51:
577:
793:, which can be used to estimate an individual's risk of HLH. In adults, soluble IL-2 receptor has been found to be a very sensitive marker for HLH, demonstrating 100% sensitivity for ruling out HLH below a cutoff of 2400 U/mL and optimal cutoff for ruling in at 2515 U/mL (sensitivity, 100%; specificity, 72.5%), with 93% specificity at >10 000 U/mL.
524:-dependent cytotoxicity. This inability to remove infected and antigen-presenting cells and terminate the immune response leads to uncontrolled proliferation and activation of the immune system with release of excessive cytokines. These cells then infiltrate organs, releasing more cytokines, which gives the clinical picture. The fever is caused by
154:
The onset of HLH occurs before the age of one year in approximately 70 percent of cases. Familial HLH should be suspected if siblings are diagnosed with HLH or if symptoms recur when therapy has been stopped. Familial HLH is an autosomal recessive disease, hence each sibling of a child with familial
1322:
Bode SF, Ammann S, Al-Herz W, Bataneant M, Dvorak CC, Gehring S, Gennery A, Gilmour KC, Gonzalez-Granado LI, GroĂź-Wieltsch U, Ifversen M, Lingman-Framme J, Matthes-Martin S, Mesters R, Meyts I, van
Montfrans JM, Pachlopnik Schmid J, Pai SY, Soler-Palacin P, Schuermann U, Schuster V, Seidel MG,
788:
Not all five out of eight criteria are required for diagnosis of HLH in adults, and a high index of suspicion is required for diagnosis as delay results in increased mortality. The diagnostic criteria were developed in pediatric populations and have not been validated for adult HLH patients.
844:(type 2). This is a rare autosomal recessive disorder characterized by partial albinism, hepatosplenomegaly, pancytopenia, hepatitis, immunologic abnormalities, and lymphohistiocytosis. Most cases have been diagnosed between 4 months and 7 years of age, with a mean age of about 17 months.
980:
A systematic review recently reported the pooled proportion are fever 97.2%, hepatomegaly 70.2%, splenomegaly 78.4%, thrombocytopenia 90.1%, anemia 76.0%, and serum ferritin ≥500 μg/L 97.1%. The case fatality rate is 14.6% among dengue hemophagocytic lymphohistiocytosis patients.
155:
HLH has a twenty-five–percent chance of developing the disease, a fifty-percent chance of carrying the defective gene (which is very rarely associated with any risk of disease), and a twenty-five–percent chance of not being affected and not carrying the gene defect.
827:
The diagnosis of acquired, or secondary, HLH is usually made in association with infection by viruses, bacteria, fungi, or parasites or in association with lymphoma, autoimmune disease, or metabolic disease. Acquired HLH may have decreased, normal, or increased
867:
HLH is a description of an immunophysiologic state in time. It can be dangerous to infer a genetic impairment of granule-mediated cytotoxicity in patients, especially older children and adults, who meet any of the various criteria for HLH. Thus, like
2505:
2490:
675:
Secondary haemophagocytic lymphohistiocytosis (acquired haemophagocytic lymphohistiocytosis) occurs after strong immunologic activation, such as that which can occur with systemic infection, immunodeficiency, or underlying malignancy.
3288:
1954:
Benson LA, Li H, Henderson LA, Solomon IH, Soldatos A, Murphy J, Bielekova B, Kennedy AL, Rivkin MJ, Davies KJ, Hsu AP, Holland SM, Gahl WA, Sundel RP, Lehmann LE, Lee MA, Alexandrescu S, Degar BA, Duncan CN, Gorman MP (May 2019).
952:
The prognosis is guarded with an overall mortality of 50%. Poor prognostic factors included HLH associated with malignancy, with half the patients dying by 1.4 months compared to 22.8 months for non-tumour associated HLH patients.
519:
The underlying causes, either inherited or acquired, lead to an unchecked immune response when exposed to triggers. Impaired NK-cell cytotoxicity is the hallmark of HLH. All genetic defects for familial HLH are related to
2307:
446:
for four of the causative genes, PRF1 (FHL2), UNC13D (FHL3), STX11 (FHL4), and STXBP2 (FHL5), is available on a clinical basis. Symptoms of FHL are usually evident within the first few months of life and may even develop
209:
The vast majority of patients who meet these criteria will not have a genetic cause of their disease, but rather HLH will be triggered by infection, malignancy, rheumatic disease, and/or certain treatments (as in the
886:
Most patients who meet HLH criteria will have secondary cases. Treatment for these patients should focus on the underlying contributors. Additionally, treatment of the inflammation of HLH itself is often required.
431:
427:
2858:
242:
3376:
679:
Both forms are characterized by the overwhelming activation of normal T lymphocytes and macrophages, invariably leading to clinical and haematologic alterations and death in the absence of treatment.
635:
level are markedly elevated. In children, a ferritin above 10000 is very sensitive and specific for the diagnosis of HLH, however, the diagnostic utility for ferritin is less for adult HLH patients.
972:
The first case report of HLH was published in 1939 under the term "Histiocytic
Medullary Reticulosis". A second report would come out in 1952 that would rename the disorder that same year.
960:
only). However, long-term remission without the use of cytotoxic and immune-suppressive therapies is unlikely in the majority of adults with HLH and in those with involvement of the
2316:
2851:
1279:
442:
Five genetic subtypes (FHL1, FHL2, FHL3, FHL4, and FHL5) are described, with an estimated overall prevalence of one in 50,000 and equal gender distribution. Molecular
1073:
956:
Secondary HLH in some individuals may be self-limited because patients are able to fully recover after having received only supportive medical treatment (i.e., IV
2844:
672:(FHL) or familial erythrophagocytic lymphohistiocytosis, is a heterogeneous autosomal recessive disorder found to be more prevalent with parental consanguinity.
2756:
2682:
2370:
337:
2672:
1531:
Daver N, McClain K, Allen CE, Parikh SA, Otrock Z, Rojas-Hernandez C, Blechacz B, Wang S, Minkov M, Jordan MB, La Rosée P, Kantarjian HM (September 2017).
3456:
2285:
855:
and haemophagocytic syndrome, with abnormal T-cell and macrophage activation. This type has a grave prognosis if untreated. Type 3 has mutations in
2636:
817:
2086:
Fardet, Laurence (September 9, 2014). "Development and
Validation of the HScore, a Score for the Diagnosis of Reactive Hemophagocytic Syndrome".
426:) are associated with, and thought to be triggered or promoted by, EBV infection. These cases of HLH are classified as belonging to the class of
1227:
Giang HT, Banno K, Minh LH, Trinh LT, Loc LT, Eltobgy A, Tai LL, Khan A, Tuan NH, Reda Y, Samsom M, Nam NT, Huy NT, Hirayama K (November 2018).
3451:
2804:
873:
3431:
1325:"The syndrome of hemophagocytic lymphohistiocytosis in primary immunodeficiencies: implications for differential diagnosis and pathogenesis"
3330:
2976:
1474:
Braga Neto, Manuel B.; Badley, Andrew D.; Parikh, Sameer A.; Graham, Rondell P.; Kamath, Patrick S. (2022-02-03). Solomon, Caren G. (ed.).
995:
810:
423:
2006:"Hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: a journey of a thousand miles begins with a single (big) step"
2520:
883:
has collected the published consensus management documents for the many contexts in which HLH occurs and they host full-text versions.
2954:
2867:
2765:
1850:"Evaluation of the role of secretory sphingomyelinase and bioactive sphingolipids as biomarkers in hemophagocytic lymphohistiocytosis"
221:
Primary HLH is caused by high-penetrance variants in genes associated with the syndrome, and thus is part of the phenotype of several
2809:
2746:
2736:
2693:
2276:"Press Announcements – FDA approves first treatment specifically for patients with rare and life-threatening type of immune disease"
1323:
Speckmann C, Stepensky P, Sykora KW, Tesi B, Vraetz T, Waruiru C, Bryceson YT, Moshous D, Lehmberg K, Jordan MB, Ehl S (July 2015).
1189:
Esteban, Ysabella M.; de Jong, Jill L. O.; Tesher, Melissa S. (1 August 2017). "An
Overview of Hemophagocytic Lymphohistiocytosis".
941:
1229:"Dengue hemophagocytic syndrome: A systematic review and meta-analysis on epidemiology, clinical signs, outcomes, and risk factors"
693:
1. A molecular diagnosis consistent with HLH. These include the identification of pathologic mutations of PRF1, UNC13D, or STX11.
3001:
2775:
368:
2926:
2920:
2229:
Machowicz R, Janka G, Wiktor-Jedrzejczak W (March 2017). "Similar but not the same: Differential diagnosis of HLH and sepsis".
3344:
802:
628:
333:
3015:
2912:
341:
50:
3219:
3191:
2731:
2654:
1667:
1374:
Mehta, Puja; McAuley, Daniel F.; Brown, Michael; Sanchez, Emilie; Tattersall, Rachel S.; Manson, Jessica J. (2020-03-28).
1065:
349:
151:
HLH as defined by the HLH-04 criteria (see below) is a descriptive diagnosis. Its individual components are non-specific.
2309:
Clinical
Commissioning Policy: Anakinra for Haemophagocytic Lymphohistiocytosis (HLH) for adults and children in all ages
934:
Clinical
Commissioning Policy: Anakinra for Haemophagocytic Lymphohistiocytosis (HLH) for adults and children in all ages
376:
2280:
1021:
576:
451:. However, symptomatic presentation throughout childhood and even into young adulthood has been observed in some cases.
345:
124:
2716:
2871:
2629:
2088:
1762:
872:, one must simultaneously manage both the acute physiologic changes associated with HLH (like systemic inflammation,
511:
Nearly half of the cases of type 2 familial hemophagocytic lymphohistiocytosis are due to bi-allelic PRF1 mutations.
2836:
1760:
Allen, Carl (June 2008). "Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis".
415:
causes such as bone marrow or other organ transplantations; chemotherapy; or therapy with immunosuppressing agents.
1638:
Zhang, Kejian; Filopovich, Alexandra H.; Johnson, Judith; Marsh, Rebecca A.; Villanueva, Joyce (January 17, 2013).
504:
318:
123:
seen more often in children than in adults. It is a life-threatening disease of severe hyperinflammation caused by
3064:
2825:
2667:
380:
3412:
1896:
222:
211:
2721:
3360:
2706:
2540:
1900:
915:
806:
326:
2342:
682:
A subtype of primary HLH where the inflammation is limited to the central nervous system has been described.
2799:
2786:
1588:
1277:
Wysocki CA (December 2017). "Comparing hemophagocytic lymphohistiocytosis in pediatric and adult patients".
2275:
890:
While optimal treatment of HLH is still being debated, current treatment regimes usually involve high dose
3461:
3247:
2726:
2677:
2622:
961:
553:
384:
322:
198:
418:
About 33% of all HLH cases, ~75% of Asian HLH cases, and nearly 100% of HLH cases caused by mutations in
2761:
2741:
561:
136:
1147:"How can an internal medicine specialist save a patient with hemophagocytic lymphohistiocytosis (HLH)?"
162:. Therefore, HLH should be included in the differential diagnosis of intensive care unit patients with
3029:
2769:
2659:
740:
357:
120:
108:
2794:
2698:
2509:
869:
771:
234:
847:
Three types of
Griscelli syndrome are recognised: type 1 has neurologic symptoms and mutations in
3163:
3122:
3102:
3086:
2113:
1787:
1513:
1304:
1256:
880:
859:
and is characterized by partial albinism. This type does not pose a threat to those so affected.
841:
624:
182:
2392:
Scott, Ronald Bodley; Robb-Smith, A.H.T. (July 22, 1939). "Histiocytic
Medullary Reticulosis".
2551:
2458:
2409:
2362:
2246:
2211:
2162:
2105:
2068:
2027:
1986:
1936:
1879:
1830:
1779:
1742:
1701:
1649:
1617:
1564:
1505:
1456:
1407:
1356:
1296:
1248:
1206:
1168:
1127:
1047:
714:
Decreased blood cell counts affecting at least two of three lineages in the peripheral blood:
565:
521:
372:
171:
167:
74:
3348:
3272:
2751:
2448:
2440:
2401:
2354:
2238:
2201:
2193:
2182:"Autoimmune lymphoproliferative syndrome misdiagnosed as hemophagocytic lymphohistiocytosis"
2152:
2144:
2097:
2058:
2017:
1976:
1968:
1926:
1869:
1861:
1820:
1771:
1732:
1691:
1683:
1607:
1597:
1554:
1546:
1537:
1495:
1487:
1446:
1438:
1397:
1387:
1346:
1338:
1288:
1240:
1198:
1158:
1117:
1107:
1037:
1029:
725:
717:
Haemoglobin <9 g/100 ml (in infants <4 weeks: haemoglobin <10 g/100 ml) (
650:
613:
609:
545:
364:
353:
230:
3045:
1811:
1533:"A consensus review on malignancy-associated hemophagocytic lymphohistiocytosis in adults"
851:. Prognosis depends on the severity of neurologic manifestations. Type 2 has mutations in
589:
443:
388:
314:
310:
186:
785:
In addition, in the case of familial HLH, no evidence of malignancy should be apparent.
743:(fasting, greater than or equal to 265 mg/100 ml) and/or decreased amounts of
309:
to attack EBV-infected cells. Malignant disorders associated with secondary HLH include
3390:
3263:
2453:
2428:
2206:
2181:
2157:
2132:
1981:
1956:
1874:
1849:
1696:
1671:
1612:
1583:
1559:
1532:
1500:
1475:
1451:
1426:
1402:
1375:
1351:
1324:
1228:
1122:
1095:
1042:
1016:
957:
903:
891:
197:. Laboratory findings may include elevated triglyceride levels, low fibrinogen levels,
159:
140:
62:
17:
2514:
2405:
1392:
3445:
3108:
2645:
2242:
2148:
1517:
1329:
990:
657:
541:
529:
525:
306:
2117:
1791:
1308:
1260:
3138:
3116:
2343:"Prognostic factors and outcomes of adults with hemophagocytic lymphohistiocytosis"
907:
856:
709:
597:
246:
2591:
2263:
925:(proprietary name Gamifant) for the treatment of pediatric and adult primary HLH.
66:
2545:
2358:
2063:
2046:
1972:
1825:
1806:
1342:
1292:
1202:
3175:
2597:
1644:
929:
911:
899:
757:
732:
581:
2556:
3338:
3324:
2890:
2602:
2499:
1931:
1914:
1639:
1112:
1096:"Your critical care patient may have HLH (hemophagocytic lymphohistiocytosis)"
922:
765:
744:
639:
605:
585:
412:
408:
302:
283:
215:
132:
128:
92:
83:
79:
58:
1602:
921:
On 20 November 2018, the FDA approved the anti-IFN-gamma monoclonal antibody
3406:
2906:
2586:
2562:
2133:"Soluble interleukin-2 receptor is a sensitive diagnostic test in adult HLH"
895:
616:. The liver function tests are usually elevated. A low level of the protein
537:
533:
163:
2462:
2444:
2366:
2250:
2215:
2197:
2166:
2109:
2072:
2031:
1990:
1940:
1883:
1834:
1807:"Marked hyperferritinemia does not predict for HLH in the adult population"
1783:
1746:
1705:
1653:
1621:
1568:
1509:
1491:
1460:
1411:
1360:
1300:
1252:
1210:
1172:
1131:
1094:
Machowicz, Rafal; Janka, Gritta; Wiktor-Jedrzejczak, Wieslaw (2016-01-01).
1051:
1033:
820:. As a syndrome of intense inflammation it needs to be differentiated from
143:
syndromes. There are inherited and non-inherited (acquired) causes of HLH.
2413:
1442:
3314:
3231:
2886:
2022:
2005:
1672:"Human perforin mutations and susceptibility to multiple primary cancers"
937:
781:(soluble IL-2 receptor) >2400 U/ml (or per local reference laboratory)
750:
632:
557:
473:
400:
396:
392:
271:
238:
226:
190:
2482:
1913:
Ponnatt, Tanya Sajan; Lilley, Cullen M.; Mirza, Kamran M. (2021-08-04).
1163:
1146:
876:, hepatitis, etc.) and look deeply for various underlying contributors.
301:
Secondary HLH (sHLH) is associated with, and thought to be promoted by,
3203:
3185:
1687:
1550:
1425:
Fajgenbaum, David C.; June, Carl H. (2020-12-03). Longo, Dan L. (ed.).
829:
643:
617:
170:. Patients in the earlier stages of HLH are frequently hospitalized at
2101:
1865:
1775:
1737:
1720:
3384:
3370:
3282:
3241:
3147:
3080:
2948:
2494:
2180:
Rudman
Spergel A, Walkovich K, Price S, et al. (November 2013).
2047:"How I treat hemophagocytic lymphohistiocytosis in the adult patient"
1244:
852:
821:
790:
761:
718:
601:
549:
548:. TNF-alpha and TNF-gamma may also lead to inhibition of lipoprotein
499:
481:
275:
263:
259:
251:
1376:"COVID-19: consider cytokine storm syndromes and immunosuppression"
1066:"Familial hemophagocytic lymphohistiocytosis: MedlinePlus Genetics"
454:
The five subtypes of FHL are each associated with a specific gene:
305:
and non-malignant diseases that likewise weaken the ability of the
3296:
3213:
3157:
3023:
3009:
2995:
2938:
2567:
848:
816:
Other conditions that may be confused with this condition include
703:
575:
490:
460:
404:
332:
In rheumatic diseases, this syndrome is more often referred to as
294:
279:
255:
178:
3058:
2970:
2900:
2614:
2534:
2531:
2528:
2525:
778:
468:
287:
267:
194:
2840:
2618:
1719:
Usmani, G. Naheed; Woda, Bruce A.; Newburger, Peter E. (2013).
1848:
Jenkins RW, Clarke CJ, Lucas JT, et al. (November 2013).
809:
that present with hemophagocytic lymphohistiocytosis, such as
789:
Attempts to improve diagnosis of HLH have included use of the
801:
The differential diagnosis of HLH includes secondary HLH and
225:. The most common and best studied causes of Primary HLH are
706:(defined as a temperature >100.3 °F, >38 °C)
1957:"Pediatric CNS-isolated hemophagocytic lymphohistiocytosis"
1584:"Epstein–Barr Virus and Hemophagocytic Lymphohistiocytosis"
2427:
Farquhar, James W.; Claireaux, Albert E. (December 1952).
428:
Epstein–Barr virus–associated lymphoproliferative diseases
27:
Immune disorder in the blood leading to hyperinflammation
699:
2. Fulfillment of five out of the eight criteria below:
249:. These mutations include those in the following genes:
237:
use to kill targeted cells, such as those infected with
158:
Patients with HLH, especially when untreated, may need
2472:
3359:
3313:
3271:
3262:
3230:
3202:
3174:
3146:
3137:
3044:
2987:
2937:
2885:
2878:
2818:
2784:
2691:
2652:
2577:
2476:
1721:"Advances in understanding the pathogenesis of HLH"
690:The current (2008) diagnostic criteria for HLH are
73:
40:
35:
1280:Current Opinion in Allergy and Clinical Immunology
1670:, Thia KY, Andrews M, et al. (April 2013).
1145:Machowicz, Rafal; Basak, Grzegorz (2020-03-05).
996:X-linked lymphoproliferative disease § XLP2
2264:https://www.histiocytesociety.org/HLH-Consensus
1919:Archives of Pathology & Laboratory Medicine
1184:
1182:
914:have also been used. Other medications include
227:loss of function, (i.e. inactivating) mutations
201:, and elevated ferritin levels (among others).
1272:
1270:
2852:
2757:Hereditary progressive mucinous histiocytosis
2683:Congenital self-healing reticulohistiocytosis
2630:
1640:"Familial Hemophagocytic Lymphohistiocytosis"
747:in the blood (≤ 150 mg/100 ml)
600:—including a decreased number of circulating
8:
670:familial haemophagocytic lymphohistiocytosis
2603:Familial Hemophagocytic Lymphohistiocytosis
424:X-linked lymphoproliferative disease type 1
3268:
3143:
3050:
2882:
2859:
2845:
2837:
2637:
2623:
2615:
2473:
1633:
1631:
49:
32:
2452:
2205:
2156:
2062:
2021:
2004:Jordan MB, Filipovich AH (October 2008).
1980:
1930:
1899:at the U.S. National Library of Medicine
1873:
1824:
1736:
1695:
1611:
1601:
1558:
1499:
1450:
1401:
1391:
1350:
1222:
1220:
1162:
1121:
1111:
1041:
840:A major differential diagnosis of HLH is
191:yellow discoloration of the skin and eyes
1017:"Hemophagocytic syndromes and infection"
2231:Critical Reviews in Oncology/Hematology
1007:
818:autoimmune lymphoproliferative syndrome
3331:Congenital generalized lipodystrophy 3
2805:Interdigitating dendritic cell sarcoma
2429:"Familial Haemophagocytic Reticulosis"
944:) to be used in the treatment of HLH.
824:, which may be extremely challenging.
2373:from the original on October 18, 2021
411:. Secondary HLH may also result from
7:
3303:Hemophagocytic lymphohistiocytosis 4
1915:"Hemophagocytic Lymphohistiocytosis"
1151:Polish Archives of Internal Medicine
811:X-linked lymphoproliferative disease
540:is due to the suppressive effect on
363:Secondary HLH also occurs rarely in
342:adult onset forms of Still's disease
334:macrophage activation syndrome (MAS)
1897:Lymphohistiocytosis,+Hemophagocytic
183:enlargement of the liver and spleen
105:haemophagocytic lymphohistiocytosis
2955:Cranio-lenticulo-sutural dysplasia
2868:Inherited disorders of trafficking
2766:Multicentric reticulohistiocytosis
2712:Hemophagocytic lymphohistiocytosis
741:High blood levels of triglycerides
97:hemophagocytic lymphohistiocytosis
36:Hemophagocytic lymphohistiocytosis
25:
3345:Limb-girdle muscular dystrophy 2B
2810:Follicular dendritic cell sarcoma
2747:Progressive nodular histiocytosis
2737:Generalized eruptive histiocytoma
2694:non-Langerhans cell histiocytosis
942:interleukin 1 receptor antagonist
387:); and infections caused by EBV,
139:. It is classified as one of the
3457:Diseases of immune dysregulation
3220:Hereditary spastic paraplegia 10
3002:X-linked intellectual disability
2776:Indeterminate cell histiocytosis
2433:Archives of Disease in Childhood
2243:10.1016/j.critrevonc.2017.03.023
2149:10.1182/bloodadvances.2017012310
1961:Neurol Neuroimmunol Neuroinflamm
1805:Schram, Alison (March 5, 2015).
598:decreased numbers of blood cells
596:The blood count typically shows
556:. Activated macrophages secrete
369:severe combined immunodeficiency
229:in genes that code for proteins
3192:Hereditary spastic paraplegia 4
2673:Hand–Schüller–Christian disease
2288:from the original on 2019-04-23
1480:New England Journal of Medicine
1431:New England Journal of Medicine
1076:from the original on 2021-01-25
223:inborn errors of immunity (IEI)
2131:Hayden, Anna (December 2017).
2045:Schram, Alison (May 7, 2015).
1725:British Journal of Haematology
803:macrophage-activation syndrome
629:erythrocyte sedimentation rate
336:and occurs most frequently in
177:HLH clinically manifests with
1:
3452:Autosomal recessive disorders
2732:Benign cephalic histiocytosis
2655:Langerhans cell histiocytosis
2406:10.1016/S0140-6736(00)61951-7
2341:Parikh, Sameer (April 2014).
1393:10.1016/S0140-6736(20)30628-0
642:level is usually low and the
350:juvenile idiopathic arthritis
135:that secrete high amounts of
3432:vesicular transport proteins
2872:vesicular transport proteins
2359:10.1016/j.mayocp.2013.12.012
2306:NHS England (October 2021).
2281:Food and Drug Administration
2089:Arthritis & Rheumatology
2064:10.1182/blood-2015-01-551622
1973:10.1212/NXI.0000000000000560
1826:10.1182/blood-2014-10-602607
1763:Pediatric Blood & Cancer
1343:10.3324/haematol.2014.121608
1293:10.1097/ACI.0000000000000405
1203:10.3928/19382359-20170717-01
964:(brain and/or spinal cord).
346:systemic lupus erythematosus
3016:Hermansky–Pudlak syndrome 2
668:Primary HLH, also known as
612:. The bone marrow may show
580:Light microscopic image of
3478:
807:primary immunodeficiencies
505:Syntaxin binding protein 2
365:immunodeficiency disorders
319:acute lymphocytic leukemia
125:uncontrolled proliferation
3427:
3413:Distal muscular dystrophy
3401:
3097:
3075:
3053:
2965:
2913:Hermansky–Pudlak syndrome
1932:10.5858/arpa.2020-0802-RA
1113:10.1186/s13054-016-1369-3
1015:Fisman, David N. (2000).
916:cytokine targeted therapy
756:Haemophagocytosis in the
710:Enlargement of the spleen
656:Bone marrow biopsy shows
212:cytokine release syndrome
57:
48:
3361:Vacuolar protein sorting
3248:Spinocerebellar ataxia 5
2927:Chédiak–Higashi syndrome
2707:Juvenile xanthogranuloma
1901:Medical Subject Headings
1603:10.3389/fimmu.2017.01902
940:(a modified recombinant
731:Neutrophils <1Ă—10/L (
724:Platelets <100Ă—10/L (
620:in the blood is common.
377:Wiskott–Aldrich syndrome
327:myelodysplastic syndrome
117:haemophagocytic syndrome
2800:Langerhans cell sarcoma
2787:malignant histiocytosis
2717:Erdheim-Chester disease
2347:Mayo Clinic Proceedings
1589:Frontiers in Immunology
1476:"Calm before the Storm"
18:Hemophagocytic syndrome
2727:Sea-blue histiocytosis
2678:Eosinophilic granuloma
2445:10.1136/adc.27.136.519
2198:10.1542/peds.2012-2748
2010:Bone Marrow Transplant
1492:10.1056/NEJMcps2111163
1034:10.3201/eid0606.000608
962:central nervous system
797:Differential diagnosis
593:
554:triglyceride synthesis
385:dyskeratosis congenita
348:. It occurs rarely in
323:acute myeloid leukemia
137:inflammatory cytokines
2826:Rosai–Dorfman disease
2762:Reticulohistiocytosis
2742:Xanthoma disseminatum
2668:Letterer–Siwe disease
1443:10.1056/NEJMra2026131
1022:Emerging Infect. Dis.
579:
562:plasminogen activator
381:ataxia–telangiectasia
3164:Griscelli syndrome 1
3123:Griscelli syndrome 3
3087:Griscelli syndrome 2
2770:Reticulohistiocytoma
2722:Niemann–Pick disease
2660:X-type histiocytosis
2023:10.1038/bmt.2008.232
588:containing numerous
358:rheumatoid arthritis
187:enlarged lymph nodes
121:hematologic disorder
65:within macrophages.
2795:Histiocytic sarcoma
2699:Non-X histiocytosis
1164:10.20452/pamw.15226
772:natural killer cell
686:Diagnostic criteria
646:level is elevated.
3349:Long QT syndrome 9
2578:External resources
2322:on 13 October 2021
1688:10.4161/onci.24185
1551:10.1002/cncr.30826
881:Histiocyte Society
879:The International
842:Griscelli syndrome
836:Griscelli syndrome
625:C reactive protein
594:
592:in their cytoplasm
338:the juvenile onset
243:Epstein-Barr virus
147:Signs and symptoms
3439:
3438:
3423:
3422:
3258:
3257:
3133:
3132:
3040:
3039:
2879:Vesicle formation
2834:
2833:
2612:
2611:
2102:10.1002/art.38690
1866:10.1002/ajh.23535
1776:10.1002/pbc.21423
1738:10.1111/bjh.12293
1582:Marsh RA (2017).
1386:(10229): 1033–4.
606:white blood cells
566:hyperfibrinolysis
544:by TNF-alpha and
373:DiGeorge syndrome
231:cytotoxic T cells
216:CART cell therapy
172:internal medicine
168:hyperferritinemia
160:intensive therapy
119:, is an uncommon
103:), also known as
89:
88:
30:Medical condition
16:(Redirected from
3469:
3273:Synaptic vesicle
3269:
3144:
3051:
2883:
2861:
2854:
2847:
2838:
2752:Papular xanthoma
2639:
2632:
2625:
2616:
2474:
2467:
2466:
2456:
2439:(136): 519–525.
2424:
2418:
2417:
2389:
2383:
2382:
2380:
2378:
2338:
2332:
2331:
2329:
2327:
2321:
2315:. Archived from
2314:
2303:
2297:
2296:
2294:
2293:
2272:
2266:
2261:
2255:
2254:
2226:
2220:
2219:
2209:
2177:
2171:
2170:
2160:
2128:
2122:
2121:
2083:
2077:
2076:
2066:
2042:
2036:
2035:
2025:
2001:
1995:
1994:
1984:
1951:
1945:
1944:
1934:
1910:
1904:
1894:
1888:
1887:
1877:
1845:
1839:
1838:
1828:
1802:
1796:
1795:
1757:
1751:
1750:
1740:
1716:
1710:
1709:
1699:
1664:
1658:
1657:
1635:
1626:
1625:
1615:
1605:
1579:
1573:
1572:
1562:
1528:
1522:
1521:
1503:
1471:
1465:
1464:
1454:
1427:"Cytokine Storm"
1422:
1416:
1415:
1405:
1395:
1371:
1365:
1364:
1354:
1319:
1313:
1312:
1274:
1265:
1264:
1245:10.1002/rmv.2005
1224:
1215:
1214:
1191:Pediatric Annals
1186:
1177:
1176:
1166:
1142:
1136:
1135:
1125:
1115:
1091:
1085:
1084:
1082:
1081:
1062:
1056:
1055:
1045:
1012:
928:In October 2021
753:≥ 500 ng/ml
726:thrombocytopenia
651:sphingomyelinase
614:hemophagocytosis
584:showing stromal
354:Kawasaki disease
214:associated with
109:British spelling
53:
33:
21:
3477:
3476:
3472:
3471:
3470:
3468:
3467:
3466:
3442:
3441:
3440:
3435:
3419:
3397:
3355:
3309:
3289:CEDNIK syndrome
3254:
3226:
3198:
3170:
3129:
3093:
3071:
3036:
2983:
2961:
2933:
2874:
2865:
2835:
2830:
2814:
2780:
2697:
2687:
2658:
2648:
2643:
2613:
2608:
2607:
2573:
2572:
2485:
2471:
2470:
2426:
2425:
2421:
2400:(6047): 194–8.
2391:
2390:
2386:
2376:
2374:
2340:
2339:
2335:
2325:
2323:
2319:
2312:
2305:
2304:
2300:
2291:
2289:
2274:
2273:
2269:
2262:
2258:
2228:
2227:
2223:
2179:
2178:
2174:
2143:(26): 2529–34.
2130:
2129:
2125:
2085:
2084:
2080:
2057:(19): 2908–14.
2044:
2043:
2039:
2003:
2002:
1998:
1953:
1952:
1948:
1912:
1911:
1907:
1895:
1891:
1860:(11): E265–72.
1847:
1846:
1842:
1819:(10): 1548–52.
1804:
1803:
1799:
1759:
1758:
1754:
1718:
1717:
1713:
1666:
1665:
1661:
1637:
1636:
1629:
1581:
1580:
1576:
1545:(17): 3229–40.
1530:
1529:
1525:
1473:
1472:
1468:
1437:(23): 2255–73.
1424:
1423:
1419:
1373:
1372:
1368:
1321:
1320:
1316:
1276:
1275:
1268:
1226:
1225:
1218:
1188:
1187:
1180:
1144:
1143:
1139:
1093:
1092:
1088:
1079:
1077:
1070:medlineplus.gov
1064:
1063:
1059:
1014:
1013:
1009:
1004:
987:
978:
970:
950:
892:corticosteroids
865:
838:
799:
688:
666:
602:red blood cells
590:red blood cells
574:
517:
515:Pathophysiology
444:genetic testing
440:
389:cytomegalovirus
315:B-cell lymphoma
311:T-cell lymphoma
207:
149:
63:red blood cells
31:
28:
23:
22:
15:
12:
11:
5:
3475:
3473:
3465:
3464:
3459:
3454:
3444:
3443:
3437:
3436:
3428:
3425:
3424:
3421:
3420:
3418:
3417:
3416:
3415:
3402:
3399:
3398:
3396:
3395:
3394:
3393:
3391:Cohen syndrome
3381:
3380:
3379:
3366:
3364:
3357:
3356:
3354:
3353:
3352:
3351:
3335:
3334:
3333:
3320:
3318:
3311:
3310:
3308:
3307:
3306:
3305:
3293:
3292:
3291:
3278:
3276:
3266:
3264:Vesicle fusion
3260:
3259:
3256:
3255:
3253:
3252:
3251:
3250:
3237:
3235:
3228:
3227:
3225:
3224:
3223:
3222:
3209:
3207:
3200:
3199:
3197:
3196:
3195:
3194:
3181:
3179:
3172:
3171:
3169:
3168:
3167:
3166:
3153:
3151:
3141:
3135:
3134:
3131:
3130:
3128:
3127:
3126:
3125:
3113:
3112:
3111:
3098:
3095:
3094:
3092:
3091:
3090:
3089:
3076:
3073:
3072:
3070:
3069:
3068:
3067:
3054:
3048:
3042:
3041:
3038:
3037:
3035:
3034:
3033:
3032:
3020:
3019:
3018:
3006:
3005:
3004:
2991:
2989:
2985:
2984:
2982:
2981:
2980:
2979:
2966:
2963:
2962:
2960:
2959:
2958:
2957:
2944:
2942:
2935:
2934:
2932:
2931:
2930:
2929:
2917:
2916:
2915:
2896:
2894:
2880:
2876:
2875:
2866:
2864:
2863:
2856:
2849:
2841:
2832:
2831:
2829:
2828:
2822:
2820:
2816:
2815:
2813:
2812:
2807:
2802:
2797:
2791:
2789:
2782:
2781:
2779:
2778:
2773:
2759:
2754:
2749:
2744:
2739:
2734:
2729:
2724:
2719:
2714:
2709:
2703:
2701:
2689:
2688:
2686:
2685:
2680:
2675:
2670:
2664:
2662:
2650:
2649:
2644:
2642:
2641:
2634:
2627:
2619:
2610:
2609:
2606:
2605:
2594:
2582:
2581:
2579:
2575:
2574:
2571:
2570:
2559:
2548:
2537:
2517:
2502:
2486:
2481:
2480:
2478:
2477:Classification
2469:
2468:
2419:
2384:
2353:(4): 484–492.
2333:
2298:
2284:. 2019-03-06.
2267:
2256:
2221:
2192:(5): e1440–4.
2172:
2137:Blood Advances
2123:
2096:(9): 2613–20.
2078:
2037:
1996:
1946:
1925:(4): 507–519.
1905:
1889:
1854:Am. J. Hematol
1840:
1797:
1770:(6): 1227–35.
1752:
1731:(5): 609–622.
1711:
1676:Oncoimmunology
1659:
1627:
1574:
1523:
1486:(5): 479–485.
1466:
1417:
1366:
1314:
1287:(6): 405–413.
1266:
1216:
1197:(8): e309–13.
1178:
1137:
1086:
1057:
1006:
1005:
1003:
1000:
999:
998:
993:
986:
983:
977:
974:
969:
966:
958:immunoglobulin
949:
946:
906:is also used.
904:immunoglobulin
902:. Intravenous
864:
861:
837:
834:
798:
795:
783:
782:
775:
770:Low or absent
768:
754:
748:
738:
737:
736:
729:
722:
712:
707:
687:
684:
665:
664:Classification
662:
573:
570:
516:
513:
509:
508:
495:
486:
477:
464:
439:
436:
206:
203:
148:
145:
141:cytokine storm
113:hemophagocytic
87:
86:
77:
71:
70:
55:
54:
46:
45:
42:
38:
37:
29:
26:
24:
14:
13:
10:
9:
6:
4:
3:
2:
3474:
3463:
3462:Histiocytosis
3460:
3458:
3455:
3453:
3450:
3449:
3447:
3434:
3433:
3426:
3414:
3411:
3410:
3409:
3408:
3404:
3403:
3400:
3392:
3389:
3388:
3387:
3386:
3382:
3378:
3375:
3374:
3373:
3372:
3368:
3367:
3365:
3362:
3358:
3350:
3346:
3343:
3342:
3341:
3340:
3336:
3332:
3329:
3328:
3327:
3326:
3322:
3321:
3319:
3316:
3312:
3304:
3301:
3300:
3299:
3298:
3294:
3290:
3287:
3286:
3285:
3284:
3280:
3279:
3277:
3274:
3270:
3267:
3265:
3261:
3249:
3246:
3245:
3244:
3243:
3239:
3238:
3236:
3233:
3229:
3221:
3218:
3217:
3216:
3215:
3211:
3210:
3208:
3205:
3201:
3193:
3190:
3189:
3188:
3187:
3183:
3182:
3180:
3177:
3173:
3165:
3162:
3161:
3160:
3159:
3155:
3154:
3152:
3149:
3145:
3142:
3140:
3136:
3124:
3121:
3120:
3119:
3118:
3114:
3110:
3109:Choroideremia
3107:
3106:
3105:
3104:
3100:
3099:
3096:
3088:
3085:
3084:
3083:
3082:
3078:
3077:
3074:
3066:
3063:
3062:
3061:
3060:
3056:
3055:
3052:
3049:
3047:
3043:
3031:
3028:
3027:
3026:
3025:
3021:
3017:
3014:
3013:
3012:
3011:
3007:
3003:
3000:
2999:
2998:
2997:
2993:
2992:
2990:
2986:
2978:
2975:
2974:
2973:
2972:
2968:
2967:
2964:
2956:
2953:
2952:
2951:
2950:
2946:
2945:
2943:
2940:
2936:
2928:
2925:
2924:
2923:
2922:
2918:
2914:
2911:
2910:
2909:
2908:
2903:
2902:
2898:
2897:
2895:
2892:
2888:
2884:
2881:
2877:
2873:
2869:
2862:
2857:
2855:
2850:
2848:
2843:
2842:
2839:
2827:
2824:
2823:
2821:
2817:
2811:
2808:
2806:
2803:
2801:
2798:
2796:
2793:
2792:
2790:
2788:
2783:
2777:
2774:
2771:
2767:
2763:
2760:
2758:
2755:
2753:
2750:
2748:
2745:
2743:
2740:
2738:
2735:
2733:
2730:
2728:
2725:
2723:
2720:
2718:
2715:
2713:
2710:
2708:
2705:
2704:
2702:
2700:
2695:
2690:
2684:
2681:
2679:
2676:
2674:
2671:
2669:
2666:
2665:
2663:
2661:
2656:
2651:
2647:
2646:Histiocytosis
2640:
2635:
2633:
2628:
2626:
2621:
2620:
2617:
2604:
2600:
2599:
2595:
2593:
2589:
2588:
2584:
2583:
2580:
2576:
2569:
2565:
2564:
2560:
2558:
2554:
2553:
2549:
2547:
2543:
2542:
2538:
2536:
2533:
2530:
2527:
2523:
2522:
2518:
2516:
2512:
2511:
2507:
2503:
2501:
2497:
2496:
2492:
2488:
2487:
2484:
2479:
2475:
2464:
2460:
2455:
2450:
2446:
2442:
2438:
2434:
2430:
2423:
2420:
2415:
2411:
2407:
2403:
2399:
2395:
2388:
2385:
2372:
2368:
2364:
2360:
2356:
2352:
2348:
2344:
2337:
2334:
2318:
2311:
2310:
2302:
2299:
2287:
2283:
2282:
2277:
2271:
2268:
2265:
2260:
2257:
2252:
2248:
2244:
2240:
2236:
2232:
2225:
2222:
2217:
2213:
2208:
2203:
2199:
2195:
2191:
2187:
2183:
2176:
2173:
2168:
2164:
2159:
2154:
2150:
2146:
2142:
2138:
2134:
2127:
2124:
2119:
2115:
2111:
2107:
2103:
2099:
2095:
2091:
2090:
2082:
2079:
2074:
2070:
2065:
2060:
2056:
2052:
2048:
2041:
2038:
2033:
2029:
2024:
2019:
2015:
2011:
2007:
2000:
1997:
1992:
1988:
1983:
1978:
1974:
1970:
1966:
1962:
1958:
1950:
1947:
1942:
1938:
1933:
1928:
1924:
1920:
1916:
1909:
1906:
1902:
1898:
1893:
1890:
1885:
1881:
1876:
1871:
1867:
1863:
1859:
1855:
1851:
1844:
1841:
1836:
1832:
1827:
1822:
1818:
1814:
1813:
1808:
1801:
1798:
1793:
1789:
1785:
1781:
1777:
1773:
1769:
1765:
1764:
1756:
1753:
1748:
1744:
1739:
1734:
1730:
1726:
1722:
1715:
1712:
1707:
1703:
1698:
1693:
1689:
1685:
1682:(4): e24185.
1681:
1677:
1673:
1669:
1663:
1660:
1655:
1651:
1647:
1646:
1641:
1634:
1632:
1628:
1623:
1619:
1614:
1609:
1604:
1599:
1595:
1591:
1590:
1585:
1578:
1575:
1570:
1566:
1561:
1556:
1552:
1548:
1544:
1540:
1539:
1534:
1527:
1524:
1519:
1515:
1511:
1507:
1502:
1497:
1493:
1489:
1485:
1481:
1477:
1470:
1467:
1462:
1458:
1453:
1448:
1444:
1440:
1436:
1432:
1428:
1421:
1418:
1413:
1409:
1404:
1399:
1394:
1389:
1385:
1381:
1377:
1370:
1367:
1362:
1358:
1353:
1348:
1344:
1340:
1337:(7): 978–88.
1336:
1332:
1331:
1330:Haematologica
1326:
1318:
1315:
1310:
1306:
1302:
1298:
1294:
1290:
1286:
1282:
1281:
1273:
1271:
1267:
1262:
1258:
1254:
1250:
1246:
1242:
1238:
1234:
1233:Rev Med Virol
1230:
1223:
1221:
1217:
1212:
1208:
1204:
1200:
1196:
1192:
1185:
1183:
1179:
1174:
1170:
1165:
1160:
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320:
316:
312:
308:
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304:
299:
297:
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291:
289:
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281:
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269:
265:
261:
257:
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248:
245:(EBV) or the
244:
240:
236:
232:
228:
224:
219:
217:
213:
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200:
199:transaminitis
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188:
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110:
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67:H&E stain
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2054:
2050:
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1964:
1960:
1949:
1922:
1918:
1908:
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1810:
1800:
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1236:
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1069:
1060:
1028:(6): 601–8.
1025:
1020:
1010:
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971:
955:
951:
933:
927:
920:
908:Methotrexate
889:
885:
878:
866:
857:melanophilin
846:
839:
826:
815:
800:
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784:
698:
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692:
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637:
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480:
467:
459:
453:
448:
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300:
293:
250:
247:Dengue virus
220:
208:
176:
157:
153:
150:
116:
112:
104:
100:
96:
90:
3176:Microtubule
2598:GeneReviews
1967:(3): e560.
1645:GeneReviews
936:, allowing
930:NHS England
912:vincristine
900:cyclosporin
766:lymph nodes
758:bone marrow
733:neutropenia
586:macrophages
582:bone marrow
564:leading to
430:and termed
352:, juvenile
133:macrophages
129:lymphocytes
41:Other names
3446:Categories
2891:Melanosome
2552:DiseasesDB
2394:The Lancet
2326:14 October
2292:2018-11-20
2186:Pediatrics
1668:Trapani JA
1656:. NBK1444.
1380:The Lancet
1106:(1): 215.
1080:2021-01-24
1002:References
932:published
923:emapalumab
832:activity.
745:fibrinogen
640:fibrinogen
638:The serum
623:The serum
485:(Munc13-4)
413:iatrogenic
409:SARS-CoV-2
127:of benign
93:hematology
84:immunology
80:Hematology
59:Micrograph
3430:See also
2819:Ungrouped
2587:eMedicine
2568:234437005
2563:SNOMED CT
1518:246488008
948:Prognosis
896:etoposide
863:Treatment
805:or other
610:platelets
572:Diagnosis
546:TNF-gamma
538:cytopenia
534:TNF-alpha
507:)/UNC18-2
303:malignant
241:like the
239:pathogens
164:cytopenia
75:Specialty
3315:Caveolae
3232:Spectrin
2977:CDOG IIE
2887:Lysosome
2785:WHO-III/
2463:13008468
2371:Archived
2367:24581757
2286:Archived
2251:28477737
2237:: 1–12.
2216:24101757
2167:29296904
2118:43419202
2110:24782338
2073:25758828
2032:18679369
1991:31044148
1941:34347856
1884:23828274
1835:25573993
1792:25546028
1784:18085676
1747:23577835
1706:23734337
1654:20301617
1622:29358936
1596:: 1902.
1569:28621800
1510:35108473
1461:33264547
1412:32192578
1361:26022711
1309:11439142
1301:28957822
1261:52002485
1253:30109914
1211:28806468
1173:32134401
1132:27389585
1074:Archived
1052:11076718
985:See also
976:Research
938:Anakinra
777:Soluble
774:activity
751:Ferritin
633:ferritin
558:ferritin
474:Perforin
449:in utero
438:Genetics
432:EBV+ HLH
401:protozoa
397:bacteria
393:HIV/AIDS
367:such as
235:NK cells
193:, and a
61:showing
3204:Kinesin
2692:WHO-II/
2592:ped/745
2546:D051359
2454:1988563
2414:6131175
2207:3813387
2158:5728644
1982:6467688
1875:4348111
1697:3654607
1613:5766650
1560:5568927
1501:8830531
1452:7727315
1403:7270045
1352:4486233
1123:4937543
1043:2640913
968:History
830:NK cell
644:D-dimer
618:albumin
522:granule
344:and in
174:wards.
111:), and
3385:VPS13B
3371:VPS33B
3283:SNAP29
3242:SPTBN2
3148:Myosin
3081:RAB27A
2949:SEC23A
2653:WHO-I/
2535:603552
2532:608898
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1979:
1939:
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1538:Cancer
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791:HScore
762:spleen
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631:, and
608:, and
550:lipase
536:; the
500:STXBP2
497:FHL5:
488:FHL4:
482:UNC13D
479:FHL3:
466:FHL2:
458:FHL1:
420:SH2D1A
383:, and
356:, and
325:, and
276:SH2D1A
264:STXBP2
260:RAB27A
252:UNC13D
205:Causes
3297:STX11
3214:KIF5A
3158:MYO5A
3030:CPSQ3
3024:AP4M1
3010:AP3B1
2996:AP1S2
2939:COPII
2557:31418
2515:288.4
2500:D76.1
2320:(PDF)
2313:(PDF)
2114:S2CID
2051:Blood
1812:Blood
1788:S2CID
1514:S2CID
1305:S2CID
1257:S2CID
870:shock
849:MYO5A
704:Fever
491:STX11
461:HPLH1
422:(see
405:fungi
295:MAGT1
280:BIRC4
256:STX11
179:fever
3407:DYSF
3339:CAV3
3325:CAV1
3186:SPG4
3117:MLPH
3065:BBS3
3059:ARL6
2988:APC:
2971:COG7
2921:LYST
2907:HPS7
2901:HPS1
2541:MeSH
2521:OMIM
2510:9-CM
2459:PMID
2410:PMID
2379:2015
2363:PMID
2328:2021
2247:PMID
2212:PMID
2163:PMID
2106:PMID
2069:PMID
2028:PMID
1987:PMID
1937:PMID
1880:PMID
1831:PMID
1780:PMID
1743:PMID
1702:PMID
1650:PMID
1618:PMID
1565:PMID
1506:PMID
1457:PMID
1408:PMID
1357:PMID
1297:PMID
1249:PMID
1207:PMID
1169:PMID
1128:PMID
1048:PMID
910:and
898:and
779:CD25
649:The
560:and
532:and
530:IL-6
526:IL-1
469:PRF1
407:and
340:and
292:and
288:CD27
272:PRF1
268:LYST
233:and
195:rash
166:and
131:and
3103:CHM
3046:Rab
2506:ICD
2491:ICD
2449:PMC
2441:doi
2402:doi
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2355:doi
2239:doi
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2202:PMC
2194:doi
2190:132
2153:PMC
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1977:PMC
1969:doi
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1030:doi
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