:
4628:
4626:
4623:
4621:
4618:
4616:
4613:
4611:
4608:
4606:
4603:
4601:
4598:
4596:
4593:
4591:
4588:
4586:
4583:
4582:
4580:
4575:
4571:
4565:
4562:
4560:
4557:
4555:
4552:
4550:
4547:
4545:
4542:
4541:
4539:
4535:
4529:
4526:
4524:
4521:
4519:
4516:
4514:
4511:
4509:
4506:
4504:
4501:
4499:
4496:
4494:
4491:
4489:
4486:
4484:
4481:
4479:
4476:
4475:
4473:
4471:
4467:
4464:
4460:
4451:
4446:
4444:
4439:
4437:
4432:
4431:
4428:
4415:
4412:
4409:
4405:
4404:
4400:
4398:
4394:
4393:
4389:
4388:
4385:
4381:
4374:
4370:
4369:
4365:
4363:
4359:
4358:
4354:
4352:
4348:
4347:
4343:
4341:
4337:
4336:
4332:
4328:
4325:
4321:
4317:
4316:
4312:
4308:
4307:
4304:
4299:
4295:
4288:
4279:
4275:
4270:
4265:
4261:
4257:
4253:
4246:
4243:
4238:
4234:
4229:
4224:
4220:
4216:
4212:
4208:
4204:
4197:
4195:
4191:
4186:
4182:
4177:
4172:
4168:
4164:
4160:
4153:
4150:
4143:
4139:
4135:
4131:
4127:
4123:
4122:
4117:
4112:
4111:
4108:
4104:
4100:
4093:
4090:
4085:
4081:
4076:
4071:
4067:
4063:
4058:
4053:
4049:
4045:
4041:
4034:
4031:
4026:
4022:
4017:
4012:
4008:
4004:
4000:
3996:
3992:
3988:
3981:
3978:
3973:
3969:
3964:
3959:
3955:
3951:
3946:
3941:
3937:
3933:
3929:
3925:
3921:
3913:
3910:
3905:
3901:
3897:
3893:
3888:
3883:
3879:
3875:
3872:(11): 844–7.
3871:
3867:
3863:
3856:
3853:
3848:
3844:
3840:
3836:
3831:
3826:
3823:(7): 1432–6.
3822:
3818:
3814:
3807:
3804:
3799:
3795:
3790:
3785:
3781:
3777:
3773:
3769:
3765:
3758:
3755:
3750:
3746:
3742:
3738:
3734:
3730:
3722:
3719:
3706:
3700:
3697:
3684:
3680:
3674:
3671:
3666:
3662:
3658:
3654:
3649:
3644:
3640:
3636:
3632:
3628:
3624:
3620:
3616:
3609:
3607:
3603:
3598:
3594:
3590:
3586:
3582:
3578:
3574:
3570:
3566:
3562:
3558:
3551:
3548:
3543:
3539:
3534:
3529:
3525:
3521:
3517:
3513:
3509:
3502:
3499:
3496:
3492:
3491:
3484:
3481:
3476:
3472:
3467:
3462:
3458:
3454:
3450:
3446:
3442:
3435:
3432:
3427:
3423:
3418:
3413:
3409:
3405:
3401:
3397:
3393:
3386:
3383:
3378:
3374:
3369:
3364:
3360:
3356:
3352:
3348:
3344:
3340:
3336:
3329:
3326:
3321:
3317:
3313:
3309:
3305:
3301:
3296:
3291:
3286:
3281:
3277:
3273:
3269:
3262:
3259:
3254:
3250:
3246:
3242:
3237:
3232:
3229:(4): 556–73.
3228:
3224:
3220:
3213:
3211:
3207:
3202:
3198:
3193:
3188:
3184:
3180:
3176:
3172:
3168:
3161:
3159:
3157:
3155:
3153:
3149:
3144:
3140:
3135:
3130:
3126:
3122:
3118:
3114:
3110:
3106:
3102:
3095:
3093:
3089:
3084:
3080:
3076:
3072:
3068:
3064:
3060:
3056:
3052:
3048:
3044:
3037:
3034:
3029:
3025:
3021:
3017:
3012:
3007:
3003:
2999:
2995:
2988:
2985:
2981:
2977:
2973:
2968:
2963:
2959:
2955:
2951:
2947:
2943:
2939:
2935:
2928:
2925:
2921:
2917:
2911:
2907:
2903:
2899:
2895:
2888:
2885:
2873:
2869:
2865:
2859:
2856:
2851:
2847:
2842:
2837:
2833:
2829:
2825:
2821:
2817:
2813:
2809:
2802:
2800:
2796:
2791:
2787:
2782:
2777:
2773:
2769:
2765:
2761:
2757:
2750:
2747:
2742:
2738:
2733:
2728:
2724:
2720:
2716:
2712:
2708:
2701:
2698:
2693:
2689:
2684:
2679:
2674:
2669:
2666:(4): e10208.
2665:
2661:
2657:
2649:
2646:
2641:
2637:
2633:
2629:
2625:
2621:
2614:
2611:
2606:
2602:
2598:
2594:
2590:
2586:
2578:
2575:
2570:
2564:
2560:
2553:
2551:
2547:
2542:
2536:
2532:
2525:
2522:
2517:
2513:
2508:
2503:
2499:
2495:
2491:
2487:
2483:
2476:
2473:
2468:
2464:
2459:
2454:
2450:
2446:
2442:
2438:
2434:
2430:
2426:
2419:
2417:
2415:
2411:
2406:
2402:
2399:(6): 669–70.
2398:
2394:
2393:
2392:Haematologica
2388:
2381:
2378:
2373:
2369:
2365:
2361:
2357:
2353:
2349:
2345:
2341:
2334:
2331:
2326:
2320:
2316:
2309:
2306:
2301:
2297:
2292:
2287:
2283:
2279:
2275:
2271:
2267:
2260:
2257:
2252:
2248:
2244:
2240:
2235:
2230:
2226:
2222:
2217:
2212:
2208:
2204:
2200:
2196:
2192:
2185:
2182:
2177:
2173:
2169:
2165:
2161:
2157:
2152:
2151:2027.42/62592
2147:
2143:
2139:
2135:
2131:
2127:
2123:
2119:
2111:
2108:
2103:
2097:
2093:
2085:
2082:
2077:
2073:
2069:
2065:
2061:
2057:
2052:
2047:
2043:
2039:
2035:
2028:
2025:
2020:
2016:
2012:
2008:
2004:
2000:
1996:
1992:
1988:
1984:
1980:
1973:
1971:
1967:
1962:
1958:
1954:
1950:
1946:
1942:
1937:
1932:
1928:
1924:
1923:Haematologica
1920:
1913:
1910:
1905:
1901:
1897:
1893:
1889:
1885:
1881:
1877:
1873:
1869:
1865:
1858:
1855:
1850:
1849:
1844:
1838:
1835:
1830:
1829:
1824:
1818:
1815:
1810:
1806:
1802:
1798:
1794:
1790:
1786:
1782:
1778:
1771:
1768:
1763:
1759:
1755:
1751:
1744:
1742:
1740:
1736:
1731:
1727:
1723:
1719:
1715:
1711:
1707:
1703:
1699:
1695:
1691:
1684:
1682:
1680:
1676:
1671:
1667:
1663:
1659:
1654:
1649:
1645:
1641:
1637:
1633:
1629:
1625:
1621:
1614:
1611:
1606:
1602:
1598:
1594:
1590:
1586:
1581:
1576:
1572:
1568:
1564:
1557:
1554:
1549:
1548:
1543:
1537:
1535:
1531:
1526:
1522:
1518:
1512:
1509:
1504:
1500:
1496:
1492:
1488:
1484:
1480:
1476:
1472:
1468:
1464:
1457:
1455:
1453:
1451:
1447:
1442:
1436:
1432:
1431:
1423:
1420:
1415:
1411:
1407:
1403:
1399:
1395:
1391:
1387:
1383:
1376:
1374:
1370:
1365:
1361:
1357:
1353:
1349:
1345:
1340:
1335:
1331:
1327:
1323:
1316:
1314:
1310:
1305:
1301:
1297:
1293:
1289:
1285:
1280:
1275:
1271:
1267:
1263:
1256:
1254:
1252:
1250:
1248:
1246:
1244:
1242:
1240:
1238:
1236:
1234:
1232:
1230:
1228:
1226:
1222:
1211:on 2018-10-20
1210:
1206:
1205:
1200:
1194:
1192:
1190:
1188:
1186:
1184:
1182:
1178:
1173:
1169:
1165:
1161:
1157:
1153:
1149:
1145:
1141:
1137:
1133:
1126:
1124:
1122:
1120:
1118:
1116:
1114:
1110:
1103:
1101:
1099:
1093:
1090:
1086:
1082:
1078:
1077:New York City
1074:
1070:
1062:
1060:
1058:
1057:
1052:
1048:
1040:
1038:
1036:
1028:
1026:
1024:
1020:
1018:
1014:
1010:
1005:
1002:
997:
995:
991:
987:
983:
979:
974:
971:
967:
963:
959:
954:
952:
948:
944:
940:
936:
934:
933:B lymphocytes
930:
927:aimed at the
926:
922:
918:
914:
910:
906:
902:
898:
894:
890:
886:
882:
878:
874:
866:
864:
862:
861:
855:
849:
847:
843:
842:renal failure
839:
835:
831:
827:
823:
819:
815:
811:
803:
801:
799:
795:
790:
785:
781:
778:
776:
772:
767:
763:
759:
755:
751:
743:
741:
739:
732:
730:
728:
724:
720:
716:
712:
708:
704:
700:
696:
692:
688:
684:
676:
674:
667:
664:
659:
655:
652:
648:
644:
640:
636:
633:
629:
625:
621:
619:
616:
614:
611:
609:
606:
604:
601:
599:
596:
594:
590:
586:
582:
579:
575:
574:
572:
569:
567:
564:
562:
559:
557:
554:
553:
552:
546:
544:
543:per million.
540:
538:
534:
530:
521:
514:
512:
510:
506:
502:
498:
494:
490:
485:
483:
482:
477:
473:
469:
461:
459:
457:
453:
449:
445:
441:
437:
433:
429:
425:
421:
417:
409:
404:
400:
397:
394:
392:
389:
386:
384:
381:
380:
379:
377:
371:
369:
365:
361:
357:
353:
349:
345:
341:
337:
333:
329:
321:
319:
317:
312:
310:
306:
302:
298:
294:
289:
287:
283:
279:
275:
271:
268:
264:
260:
256:
252:
248:
243:
241:
237:
233:
229:
225:
221:
220:large bruises
217:
213:
209:
205:
201:
197:
196:blood vessels
193:
189:
185:
181:
172:
168:
164:
162:
158:
155:
151:
148:
144:
140:
136:
133:
131:
127:
123:
121:
117:
114:
110:
106:
102:
98:
94:
90:
87:
84:, confusion,
83:
79:
75:
74:Large bruises
72:
70:
66:
63:
60:
58:
54:
49:
45:
40:
36:
32:
27:
19:
6117:Hemarthrosis
5881:
5869:
5857:
5820:
5747:Pratt's sign
5559:coagulopathy
5417:Ingrown nail
4989:Dermatophyte
4984:Gonococcemia
4969:Folliculitis
4610:Neurofibroma
4578:subcutaneous
4401:
4390:
4366:
4355:
4344:
4329:
4309:
4262:(6): 393–7.
4259:
4255:
4245:
4213:(1): 11–18.
4210:
4206:
4166:
4162:
4152:
4128:(1): 89–93.
4125:
4119:
4106:
4102:
4092:
4047:
4043:
4033:
3998:
3994:
3980:
3927:
3923:
3912:
3869:
3865:
3855:
3820:
3816:
3806:
3771:
3767:
3757:
3732:
3729:N Engl J Med
3728:
3721:
3709:. Retrieved
3699:
3687:. Retrieved
3682:
3673:
3622:
3618:
3567:(1): 19–29.
3564:
3560:
3550:
3515:
3511:
3501:
3488:
3483:
3448:
3444:
3434:
3399:
3395:
3385:
3345:(1): 491–4.
3342:
3338:
3328:
3275:
3271:
3261:
3226:
3222:
3174:
3170:
3108:
3104:
3050:
3046:
3036:
3001:
2997:
2987:
2979:
2941:
2937:
2927:
2919:
2897:
2887:
2876:. Retrieved
2874:. 2023-04-26
2867:
2858:
2815:
2811:
2763:
2759:
2749:
2714:
2710:
2700:
2663:
2659:
2648:
2623:
2619:
2613:
2588:
2584:
2577:
2558:
2530:
2524:
2489:
2485:
2475:
2432:
2428:
2396:
2390:
2380:
2347:
2343:
2333:
2314:
2308:
2273:
2269:
2259:
2198:
2194:
2184:
2125:
2121:
2117:
2110:
2091:
2084:
2044:(1): 47–54.
2041:
2037:
2027:
1986:
1982:
1929:(2): 172–7.
1926:
1922:
1912:
1874:(1): 60–62.
1871:
1867:
1857:
1846:
1837:
1826:
1817:
1784:
1780:
1770:
1753:
1697:
1693:
1630:(1): 530–8.
1627:
1623:
1613:
1573:(1): 89–95.
1570:
1566:
1556:
1545:
1520:
1511:
1470:
1466:
1429:
1422:
1389:
1385:
1329:
1325:
1269:
1265:
1213:. Retrieved
1209:the original
1202:
1139:
1135:
1094:
1089:disseminated
1066:
1054:
1051:Black people
1044:
1041:Epidemiology
1032:
1021:
1017:schistocytes
1006:
998:
975:
966:caplacizumab
955:
947:caplacizumab
939:Caplacizumab
937:
931:molecule on
917:prednisolone
893:blood plasma
870:
859:
850:
846:neurological
807:
794:caplacizumab
786:
782:
779:
747:
736:
711:shear stress
680:
671:
651:interferon-α
585:chemotherapy
550:
541:
526:
509:shear stress
486:
479:
476:pathogenesis
465:
440:autoimmunity
436:schistocytes
413:
372:
325:
313:
290:
244:
206:, and often
183:
179:
178:
80:, weakness,
5870:aggregation
5752:Rose's sign
5722:Homans sign
5554:Coagulation
5472:Herpesvirus
5452:Leukoplakia
5277:Scleroderma
5164:Specialized
5092:Generalized
5067:involvement
4979:Candidiasis
4671:involvement
4645:Myxoid cyst
4493:Acrochordon
4392:MedlinePlus
4001:: 249–277.
3774:: 419–436.
3711:30 November
3689:30 November
3490:NCT05468320
3295:2434/829303
3125:10072/61440
2711:Ann Lab Med
2620:Transfusion
2435:(1): 1–19.
1392:(1): 4–14.
1056:post partum
994:splenectomy
982:vincristine
854:shiga-toxin
723:schistocyte
703:capillaries
695:endothelial
639:ciclosporin
628:clopidogrel
624:ticlopidine
613:Bevacizumab
603:Oxymorphone
593:mitomycin C
589:gemcitabine
505:coagulation
501:blood clots
424:coagulation
420:aggregation
284:, known as
192:blood clots
92:Usual onset
34:Other names
6138:Categories
6102:Hematocele
6067:Hemothorax
6042:Hemoptysis
5914:Hemophilia
5686:Thrombosis
5412:Paronychia
5253:Vasculitis
5177:Multiforme
5175:Erythema (
5130:Cellulitis
5081:Blanchable
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4820:Ichthyosis
4678:Eczematous
4590:Hemangioma
4368:DiseasesDB
4169:(2): 139.
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3985:Zheng XL,
3339:Hematology
2878:2023-08-23
1754:StatPearls
1624:Hematology
1215:2018-10-10
1104:References
913:prednisone
824:(HUS) and
738:Depression
699:arterioles
647:tacrolimus
608:Quetiapine
571:Medication
529:congenital
472:antibodies
462:Autoimmune
376:ecchymoses
299:, such as
263:antibodies
62:Hematology
6092:Hemobilia
6037:Epistaxis
5624:Platelets
5407:Psoriasis
5315:disorders
5270:Indurated
5171:Urticaria
5123:Localized
5065:epidermal
4848:Varicella
4758:Psoriasis
4669:epidermal
4635:Leiomyoma
4537:Pigmented
4470:Epidermal
4411:neuro/499
4408:emerg/579
4403:eMedicine
4142:0730-188X
4066:2077-0383
3987:Sadler JE
3954:1932-6203
3639:1538-7836
3581:1432-198X
3359:1520-4391
3304:0028-4793
3067:1098-9064
3020:0006-4971
2958:1528-0020
2832:0820-3946
2449:1865-3774
2364:1528-0020
2270:Blood Adv
2225:0027-8424
2160:1476-4687
2060:0094-6176
2019:204864996
2003:0028-4793
1945:1592-8721
1888:1077-4114
1801:0028-4793
1714:0028-4793
1644:1520-4391
1605:139098502
1589:1348-4214
1487:0025-7125
1406:0037-1963
1348:0006-4971
1288:0006-4971
1156:2056-676X
1085:hematuria
1029:Prognosis
1013:platelets
970:rituximab
962:rituximab
921:Rituximab
897:apheresis
867:Treatment
798:rituximab
754:petechiae
744:Diagnosis
668:infection
643:mitomycin
632:prasugrel
618:Sunitinib
578:acyclovir
566:Pregnancy
547:Secondary
497:platelets
364:petechiae
340:headaches
336:confusion
305:rituximab
282:inherited
274:multimers
259:pregnancy
236:confusion
170:Frequency
161:Prognosis
146:Treatment
113:pregnancy
103:Unknown,
95:Adulthood
57:Specialty
6083:abdomen
6006:Petechia
6001:Hematoma
5991:Bleeding
5858:adhesion
5784:By cause
5777:Bleeding
5681:Thrombus
5580:By cause
5573:Clotting
5543:clotting
5539:bleeding
5482:Syphilis
5429:membrane
5084:Erythema
5020:Vitiligo
4974:Impetigo
4951:Pustular
4916:Miliaria
4764:Corporis
4615:Xanthoma
4564:Melanoma
4544:Freckles
4414:med/2265
4326:M31.110)
4237:18574040
4185:71943329
4163:Medicine
4109:: 21–24.
4084:33540569
4025:18215115
3989:(2008).
3972:27383202
3924:PLOS ONE
3904:23118040
3896:20799358
3847:24914279
3839:15978100
3798:16409158
3749:38692292
3665:40493167
3657:28662310
3597:22209831
3589:18574602
3542:32914526
3475:32757435
3426:33280030
3377:36485149
3320:19482364
3312:26863353
3253:38774829
3245:12588343
3201:25573992
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3075:20865636
3028:24599547
2976:23847193
2850:27754896
2790:32914582
2741:37080750
2732:10151285
2692:20436664
2660:PLoS One
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2605:28259520
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2467:20058209
2405:11418383
2372:10381511
2300:38935915
2291:11375255
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2243:12181489
2168:11586351
2076:13963783
2068:22314603
2011:31644845
1961:17777111
1953:18245649
1896:28678087
1848:Orphanet
1762:28613472
1730:40055198
1722:12192020
1670:54564230
1662:30504354
1597:15969259
1503:24123004
1495:28189178
1414:14727254
1364:26844964
1356:20686117
1296:28416507
1172:11960153
1164:28382967
1098:protease
949:without
895:through
771:emergent
769:initial
733:Recovery
683:ADAMTS13
649:/FK506,
583:Certain
533:ADAMTS13
468:ADAMTS13
448:ADAMTS13
444:acquired
352:jaundice
344:Seizures
270:ADAMTS13
253:such as
240:headache
228:weakness
86:headache
69:Symptoms
6052:Hyphema
6027:By site
6011:Purpura
5705:By site
5281:morphea
5241:Papular
5220:Macular
5213:Purpura
5197:Ab igne
5182:Migrans
5135:Abscess
5063:Without
5045:Leprosy
4964:Rosacea
4901:Scabies
4893:Papular
4762:Tinea (
4750:Scaling
4554:Melasma
4549:Lentigo
4462:Growths
4362:D011697
4278:2062330
4228:2435681
4075:7867179
4016:2582586
3963:4934773
3932:Bibcode
3887:3420337
3789:2426955
3648:5630501
3533:8091490
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2130:Bibcode
1904:3470460
1809:9828253
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1071:at the
1063:History
943:placebo
860:E. coli
758:purpura
707:thrombi
598:Quinine
515:Genetic
432:rupture
186:) is a
137:(HUS),
6113:joint
6063:torso
5996:Bruise
5919:A/VIII
5427:Mucous
5323:Ulcers
4784:Faciei
4779:Manuum
4769:Cruris
4659:Rashes
4605:Lipoma
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4397:000552
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410:Causes
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267:enzyme
257:, and
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4207:Blood
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