72:
645:
276:
822:
48:
766:
2802:
2755:
2230:
477:(after Dr Thomas Ham, who described the test in 1937) was performed for confirmation. The Ham test involves placing red blood cells in mild acid; a positive result (increased RBC fragility) indicates PNH or Congenital dyserythropoietic anemia. This is now an obsolete test for diagnosing PNH due to its low sensitivity and specificity.
284:
540:
There are several groups where screening for PNH should be undertaken. These include patients with unexplained thrombosis who are young, have thrombosis in an unusual site (e.g. intra-abdominal veins, cerebral veins, dermal veins), have any evidence of hemolysis (e.g. a raised LDH), or have a low red
164:
and improves quality of life for those affected by PNH. Eculizumab dramatically alters the natural course of PNH, reducing symptoms and disease complications as well as improving survival to the extent that it may be equivalent to that of the general population. Eculizumab costs at least US$ 440,000
732:
inhibitor. It binds to the complement C5 protein and inhibits activation of the complement system, a part of the body's immune system. This binding prevents the breakdown of red blood cells in the bloodstream (intravascular hemolysis) in people with paroxysmal nocturnal hemoglobinuria and atypical
1621:
Röth, Alexander; Nishimura, Jun-ichi; Nagy, Zsolt; Gaà l-Weisinger, Julia; Panse, Jens; Yoon, Sung-Soo; Egyed, Miklos; Ichikawa, Satoshi; Ito, Yoshikazu; Kim, Jin Seok; Ninomiya, Haruhiko; Schrezenmeier, Hubert; Sica, Simona; Usuki, Kensuke; Sicre de
Fontbrune, Flore; Soret, Juliette; Sostelly,
736:
The most frequently reported adverse reactions for people with paroxysmal nocturnal hemoglobinuria include headache, nasopharyngitis (common cold), back pain and nausea The most frequently reported adverse reactions for people with atypical hemolytic uremic syndrome include headache, diarrhea,
185:. As the urine is more concentrated in the morning, this is when the color is most pronounced. This phenomenon mainly occurs in those who have the primary form of PNH, who will notice this at some point in their disease course. The remainder mainly experience the symptoms of anemia, such as
566:, this suppresses the production of PNH cells by the bone marrow, and indirectly the severity of the hemolysis. Iron deficiency develops with time, due to losses in urine, and may have to be treated if present. Iron therapy can result in more hemolysis as more PNH cells are produced.
1878:
574:
PNH is a chronic condition. In patients with only a small clone and few problems, monitoring of the flow cytometry every six months gives information on the severity and risk of potential complications. Given the high risk of thrombosis in PNH, preventive treatment with
326:, and, without these anchors, the cells are more easily targeted by the complement proteins. Although red blood cells, white blood cells, and platelets are targeted by complement, red blood cells are particularly vulnerable to lysis. The complement system is part of the
2323:
400:
and accumulation of carbon dioxide in the blood during sleep. This hypothesis has been questioned by researchers who note that not all those with PNH have increased hemolysis during sleep, so it is uncertain how important a role sleep actually plays in this disease.
291:
All cells have proteins attached to their membranes, often serving as a mode of communication or signaling between the cell and the surrounding environment. These signaling proteins are physically attached to the cell membrane in various ways, commonly anchored by
1622:
Alexandre; Higginson, James; Dieckmann, Andreas; Gentile, Brittany; Anzures-Cabrera, Judith; Shinomiya, Kenji; Jordan, Gregor; Biedzka-Sarek, Marta; Klughammer, Barbara; Jahreis, Angelika; Bucher, Christoph; Peffault de Latour, RĂ©gis (19 March 2020).
3050:
3035:
1870:
2040:
500:. Based on the levels of these cell proteins, erythrocytes may be classified as type I, II, or III PNH cells. Type I cells have normal levels of CD55 and CD59; type II have reduced levels; and type III have absent levels. The
2315:
315:), only one active copy of the gene for PIGA is present in each cell regardless of sex. A mutation in the PIGA gene can lead to the absence of GPI anchors expressed on the cell membrane. When this mutation occurs in a
2248:
2357:
3696:
901:
25% of female cases of PNH are discovered during pregnancy. This group has a high rate of thrombosis, and the risk of death of both mother and child are significantly increased (20% and 8% respectively).
504:(FLAER) test is being used more frequently to diagnose PNH. FLAER binds selectively to the glycophosphatidylinositol anchor and is more accurate in demonstrating a deficit than simply for CD59 or CD55.
2065:
Rother RP, Rollins SA, Mojcik CF, Brodsky RA, Bell L (November 2007). "Discovery and development of the complement inhibitor eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria".
886:
PNH is rare, with an annual rate of 1-2 cases per million. The prognosis without disease-modifying treatment is 10–20 years. Many cases develop in people who have previously been diagnosed with
618:
Crovalimab was approved for use in China in
February 2024, in Japan in April 2024, in the United States in June 2024 and in the European Union in August 2024. It was developed and is marketed by
2215:
4121:
2740:
2032:
381:(Viagra), which improves the effect of nitric oxide on muscle cells. There is a suspicion that chronic hemolysis causing chronically depleted nitric oxide may lead to the development of
720:, but does not appear to affect the risk of death. Eculizumab was the first medication approved for each of its uses, and its approval was granted based on small trials. It is given by
223:
Forty percent of people with PNH develop thrombosis at some point in their illness. This is the main cause of severe complications and death in PNH. These may develop in common sites (
3689:
2316:"Novartis receives FDA approval for Fabhalta (iptacopan), offering superior hemoglobin improvement in the absence of transfusions as the first oral monotherapy for adults with PNH"
139:
or GPI) leading to the absence of protective exterior surface proteins that normally attach via a GPI anchor. It may develop on its own ("primary PNH") or in the context of other
737:
hypertension, upper respiratory infection, abdominal pain, vomiting, nasopharyngitis, anemia, cough, swelling of lower legs or hands, nausea, urinary tract infections and fever
124:(a blood clot) is caused by both the absence of GPI-anchored complement regulatory proteins (CD55 and CD59) on PNH platelets and the excessive consumption of nitric oxide (NO).
2255:
1709:
396:
Historically, the role of sleep and night in this disease (the "nocturnal" component of the name) has been attributed to acidification of the blood at night due to relative
4186:
2431:
2293:
1945:
1810:
3682:
2345:
671:
The most common side effects include fever, headache, increased levels of liver enzymes (a sign of possible liver problems) and pain in the extremities (arms and legs).
4114:
4212:
1752:
1735:
1483:
Ham TH (1937). "Chronic haemolytic anaemia with paroxysmal nocturnal haemoglobinuria: study of the mechanism of haemolysis in relation to acid-base equilibrium".
471:, in which a patient's red blood cells are placed in low-ionic-strength solution and observed for hemolysis, was used for screening. If this was positive, the
3303:
3508:
582:
Episodes of thrombosis are treated as they would in other patients, but, given that PNH is a persisting underlying cause, it is likely that treatment with
1912:
1777:
4107:
3914:
3762:
3659:
3161:
1679:
3951:
3611:
3433:
2732:
2207:
3503:
3443:
4078:
150:
147:("secondary PNH"). Only a minority of affected people have the telltale red urine in the morning that originally gave the condition its name.
1860:
Text was copied from this source which is copyright
European Medicines Agency. Reproduction is authorized provided the source is acknowledged.
3780:
1871:"Voydeya (danicopan) granted first-ever regulatory approval in Japan for adults with PNH to be used in combination with C5 inhibitor therapy"
1057:
673:
Danicopan was approved for medical use in Japan in
January 2024, in the United States in March 2024, and in the European Union in April 2024.
1997:
MartĂ-Carvajal AJ, Anand V, Cardona AF, SolĂ I (October 2014). "Eculizumab for treating patients with paroxysmal nocturnal hemoglobinuria".
1312:
MartĂ-Carvajal AJ, Anand V, Cardona AF, SolĂ I (October 2014). "Eculizumab for treating patients with paroxysmal nocturnal hemoglobinuria".
863:(red blood cells unable to carry enough oxygen to tissues), blood clots, and impaired bone marrow function (not making enough blood cells).
3320:
108:, which normally inhibits such immune reactions. Since the complement cascade attacks the red blood cells within the blood vessels of the
3065:
2464:
1975:
1847:
464:
counts may be seen at this. In this case, anemia may be caused by insufficient red blood cell production in addition to the hemolysis.
4217:
701:
2680:"Pegcetacoplan treatment for geographic atrophy due to age-related macular degeneration: a plain language summary of the FILLY study"
3484:
3455:
3387:
4018:
2033:"FDA approves first treatment for neuromyelitis optica spectrum disorder, a rare autoimmune disease of the central nervous system"
1701:
300:(GPI). PNH occurs as a result of a defect in the assembling of these glycolipid-protein structures on the surface of blood cells.
4134:
3935:
3846:
3515:
501:
2652:
2421:
2285:
1937:
1802:
1595:
4088:
3958:
3830:
3448:
3154:
3494:
656:, sold under the brand name Voydeya, is a medication used for the treatment of paroxysmal nocturnal hemoglobinuria. It is a
541:
blood cell, white blood cell, or platelet count. Those who have a diagnosis of aplastic anemia should be screened annually.
3542:
2736:
1378:"The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease"
799:(FDA) for the treatment of adults with paroxysmal nocturnal hemoglobinuria in December 2023. The FDA considers it to be a
697:
562:) can decrease the severity of hemolytic crises. Transfusion therapy may be needed; in addition to correcting significant
304:
3825:
116:
hemolytic anemia. There is ongoing research into other key features of the disease, such as the high incidence of venous
3399:
2781:
2388:
2351:
2036:
1906:
1771:
870:
796:
297:
3551:
3244:
2787:
2394:
4155:
4099:
3938:
3790:
3757:
3425:
3289:
322:
Several of the proteins that anchor to GPI on the cell membrane are used to protect the cell from destruction by the
4130:
3489:
3315:
1672:"Crovalimab Approved in China as the First Country, for the Treatment of Paroxysmal Nocturnal Hemoglobinuria (PNH)"
260:
3904:
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3799:
3734:
3416:
3406:
3394:
3147:
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874:
800:
339:
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when these clots break off and enter the lungs), but in PNH blood clots may also form in more unusual sites: the
136:
105:
3973:
3721:
3382:
3076:
2981:
Enneking J (1928). "Eine neue form intermittierender haemoglobinurie (Haemoglobinuria paroxysmalis nocturia)".
1900:
1765:
887:
445:
252:
248:
1671:
1347:
918:, 1852–1915) during a lecture in 1881, later published in 1882. Later comprehensive descriptions were made by
3674:
1286:
922:
and
Alessio Nazari in 1911, with further elaborations by Marchiafava in 1928 and Ferdinando Micheli in 1931.
4073:
4058:
4054:
3587:
3438:
744:
351:
335:
316:
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in men; this occurs mainly when the breakdown of red blood cells is rapid, and is attributable to spasm of
4207:
4165:
3578:
3239:
866:
613:
382:
355:
244:
2110:"FDA report: eculizumab (Soliris) for the treatment of patients with paroxysmal nocturnal hemoglobinuria"
1417:
4048:
4028:
3749:
3558:
3325:
865:
Pegcetacoplan is the first treatment for paroxysmal nocturnal hemoglobinuria that binds to and inhibits
440:
to replace the destroyed cells) if there is no concurrent problem with production of red cells (such as
421:
2606:
Xu B (July 2023). "Novel targeted C3 inhibitor pegcetacoplan for paroxysmal nocturnal hemoglobinuria".
2157:
Keating GM (December 2013). "Eculizumab: a review of its use in atypical haemolytic uraemic syndrome".
4068:
3772:
3568:
3563:
3371:
3257:
721:
709:
327:
236:
224:
217:
213:
97:
1553:"Primary prophylaxis with warfarin prevents thrombosis in paroxysmal nocturnal hemoglobinuria (PNH)"
377:. This theory is supported by the fact that these symptoms improve on administration of nitrates or
71:
17:
3617:
3362:
3170:
3054:
782:
725:
609:
256:
154:
579:
decreases the risk of thrombosis in those with a large clone (50% of white blood cells type III).
307:(PIGA), one of several enzymes needed to make GPI. The gene that codes for PIGA is located on the
3920:
3795:
3649:
3622:
3227:
2998:
2889:
2709:
2631:
2570:
2527:
2182:
2139:
2090:
1015:
919:
838:
468:
425:
228:
165:
for a single year of treatment and has been reported as one of the world's most expensive drugs.
109:
2907:
Marchiafava E, Nazari A (1911). "Nuovo contributo allo studio degli itteri cronici emolitici".
385:(increased pressure in the blood vessels supplying the lung), which in turn puts strain on the
3889:
3880:
3865:
3709:
3654:
3644:
3603:
3598:
3593:
3534:
3366:
3355:
3345:
3279:
3262:
3253:
3235:
3197:
3116:
3087:
2850:
2701:
2623:
2588:
2519:
2174:
2131:
2082:
2014:
1653:
1574:
1533:
1465:
1399:
1329:
1244:
1195:
1139:
1053:
1007:
972:
842:
729:
717:
705:
657:
493:
323:
161:
93:
60:
2456:
3987:
3968:
3899:
3713:
3705:
3350:
3335:
3274:
2990:
2881:
2840:
2832:
2691:
2615:
2578:
2562:
2509:
2499:
2166:
2121:
2074:
2006:
1967:
1839:
1643:
1635:
1564:
1523:
1492:
1455:
1389:
1321:
1234:
1226:
1185:
1177:
1129:
1121:
1108:
Parker C, Omine M, Richards S, Nishimura J, Bessler M, Ware R, et al. (December 2005).
999:
962:
740:
457:
413:
362:
89:
1263:"Alexion Pharmaceuticals ordered to lower price of $ 500K a year drug in Canada | CBC News"
890:. The fact that PNH develops in MDS also explains why there appears to be a higher rate of
4010:
3529:
2558:
895:
497:
441:
397:
178:
153:
is the only cure, but has significant rates of additional medical problems and death. The
144:
3127:
1262:
2969:
2514:
2487:
1032:
Brodsky, R. A. (2014a). Paroxysmal nocturnal hemoglobinuria. Blood, 124(18), 2804–2811.
4159:
4063:
3963:
3785:
3546:
3469:
3377:
3174:
2845:
2820:
2583:
2546:
1648:
1623:
1348:"Paroxysmal Nocturnal Hemoglobinuria - NORD (National Organization for Rare Disorders)"
1239:
1214:
1190:
1165:
1134:
1109:
925:
The Dutch physician
Enneking coined the term "paroxysmal nocturnal hemoglobinuria" (or
853:
555:
481:
312:
201:
182:
174:
101:
3059:
869:. Pegcetacoplan was approved for medical use in the United States in May 2021. The US
275:
4201:
2893:
2836:
2806:
2759:
2713:
2635:
2531:
2234:
1460:
1443:
830:
814:
665:
660:
inhibitor which reversibly binds to factor D to prevent alternative pathway-mediated
456:. If the PNH occurs in the setting of known (or suspected) aplastic anemia, abnormal
390:
374:
343:
132:
3002:
2186:
2143:
2126:
2109:
2094:
1444:"Historical aspects of paroxysmal nocturnal haemoglobinuria: 'defining the disease'"
1019:
859:
Paroxysmal nocturnal hemoglobinuria is characterized by red blood cell destruction,
311:. As males have only a single X chromosome and, in females, one is silenced through
4138:
4039:
3851:
3189:
2010:
1325:
644:
612:
used for the treatment of people with paroxysmal nocturnal hemoglobinuria. It is a
559:
433:
370:
331:
308:
232:
2108:
Dmytrijuk A, Robie-Suh K, Cohen MH, Rieves D, Weiss K, Pazdur R (September 2008).
47:
3081:
1528:
1511:
1181:
1003:
967:
950:
586:
or similar drugs needs to be continued long-term after an episode of thrombosis.
3340:
3298:
3111:
1496:
821:
437:
429:
293:
240:
209:
194:
140:
2964:
2619:
2504:
1624:"The complement C5 inhibitor crovalimab in paroxysmal nocturnal hemoglobinuria"
1569:
1552:
1125:
1033:
4044:
4005:
3309:
3139:
3092:
3044:
2945:
Micheli F (1931). "Uno caso di anemia emolitica con emosiderinuria perpetua".
2566:
2170:
1230:
915:
834:
778:
693:
684:
605:
596:
417:
409:
378:
366:
157:
121:
117:
88:) is a rare, acquired, life-threatening disease of the blood characterized by
65:
2574:
2547:"Safety and efficacy of pegcetacoplan in paroxysmal nocturnal hemoglobinuria"
1639:
1394:
1377:
781:
used for the treatment of paroxysmal nocturnal hemoglobinuria (PNH). It is a
3122:
2772:
2379:
774:
758:
713:
661:
653:
637:
623:
449:
319:
in the bone marrow, all of the cells it produces will also have the defect.
205:
2885:
2854:
2705:
2627:
2592:
2523:
2178:
2135:
2086:
2018:
1657:
1578:
1537:
1469:
1403:
1333:
1248:
1199:
1143:
1011:
976:
848:
The most common side effects include injection-site reactions, infections,
365:, erectile dysfunction, and abdominal pain are attributed to the fact that
330:
and has a variety of functions, from destroying invading microorganisms by
2696:
2679:
712:. In people with paroxysmal nocturnal hemoglobinuria, it reduces both the
4147:
3995:
3730:
2426:
891:
849:
790:
786:
583:
576:
473:
461:
453:
3027:
2994:
2733:"FDA approves new treatment for adults with serious rare blood disease"
911:
190:
186:
2926:
Marchiafava E (1928). "Anemia emolitica con emosiderinuria perpetua".
1371:
1369:
765:
3870:
3739:
3218:
3039:
2208:"FDA Approves First Interchangeable Biosimilar for Two Rare Diseases"
1287:"British watchdog wants U.S. biotech Alexion to justify cost of drug"
860:
563:
264:
2869:
2773:
Advancing Health
Through Innovation: New Drug Therapy Approvals 2021
2078:
2416:
2414:
2412:
929:
in Latin) in 1928, which has since become the default description.
696:, sold under the brand name Soliris among others, is a recombinant
2647:
2645:
820:
764:
643:
619:
386:
338:. The main proteins that protect blood cells from destruction are
282:
274:
41:
Paroxysmal nocturnal haemoglobinuria, Marchiafava–Micheli syndrome
2805:
This article incorporates text from this source, which is in the
2758:
This article incorporates text from this source, which is in the
2488:"Pegcetacoplan: A Review in Paroxysmal Nocturnal Haemoglobinuria"
2233:
This article incorporates text from this source, which is in the
1110:"Diagnosis and management of paroxysmal nocturnal hemoglobinuria"
531:. PNH abnormalities on flow cytometry without signs of hemolysis.
519:. Evidence of PNH in the absence of another bone marrow disorder.
220:
due to depletion of nitric oxide by red cell breakdown products.
3070:
1803:"Voydeya- danicopan tablet, film coated; Voydeya- danicopan kit"
489:
485:
347:
175:
red discoloration of the urine due to the presence of hemoglobin
4103:
3678:
3143:
2280:
2278:
2276:
1932:
1930:
283:
2451:
2449:
1797:
1795:
1590:
1588:
910:
The first description of paroxysmal hemoglobinuria was by the
512:
PNH is classified by the context under which it is diagnosed:
112:, the red blood cell destruction (hemolysis) is considered an
2727:
2725:
2723:
2202:
2200:
2198:
2196:
990:
Parker CJ (May 2012). "Paroxysmal nocturnal hemoglobinuria".
856:, respiratory tract infection, viral infection, and fatigue.
428:(a breakdown product of hemoglobin), and decreased levels of
523:
PNH in the setting of another specified bone marrow disorder
2821:"Paroxysmal nocturnal hemoglobinuria from bench to bedside"
525:
such as aplastic anemia and myelodysplastic syndrome (MDS).
100:. This destructive process occurs due to deficiency of the
1834:
1832:
1830:
1828:
833:, sold under the brand name Empaveli, among others, is a
2060:
2058:
894:
in PNH, as MDS can sometimes transform into leukemia or
1702:"Japan Becomes Second Market to Approve Roche's Piasky"
1376:
Rother RP, Bell L, Hillmen P, Gladwin MT (April 2005).
1938:"Soliris- eculizumab injection, solution, concentrate"
837:
used to treat paroxysmal nocturnal hemoglobinuria and
1048:
Kumar Vinay; Abbas AK; Fausto N; Mitchell RN (2007).
3017:
4174:
4146:
4027:
4004:
3986:
3934:
3864:
3839:
3771:
3748:
3729:
3720:
3637:
3577:
3528:
3424:
3415:
3288:
3273:
3226:
3217:
3210:
3188:
3181:
3102:
3021:
1512:"Thrombosis in paroxysmal nocturnal hemoglobinuria"
700:used to treat paroxysmal nocturnal hemoglobinuria,
59:
37:
32:
4187:Hyperphosphatasia with mental retardation syndrome
1215:"Diseases of complement dysregulation-an overview"
448:(DAT, or direct Coombs' test) is negative, as the
412:in PNH show changes consistent with intravascular
2678:Liao DS, Metlapally R, Joshi P (September 2022).
1166:"How I treat paroxysmal nocturnal hemoglobinuria"
369:released during hemolysis binds with circulating
200:A small proportion of patients report attacks of
1052:(8th ed.). Saunders Elsevier. p. 652.
279:CD55 protein/Decay Accelerating Factor structure
131:(rather than inherited) intrinsic defect in the
1551:Hall C, Richards S, Hillmen P (November 2003).
127:PNH is the only hemolytic anemia caused by an
4115:
3690:
3155:
2422:"Empaveli- pegcetacoplan injection, solution"
1103:
1101:
1099:
1097:
1095:
1093:
1091:
1089:
8:
3304:Glucose-6-phosphate dehydrogenase deficiency
2653:"Syfovre- pegcetacoplan injection, solution"
1087:
1085:
1083:
1081:
1079:
1077:
1075:
1073:
1071:
1069:
1034:https://doi.org/10.1182/blood-2014-02-522128
1999:The Cochrane Database of Systematic Reviews
1314:The Cochrane Database of Systematic Reviews
777:, sold under the brand name Fabhalta, is a
303:The most common defective enzyme in PNH is
4122:
4108:
4100:
3915:Purine nucleoside phosphorylase deficiency
3763:Transient hypogammaglobulinemia of infancy
3745:
3726:
3697:
3683:
3675:
3660:Hereditary persistence of fetal hemoglobin
3421:
3285:
3223:
3214:
3185:
3162:
3148:
3140:
3018:
944:
942:
70:
46:
29:
3612:Mean corpuscular hemoglobin concentration
3434:Warm antibody autoimmune hemolytic anemia
2844:
2695:
2582:
2513:
2503:
2125:
1647:
1568:
1527:
1510:Hill A, Kelly RJ, Hillmen P (June 2013).
1459:
1393:
1238:
1189:
1159:
1157:
1155:
1153:
1133:
1043:
1041:
966:
608:, sold under the brand name Piasky, is a
1596:"Piasky- crovalimab injection, solution"
951:"PNH from mutations of another PIG gene"
342:(DAF/CD55), which disrupts formation of
1751:was invoked but never defined (see the
1734:was invoked but never defined (see the
938:
4079:Terminal complement pathway deficiency
2043:from the original on 14 September 2019
373:, a substance that is needed to relax
151:Allogeneic bone marrow transplantation
4213:Vascular-related cutaneous conditions
2965:StrĂĽbing-Marchiafava-Micheli syndrome
2467:from the original on 18 February 2023
2326:from the original on 12 December 2023
2296:from the original on 10 December 2023
1881:from the original on 24 February 2024
1850:from the original on 23 February 2024
1418:"Paroxysmal Nocturnal Hemoglobinuria"
927:haemoglobinuria paroxysmalis nocturna
7:
3321:triosephosphate isomerase deficiency
2790:from the original on 6 December 2022
2743:from the original on 17 October 2021
2397:from the original on 10 January 2024
2360:from the original on 21 January 2023
2249:An Outlook on Biosimilar Competition
2247:Bourgoin AF, Nuskey B (April 2015).
1978:from the original on 19 October 2019
1422:The Lecturio Medical Concept Library
1213:Wong EK, Kavanagh D (January 2018).
554:There is disagreement as to whether
436:(immature red cells released by the
350:(CD59/MIRL/MAC-IP), which binds the
267:, is more common in those with PNH.
18:Paroxysmal nocturnal haemoglobinuria
4182:Paroxysmal nocturnal hemoglobinuria
4084:Paroxysmal nocturnal hemoglobinuria
3476:paroxysmal nocturnal hemoglobinuria
3444:Donath–Landsteiner hemolytic anemia
1746:
1729:
82:Paroxysmal nocturnal hemoglobinuria
33:Paroxysmal nocturnal hemoglobinuria
2825:Clinical and Translational Science
2608:Clinical and Experimental Medicine
2551:Therapeutic Advances in Hematology
1915:from the original on 30 April 2024
1877:(Press release). 19 January 2024.
1813:from the original on 25 April 2024
1780:from the original on 30 April 2024
1712:from the original on 18 April 2024
1682:from the original on 18 April 2024
702:atypical hemolytic uremic syndrome
25:
3485:Microangiopathic hemolytic anemia
3456:Mixed autoimmune hemolytic anemia
2434:from the original on 13 July 2021
2212:U.S. Food and Drug Administration
1948:from the original on 9 April 2022
795:Iptacopan was approved by the US
334:to direct destabilization by the
3847:Common variable immunodeficiency
3516:Hemolytic disease of the newborn
2837:10.1111/j.1752-8062.2011.00262.x
2800:
2753:
2228:
2218:from the original on 30 May 2024
1747:Cite error: The named reference
1730:Cite error: The named reference
1461:10.1046/j.1365-2141.2002.03374.x
839:geographic atrophy of the retina
813:This section is an excerpt from
785:inhibitor that was developed by
757:This section is an excerpt from
743:, manufactured, and marketed by
683:This section is an excerpt from
636:This section is an excerpt from
595:This section is an excerpt from
502:fluorescein-labeled proaerolysin
287:CD59 protein/Protectin structure
4019:Idiopathic CD4+ lymphocytopenia
2819:Pu JJ, Brodsky RA (June 2011).
2380:New Drug Therapy Approvals 2023
2346:"Novel Drug Approvals for 2023"
2127:10.1634/theoncologist.2008-0086
1901:"Novel Drug Approvals for 2024"
1766:"Novel Drug Approvals for 2024"
4089:Complement receptor deficiency
3959:Adenosine deaminase deficiency
3449:Paroxysmal cold hemoglobinuria
2737:Food & Drug Administration
2011:10.1002/14651858.CD010340.pub2
1448:British Journal of Haematology
1326:10.1002/14651858.CD010340.pub2
714:destruction of red blood cells
298:glycosyl phosphatidylinositols
90:destruction of red blood cells
55:Intravascular hemolytic anemia
1:
4156:3-Methylglutaconic aciduria 2
2286:"Fabhalta- iptacopan capsule"
992:Current Opinion in Hematology
698:humanized monoclonal antibody
668:proteins on red blood cells.
305:phosphatidylinositol glycan A
120:. Research suggests that PNH
3400:Southeast Asian ovalocytosis
3388:Minkowski–Chauffard syndrome
2870:"Paroxysmale Hämoglobinurie"
2782:Food and Drug Administration
2389:Food and Drug Administration
2352:Food and Drug Administration
2037:Food and Drug Administration
1907:Food and Drug Administration
1772:Food and Drug Administration
1529:10.1182/blood-2012-09-311381
1182:10.1182/blood-2009-03-195966
1004:10.1097/MOH.0b013e328351c348
968:10.1182/blood-2013-06-508556
871:Food and Drug Administration
797:Food and Drug Administration
480:Today, the gold standard is
3758:X-linked agammaglobulinemia
1497:10.1056/NEJM193712022172307
1219:Seminars in Immunopathology
873:(FDA) considers it to be a
733:hemolytic uremic syndrome.
173:The classic sign of PNH is
4234:
3509:Drug-induced nonautoimmune
3490:Thrombotic microangiopathy
3316:pyruvate kinase deficiency
2620:10.1007/s10238-022-00830-3
2505:10.1007/s40265-022-01809-w
1570:10.1182/blood-2003-01-0009
1522:(25): 4985–96, quiz 5105.
1126:10.1182/blood-2005-04-1717
949:Luzzatto L (August 2013).
812:
756:
728:functioning as a terminal
682:
635:
594:
358:from binding to the cell.
261:Cerebral venous thrombosis
4218:Acquired hemolytic anemia
3495:Hemolytic–uremic syndrome
3407:Hereditary stomatocytosis
3395:Hereditary elliptocytosis
2567:10.1177/20406207221114673
2463:(EMA). 6 September 2022.
2461:European Medicines Agency
2171:10.1007/s40265-013-0147-7
1972:European Medicines Agency
1844:European Medicines Agency
1231:10.1007/s00281-017-0663-8
914:physician Paul StrĂĽbing (
875:first-in-class medication
801:first-in-class medication
474:Ham's acid hemolysis test
340:decay-accelerating factor
259:) and veins of the skin.
227:of the leg and resultant
137:glycophosphatidylinositol
54:
45:
3974:Bare lymphocyte syndrome
3826:Wiskott–Aldrich syndrome
3383:Hereditary spherocytosis
2545:Wong RS (28 July 2022).
2486:Heo YA (December 2022).
2393:(Report). January 2024.
2254:(Report). Archived from
1640:10.1182/blood.2019003399
1442:Parker CJ (April 2002).
1395:10.1001/jama.293.13.1653
1164:Brodsky RA (June 2009).
888:myelodysplastic syndrome
739:Eculizumab (Soliris) is
452:of PNH is not caused by
446:direct antiglobulin test
253:inferior mesenteric vein
4074:Complement 3 deficiency
4059:Complement 4 deficiency
4055:Complement 2 deficiency
3588:Mean corpuscular volume
3552:Diamond–Blackfan anemia
3504:Drug-induced autoimmune
3439:Cold agglutinin disease
3245:Plummer–Vinson syndrome
2786:(Report). 13 May 2022.
1050:Robbins Basic Pathology
745:Alexion Pharmaceuticals
352:membrane attack complex
336:membrane attack complex
317:hematopoietic stem cell
96:, a part of the body's
3240:Iron-deficiency anemia
2886:10.1055/s-0029-1196307
2035:(Press release). U.S.
1732:Crovalimab Piasky EPAR
825:
769:
648:
614:complement component 5
432:; there can be raised
383:pulmonary hypertension
288:
280:
263:, an uncommon form of
245:portal vein thrombosis
243:of the liver (causing
210:pain during swallowing
181:from the breakdown of
4049:Hereditary angioedema
3905:Ataxia–telangiectasia
3750:Hypogammaglobulinemia
3559:Pure red cell aplasia
3326:hexokinase deficiency
2697:10.2217/imt-2022-0078
2561:: 20406207221114673.
2039:(FDA). 27 June 2019.
1974:. 17 September 2018.
867:complement protein C3
841:. Pegcetacoplan is a
824:
768:
647:
460:counts and decreased
422:lactate dehydrogenase
286:
278:
206:difficulty swallowing
160:reduces the need for
4141:metabolism disorders
4069:Properdin deficiency
3890:Di George's syndrome
3773:Dysgammaglobulinemia
3564:Sideroblastic anemia
3372:Hemoglobin C disease
3258:Megaloblastic anemia
2874:Dtsch Med Wochenschr
2739:(FDA). 18 May 2021.
2067:Nature Biotechnology
1846:. 22 February 2024.
1749:Crovalimab Piasky PI
724:. It is a humanized
722:intravenous infusion
710:neuromyelitis optica
328:innate immune system
237:Budd-Chiari syndrome
225:deep vein thrombosis
214:erectile dysfunction
118:blood clot formation
98:innate immune system
3363:Sickle cell disease
2868:StrĂĽbing P (1882).
2356:. 6 December 2023.
2292:. 5 December 2023.
1678:. 8 February 2024.
783:complement factor B
726:monoclonal antibody
610:monoclonal antibody
257:mesenteric ischemia
191:shortness of breath
155:monoclonal antibody
3921:Hyper IgM syndrome
3831:Hyper-IgE syndrome
3796:Hyper IgM syndrome
3712:disorders causing
3650:Sulfhemoglobinemia
3103:External resources
2995:10.1007/BF01846778
2947:G Accad Med Torino
2659:. 23 February 2023
1676:chugai-pharm.co.jp
920:Ettore Marchiafava
826:
770:
664:and deposition of
649:
469:sucrose lysis test
467:Historically, the
289:
281:
229:pulmonary embolism
169:Signs and symptoms
162:blood transfusions
143:disorders such as
110:circulatory system
4195:
4194:
4097:
4096:
3982:
3981:
3881:thymic hypoplasia
3866:T cell deficiency
3860:
3859:
3672:
3671:
3668:
3667:
3655:Reticulocytopenia
3645:Methemoglobinemia
3633:
3632:
3524:
3523:
3464:
3463:
3263:Pernicious anemia
3206:
3205:
3198:Polycythemia vera
3137:
3136:
2989:(43): 2045–2047.
2880:: 1–3 and 17–21.
2498:(18): 1727–1735.
2430:. 13 April 2023.
2322:(Press release).
2165:(18): 2053–2066.
2073:(11): 1256–1264.
1944:. 20 April 2021.
1911:. 29 April 2024.
1059:978-1-4160-2973-1
718:blood transfusion
706:myasthenia gravis
324:complement system
94:complement system
79:
78:
27:Medical condition
16:(Redirected from
4225:
4135:lipid metabolism
4124:
4117:
4110:
4101:
3969:ZAP70 deficiency
3900:Nezelof syndrome
3746:
3727:
3714:immunodeficiency
3699:
3692:
3685:
3676:
3422:
3336:hemoglobinopathy
3286:
3224:
3215:
3186:
3164:
3157:
3150:
3141:
3019:
3007:
3006:
2978:
2972:
2961:
2955:
2954:
2942:
2936:
2935:
2923:
2917:
2916:
2904:
2898:
2897:
2865:
2859:
2858:
2848:
2816:
2810:
2804:
2803:
2799:
2797:
2795:
2777:
2769:
2763:
2757:
2756:
2752:
2750:
2748:
2729:
2718:
2717:
2699:
2690:(13): 995–1006.
2675:
2669:
2668:
2666:
2664:
2649:
2640:
2639:
2603:
2597:
2596:
2586:
2542:
2536:
2535:
2517:
2507:
2483:
2477:
2476:
2474:
2472:
2453:
2444:
2443:
2441:
2439:
2418:
2407:
2406:
2404:
2402:
2384:
2376:
2370:
2369:
2367:
2365:
2342:
2336:
2335:
2333:
2331:
2312:
2306:
2305:
2303:
2301:
2282:
2271:
2270:
2268:
2266:
2260:
2253:
2244:
2238:
2232:
2231:
2227:
2225:
2223:
2204:
2191:
2190:
2154:
2148:
2147:
2129:
2105:
2099:
2098:
2062:
2053:
2052:
2050:
2048:
2029:
2023:
2022:
2005:(10): CD010340.
1994:
1988:
1987:
1985:
1983:
1964:
1958:
1957:
1955:
1953:
1934:
1925:
1924:
1922:
1920:
1897:
1891:
1890:
1888:
1886:
1867:
1861:
1859:
1857:
1855:
1836:
1823:
1822:
1820:
1818:
1809:. 3 April 2024.
1799:
1790:
1789:
1787:
1785:
1776:. 21 June 2024.
1762:
1756:
1750:
1745:
1739:
1733:
1728:
1722:
1721:
1719:
1717:
1708:. 3 April 2024.
1698:
1692:
1691:
1689:
1687:
1668:
1662:
1661:
1651:
1618:
1612:
1611:
1609:
1607:
1592:
1583:
1582:
1572:
1548:
1542:
1541:
1531:
1507:
1501:
1500:
1480:
1474:
1473:
1463:
1439:
1433:
1432:
1430:
1428:
1414:
1408:
1407:
1397:
1373:
1364:
1363:
1361:
1359:
1344:
1338:
1337:
1320:(10): CD010340.
1309:
1303:
1302:
1300:
1298:
1283:
1277:
1276:
1274:
1273:
1259:
1253:
1252:
1242:
1210:
1204:
1203:
1193:
1161:
1148:
1147:
1137:
1120:(12): 3699–709.
1105:
1064:
1063:
1045:
1036:
1030:
1024:
1023:
987:
981:
980:
970:
946:
616:(C5) inhibitor.
458:white blood cell
426:raised bilirubin
414:hemolytic anemia
363:esophageal spasm
361:The symptoms of
104:surface protein
75:
74:
50:
30:
21:
4233:
4232:
4228:
4227:
4226:
4224:
4223:
4222:
4198:
4197:
4196:
4191:
4170:
4142:
4128:
4098:
4093:
4030:
4023:
4011:Lymphocytopenia
4009:
4000:
3978:
3954:
3941:
3930:
3886:hypoparathyroid
3868:
3856:
3835:
3767:
3737:
3716:
3703:
3673:
3664:
3629:
3573:
3532:
3520:
3460:
3411:
3277:
3269:
3202:
3177:
3175:red blood cells
3168:
3138:
3133:
3132:
3098:
3097:
3030:
3016:
3011:
3010:
2983:Klin Wochenschr
2980:
2979:
2975:
2962:
2958:
2944:
2943:
2939:
2925:
2924:
2920:
2906:
2905:
2901:
2867:
2866:
2862:
2818:
2817:
2813:
2801:
2793:
2791:
2775:
2771:
2770:
2766:
2754:
2746:
2744:
2731:
2730:
2721:
2677:
2676:
2672:
2662:
2660:
2651:
2650:
2643:
2605:
2604:
2600:
2559:SAGE Publishing
2544:
2543:
2539:
2485:
2484:
2480:
2470:
2468:
2457:"Aspaveli EPAR"
2455:
2454:
2447:
2437:
2435:
2420:
2419:
2410:
2400:
2398:
2382:
2378:
2377:
2373:
2363:
2361:
2344:
2343:
2339:
2329:
2327:
2314:
2313:
2309:
2299:
2297:
2284:
2283:
2274:
2264:
2262:
2258:
2251:
2246:
2245:
2241:
2229:
2221:
2219:
2214:. 28 May 2024.
2206:
2205:
2194:
2156:
2155:
2151:
2120:(9): 993–1000.
2107:
2106:
2102:
2079:10.1038/nbt1344
2064:
2063:
2056:
2046:
2044:
2031:
2030:
2026:
1996:
1995:
1991:
1981:
1979:
1966:
1965:
1961:
1951:
1949:
1936:
1935:
1928:
1918:
1916:
1899:
1898:
1894:
1884:
1882:
1869:
1868:
1864:
1853:
1851:
1838:
1837:
1826:
1816:
1814:
1801:
1800:
1793:
1783:
1781:
1764:
1763:
1759:
1748:
1742:
1731:
1725:
1715:
1713:
1700:
1699:
1695:
1685:
1683:
1670:
1669:
1665:
1634:(12): 912–920.
1620:
1619:
1615:
1605:
1603:
1594:
1593:
1586:
1563:(10): 3587–91.
1550:
1549:
1545:
1509:
1508:
1504:
1491:(23): 915–918.
1482:
1481:
1477:
1441:
1440:
1436:
1426:
1424:
1416:
1415:
1411:
1388:(13): 1653–62.
1375:
1374:
1367:
1357:
1355:
1346:
1345:
1341:
1311:
1310:
1306:
1296:
1294:
1293:. March 3, 2014
1285:
1284:
1280:
1271:
1269:
1261:
1260:
1256:
1212:
1211:
1207:
1163:
1162:
1151:
1107:
1106:
1067:
1060:
1047:
1046:
1039:
1031:
1027:
989:
988:
984:
961:(7): 1099–100.
948:
947:
940:
935:
908:
896:aplastic anemia
884:
879:
878:
818:
810:
805:
804:
762:
754:
749:
748:
688:
680:
675:
674:
641:
633:
628:
627:
600:
592:
572:
552:
547:
538:
529:Subclinical PNH
510:
498:red blood cells
442:iron deficiency
407:
398:hypoventilation
273:
271:Pathophysiology
183:red blood cells
171:
145:aplastic anemia
135:(deficiency of
69:
28:
23:
22:
15:
12:
11:
5:
4231:
4229:
4221:
4220:
4215:
4210:
4200:
4199:
4193:
4192:
4190:
4189:
4184:
4178:
4176:
4172:
4171:
4169:
4168:
4163:
4160:Barth syndrome
4152:
4150:
4144:
4143:
4129:
4127:
4126:
4119:
4112:
4104:
4095:
4094:
4092:
4091:
4086:
4081:
4076:
4071:
4066:
4064:MBL deficiency
4061:
4052:
4035:
4033:
4025:
4024:
4022:
4021:
4015:
4013:
4002:
4001:
3999:
3998:
3992:
3990:
3984:
3983:
3980:
3979:
3977:
3976:
3971:
3966:
3964:Omenn syndrome
3961:
3945:
3943:
3932:
3931:
3929:
3928:
3909:
3908:
3902:
3893:
3876:
3874:
3862:
3861:
3858:
3857:
3855:
3854:
3849:
3843:
3841:
3837:
3836:
3834:
3833:
3828:
3823:
3817:
3812:
3807:
3802:
3793:
3791:IgM deficiency
3788:
3786:IgG deficiency
3783:
3781:IgA deficiency
3777:
3775:
3769:
3768:
3766:
3765:
3760:
3754:
3752:
3743:
3724:
3718:
3717:
3704:
3702:
3701:
3694:
3687:
3679:
3670:
3669:
3666:
3665:
3663:
3662:
3657:
3652:
3647:
3641:
3639:
3635:
3634:
3631:
3630:
3628:
3627:
3626:
3625:
3620:
3608:
3607:
3606:
3601:
3596:
3583:
3581:
3575:
3574:
3572:
3571:
3566:
3561:
3554:
3549:
3547:Fanconi anemia
3540:
3538:
3526:
3525:
3522:
3521:
3519:
3518:
3512:
3511:
3506:
3500:
3499:
3498:
3497:
3487:
3481:
3480:
3479:
3478:
3465:
3462:
3461:
3459:
3458:
3453:
3452:
3451:
3441:
3436:
3430:
3428:
3419:
3413:
3412:
3410:
3409:
3404:
3403:
3402:
3392:
3391:
3390:
3374:
3369:
3360:
3359:
3358:
3353:
3348:
3331:
3330:
3329:
3328:
3323:
3318:
3306:
3294:
3292:
3283:
3271:
3270:
3268:
3267:
3266:
3265:
3250:
3249:
3248:
3247:
3232:
3230:
3221:
3212:
3208:
3207:
3204:
3203:
3201:
3200:
3194:
3192:
3183:
3179:
3178:
3169:
3167:
3166:
3159:
3152:
3144:
3135:
3134:
3131:
3130:
3128:article/207468
3119:
3107:
3106:
3104:
3100:
3099:
3096:
3095:
3084:
3073:
3062:
3047:
3031:
3026:
3025:
3023:
3022:Classification
3015:
3014:External links
3012:
3009:
3008:
2973:
2956:
2949:(in Italian).
2937:
2930:(in Italian).
2918:
2911:(in Italian).
2899:
2860:
2811:
2764:
2719:
2670:
2641:
2614:(3): 717–726.
2598:
2537:
2478:
2445:
2408:
2371:
2337:
2307:
2272:
2261:on 13 May 2015
2239:
2192:
2149:
2114:The Oncologist
2100:
2054:
2024:
1989:
1968:"Soliris EPAR"
1959:
1926:
1892:
1862:
1840:"Voydeya EPAR"
1824:
1791:
1757:
1740:
1723:
1693:
1663:
1613:
1602:. 28 June 2024
1584:
1543:
1502:
1475:
1434:
1409:
1365:
1339:
1304:
1278:
1254:
1205:
1176:(26): 6522–7.
1149:
1065:
1058:
1037:
1025:
982:
937:
936:
934:
931:
907:
904:
883:
880:
854:abdominal pain
819:
811:
809:
806:
789:. It is taken
763:
755:
753:
750:
704:, generalized
689:
681:
679:
676:
642:
634:
632:
629:
601:
593:
591:
588:
571:
568:
551:
548:
546:
543:
537:
534:
533:
532:
526:
520:
509:
508:Classification
506:
482:flow cytometry
406:
403:
313:X-inactivation
272:
269:
202:abdominal pain
170:
167:
102:red blood cell
77:
76:
63:
57:
56:
52:
51:
43:
42:
39:
35:
34:
26:
24:
14:
13:
10:
9:
6:
4:
3:
2:
4230:
4219:
4216:
4214:
4211:
4209:
4208:Rare diseases
4206:
4205:
4203:
4188:
4185:
4183:
4180:
4179:
4177:
4173:
4167:
4164:
4161:
4157:
4154:
4153:
4151:
4149:
4145:
4140:
4136:
4132:
4125:
4120:
4118:
4113:
4111:
4106:
4105:
4102:
4090:
4087:
4085:
4082:
4080:
4077:
4075:
4072:
4070:
4067:
4065:
4062:
4060:
4056:
4053:
4050:
4046:
4042:
4041:
4037:
4036:
4034:
4032:
4026:
4020:
4017:
4016:
4014:
4012:
4007:
4003:
3997:
3994:
3993:
3991:
3989:
3985:
3975:
3972:
3970:
3967:
3965:
3962:
3960:
3957:
3953:
3950:
3947:
3946:
3944:
3940:
3937:
3933:
3926:
3922:
3919:
3918:
3917:
3916:
3913:
3906:
3903:
3901:
3897:
3896:euparathyroid
3894:
3891:
3887:
3884:
3882:
3878:
3877:
3875:
3872:
3867:
3863:
3853:
3850:
3848:
3845:
3844:
3842:
3838:
3832:
3829:
3827:
3824:
3821:
3818:
3816:
3813:
3811:
3808:
3806:
3803:
3801:
3797:
3794:
3792:
3789:
3787:
3784:
3782:
3779:
3778:
3776:
3774:
3770:
3764:
3761:
3759:
3756:
3755:
3753:
3751:
3747:
3744:
3741:
3736:
3732:
3728:
3725:
3723:
3719:
3715:
3711:
3707:
3700:
3695:
3693:
3688:
3686:
3681:
3680:
3677:
3661:
3658:
3656:
3653:
3651:
3648:
3646:
3643:
3642:
3640:
3636:
3624:
3621:
3619:
3616:
3615:
3614:
3613:
3609:
3605:
3602:
3600:
3597:
3595:
3592:
3591:
3590:
3589:
3585:
3584:
3582:
3580:
3576:
3570:
3569:Myelophthisic
3567:
3565:
3562:
3560:
3556:
3555:
3553:
3550:
3548:
3544:
3541:
3539:
3536:
3531:
3527:
3517:
3514:
3513:
3510:
3507:
3505:
3502:
3501:
3496:
3493:
3492:
3491:
3488:
3486:
3483:
3482:
3477:
3474:
3473:
3472:
3471:
3467:
3466:
3457:
3454:
3450:
3447:
3446:
3445:
3442:
3440:
3437:
3435:
3432:
3431:
3429:
3427:
3423:
3420:
3418:
3414:
3408:
3405:
3401:
3398:
3397:
3396:
3393:
3389:
3386:
3385:
3384:
3381:
3379:
3375:
3373:
3370:
3368:
3364:
3361:
3357:
3354:
3352:
3349:
3347:
3344:
3343:
3342:
3339:
3337:
3333:
3332:
3327:
3324:
3322:
3319:
3317:
3314:
3313:
3312:
3311:
3307:
3305:
3302:
3300:
3296:
3295:
3293:
3291:
3287:
3284:
3281:
3276:
3272:
3264:
3261:
3260:
3259:
3255:
3252:
3251:
3246:
3243:
3242:
3241:
3237:
3234:
3233:
3231:
3229:
3225:
3222:
3220:
3216:
3213:
3209:
3199:
3196:
3195:
3193:
3191:
3187:
3184:
3180:
3176:
3172:
3165:
3160:
3158:
3153:
3151:
3146:
3145:
3142:
3129:
3125:
3124:
3120:
3118:
3114:
3113:
3109:
3108:
3105:
3101:
3094:
3090:
3089:
3085:
3083:
3079:
3078:
3074:
3072:
3068:
3067:
3063:
3061:
3057:
3056:
3052:
3048:
3046:
3042:
3041:
3037:
3033:
3032:
3029:
3024:
3020:
3013:
3004:
3000:
2996:
2992:
2988:
2985:(in German).
2984:
2977:
2974:
2971:
2970:Who Named It?
2967:
2966:
2960:
2957:
2952:
2948:
2941:
2938:
2933:
2929:
2922:
2919:
2914:
2910:
2903:
2900:
2895:
2891:
2887:
2883:
2879:
2876:(in German).
2875:
2871:
2864:
2861:
2856:
2852:
2847:
2842:
2838:
2834:
2831:(3): 219–24.
2830:
2826:
2822:
2815:
2812:
2808:
2807:public domain
2789:
2785:
2783:
2774:
2768:
2765:
2761:
2760:public domain
2742:
2738:
2734:
2728:
2726:
2724:
2720:
2715:
2711:
2707:
2703:
2698:
2693:
2689:
2685:
2684:Immunotherapy
2681:
2674:
2671:
2658:
2654:
2648:
2646:
2642:
2637:
2633:
2629:
2625:
2621:
2617:
2613:
2609:
2602:
2599:
2594:
2590:
2585:
2580:
2576:
2572:
2568:
2564:
2560:
2556:
2552:
2548:
2541:
2538:
2533:
2529:
2525:
2521:
2516:
2511:
2506:
2501:
2497:
2493:
2489:
2482:
2479:
2466:
2462:
2458:
2452:
2450:
2446:
2433:
2429:
2428:
2423:
2417:
2415:
2413:
2409:
2396:
2392:
2390:
2381:
2375:
2372:
2359:
2355:
2353:
2347:
2341:
2338:
2325:
2321:
2317:
2311:
2308:
2295:
2291:
2287:
2281:
2279:
2277:
2273:
2257:
2250:
2243:
2240:
2236:
2235:public domain
2217:
2213:
2209:
2203:
2201:
2199:
2197:
2193:
2188:
2184:
2180:
2176:
2172:
2168:
2164:
2160:
2153:
2150:
2145:
2141:
2137:
2133:
2128:
2123:
2119:
2115:
2111:
2104:
2101:
2096:
2092:
2088:
2084:
2080:
2076:
2072:
2068:
2061:
2059:
2055:
2042:
2038:
2034:
2028:
2025:
2020:
2016:
2012:
2008:
2004:
2000:
1993:
1990:
1977:
1973:
1969:
1963:
1960:
1947:
1943:
1939:
1933:
1931:
1927:
1914:
1910:
1908:
1902:
1896:
1893:
1880:
1876:
1872:
1866:
1863:
1849:
1845:
1841:
1835:
1833:
1831:
1829:
1825:
1812:
1808:
1804:
1798:
1796:
1792:
1779:
1775:
1773:
1767:
1761:
1758:
1754:
1744:
1741:
1737:
1727:
1724:
1711:
1707:
1703:
1697:
1694:
1681:
1677:
1673:
1667:
1664:
1659:
1655:
1650:
1645:
1641:
1637:
1633:
1629:
1625:
1617:
1614:
1601:
1597:
1591:
1589:
1585:
1580:
1576:
1571:
1566:
1562:
1558:
1554:
1547:
1544:
1539:
1535:
1530:
1525:
1521:
1517:
1513:
1506:
1503:
1498:
1494:
1490:
1486:
1479:
1476:
1471:
1467:
1462:
1457:
1453:
1449:
1445:
1438:
1435:
1423:
1419:
1413:
1410:
1405:
1401:
1396:
1391:
1387:
1383:
1379:
1372:
1370:
1366:
1353:
1349:
1343:
1340:
1335:
1331:
1327:
1323:
1319:
1315:
1308:
1305:
1292:
1288:
1282:
1279:
1268:
1264:
1258:
1255:
1250:
1246:
1241:
1236:
1232:
1228:
1224:
1220:
1216:
1209:
1206:
1201:
1197:
1192:
1187:
1183:
1179:
1175:
1171:
1167:
1160:
1158:
1156:
1154:
1150:
1145:
1141:
1136:
1131:
1127:
1123:
1119:
1115:
1111:
1104:
1102:
1100:
1098:
1096:
1094:
1092:
1090:
1088:
1086:
1084:
1082:
1080:
1078:
1076:
1074:
1072:
1070:
1066:
1061:
1055:
1051:
1044:
1042:
1038:
1035:
1029:
1026:
1021:
1017:
1013:
1009:
1005:
1001:
997:
993:
986:
983:
978:
974:
969:
964:
960:
956:
952:
945:
943:
939:
932:
930:
928:
923:
921:
917:
913:
905:
903:
899:
897:
893:
889:
881:
876:
872:
868:
864:
862:
857:
855:
851:
846:
844:
840:
836:
832:
831:Pegcetacoplan
828:
823:
816:
815:Pegcetacoplan
808:Pegcetacoplan
807:
802:
798:
794:
792:
788:
784:
780:
776:
772:
767:
760:
751:
746:
742:
738:
734:
731:
727:
723:
719:
716:and need for
715:
711:
707:
703:
699:
695:
691:
686:
677:
672:
669:
667:
666:complement C3
663:
659:
655:
651:
646:
639:
630:
625:
621:
617:
615:
611:
607:
603:
598:
589:
587:
585:
580:
578:
569:
567:
565:
561:
557:
550:Acute attacks
549:
544:
542:
535:
530:
527:
524:
521:
518:
515:
514:
513:
507:
505:
503:
499:
495:
491:
487:
483:
478:
476:
475:
470:
465:
463:
459:
455:
451:
447:
443:
439:
435:
434:reticulocytes
431:
427:
423:
419:
415:
411:
404:
402:
399:
394:
392:
391:heart failure
388:
384:
380:
376:
375:smooth muscle
372:
368:
364:
359:
357:
354:and prevents
353:
349:
345:
344:C3-convertase
341:
337:
333:
329:
325:
320:
318:
314:
310:
306:
301:
299:
295:
285:
277:
270:
268:
266:
262:
258:
254:
250:
246:
242:
238:
234:
230:
226:
221:
219:
218:smooth muscle
215:
212:, as well as
211:
207:
203:
198:
196:
192:
188:
184:
180:
176:
168:
166:
163:
159:
156:
152:
148:
146:
142:
138:
134:
133:cell membrane
130:
125:
123:
119:
115:
114:intravascular
111:
107:
103:
99:
95:
91:
87:
83:
73:
67:
64:
62:
58:
53:
49:
44:
40:
36:
31:
19:
4181:
4139:Phospholipid
4131:Inborn error
4083:
4040:C1-inhibitor
4038:
3955:
3948:
3911:
3910:
3895:
3885:
3879:
3852:ICF syndrome
3618:normochromic
3610:
3586:
3475:
3468:
3376:
3334:
3308:
3297:
3190:Polycythemia
3121:
3110:
3086:
3075:
3064:
3049:
3034:
2986:
2982:
2976:
2963:
2959:
2950:
2946:
2940:
2931:
2928:Policlinico
2927:
2921:
2912:
2909:Policlinico
2908:
2902:
2877:
2873:
2863:
2828:
2824:
2814:
2792:. Retrieved
2779:
2767:
2745:. Retrieved
2687:
2683:
2673:
2661:. Retrieved
2656:
2611:
2607:
2601:
2554:
2550:
2540:
2495:
2491:
2481:
2469:. Retrieved
2436:. Retrieved
2425:
2399:. Retrieved
2386:
2374:
2362:. Retrieved
2349:
2340:
2328:. Retrieved
2319:
2310:
2298:. Retrieved
2289:
2263:. Retrieved
2256:the original
2242:
2220:. Retrieved
2211:
2162:
2158:
2152:
2117:
2113:
2103:
2070:
2066:
2045:. Retrieved
2027:
2002:
1998:
1992:
1980:. Retrieved
1971:
1962:
1950:. Retrieved
1941:
1917:. Retrieved
1904:
1895:
1883:. Retrieved
1874:
1865:
1852:. Retrieved
1843:
1815:. Retrieved
1806:
1782:. Retrieved
1769:
1760:
1743:
1726:
1714:. Retrieved
1706:Navlin Daily
1705:
1696:
1684:. Retrieved
1675:
1666:
1631:
1627:
1616:
1604:. Retrieved
1599:
1560:
1556:
1546:
1519:
1515:
1505:
1488:
1485:N Engl J Med
1484:
1478:
1451:
1447:
1437:
1425:. Retrieved
1421:
1412:
1385:
1381:
1356:. Retrieved
1351:
1342:
1317:
1313:
1307:
1295:. Retrieved
1290:
1281:
1270:. Retrieved
1266:
1257:
1225:(1): 49–64.
1222:
1218:
1208:
1173:
1169:
1117:
1113:
1049:
1028:
998:(3): 141–8.
995:
991:
985:
958:
954:
926:
924:
909:
900:
885:
882:Epidemiology
858:
847:
829:
827:
773:
771:
735:
692:
690:
670:
652:
650:
604:
602:
581:
573:
560:prednisolone
553:
539:
528:
522:
516:
511:
479:
472:
466:
408:
395:
371:nitric oxide
360:
332:opsonization
321:
309:X chromosome
302:
290:
233:hepatic vein
222:
199:
195:palpitations
172:
149:
128:
126:
113:
85:
81:
80:
3912:peripheral:
3623:hypochromic
3579:Blood tests
3341:Thalassemia
3299:enzymopathy
3228:Nutritional
3112:MedlinePlus
2747:18 February
2471:18 February
2364:10 December
2300:10 December
1885:24 February
1875:AstraZeneca
1854:24 February
1606:5 September
1454:(1): 3–22.
1427:2 September
845:inhibitor.
517:Classic PNH
438:bone marrow
430:haptoglobin
410:Blood tests
389:and causes
294:glycolipids
241:portal vein
179:hemosiderin
141:bone marrow
38:Other names
4202:Categories
4045:Angioedema
4031:deficiency
4029:Complement
4006:Leukopenia
3956:autosomal:
3710:complement
3604:macrocytic
3599:microcytic
3594:normocytic
3557:Acquired:
3543:Hereditary
3310:glycolysis
3290:Hereditary
3088:DiseasesDB
2934:: 105–117.
2915:: 241–254.
2794:22 January
2553:(Review).
2330:6 December
1272:2018-11-29
933:References
916:Greifswald
843:complement
835:medication
779:medication
730:complement
694:Eculizumab
685:Eculizumab
678:Eculizumab
658:complement
606:Crovalimab
597:Crovalimab
590:Crovalimab
454:antibodies
418:hemoglobin
379:sildenafil
367:hemoglobin
158:eculizumab
122:thrombosis
66:Hematology
3949:x-linked:
3275:Hemolytic
3123:eMedicine
2894:260089988
2714:250731980
2636:248245151
2575:2040-6215
2532:254149706
2401:9 January
1753:help page
1736:help page
775:Iptacopan
759:Iptacopan
752:Iptacopan
741:developed
662:hemolysis
654:Danicopan
638:Danicopan
631:Danicopan
624:Genentech
570:Long-term
558:(such as
545:Treatment
536:Screening
450:hemolysis
420:, raised
405:Diagnosis
348:protectin
255:(causing
235:(causing
61:Specialty
4148:Tafazzin
3996:HIV/AIDS
3988:Acquired
3939:combined
3731:Antibody
3706:Lymphoid
3533:(mostly
3530:Aplastic
3470:membrane
3417:Acquired
3378:membrane
3278:(mostly
3171:Diseases
3003:30149910
2855:21707954
2788:Archived
2741:Archived
2706:35860926
2657:DailyMed
2628:35441351
2593:35923770
2524:36459381
2515:10234880
2465:Archived
2432:Archived
2427:DailyMed
2395:Archived
2358:Archived
2324:Archived
2320:Novartis
2294:Archived
2290:DailyMed
2216:Archived
2187:36682579
2179:24249647
2144:21972428
2136:18784156
2095:22732675
2087:17989688
2041:Archived
2019:25356860
1976:Archived
1946:Archived
1942:DailyMed
1919:30 April
1913:Archived
1879:Archived
1848:Archived
1817:25 April
1811:Archived
1807:DailyMed
1778:Archived
1710:Archived
1686:18 April
1680:Archived
1658:31978221
1600:DailyMed
1579:12893760
1538:23610373
1470:11918528
1404:15811985
1334:25356860
1249:29327071
1200:19372253
1144:16051736
1020:21266914
1012:22395662
977:23950173
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