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Secondary hemophagocytic lymphohistiocytosis

MedGen UID:
883550
•Concept ID:
C4054044
•
Disease or Syndrome
Synonyms: Acquired hemophagocytic lymphohistiocytosis; acquired hemophagocytic lymphohistiocytosis; acquired hemophagocytic syndrome; Reactive hemophagocytic syndrome; reactive hemophagocytic syndrome; Secondary Hemophagocytic Lymphohistiocytosis; secondary hemophagocytic lymphohistiocytosis
SNOMED CT: Secondary hemophagocytic lymphohistiocytosis (1153417000)
 
Monarch Initiative: MONDO:0015542
Orphanet: ORPHA158041

Definition

Hemophagocytic lymphohistiocytosis due to infections, autoimmune disorders, or underlying malignancies. Signs and symptoms include fever, lymphadenopathy, hepatomegaly, splenomegaly, and pancytopenia. [from NCI]

Professional guidelines

PubMed

Management of Dengue: An Updated Review.
Tayal A, Kabra SK, Lodha R
Indian J Pediatr 2023 Feb;90(2):168-177. Epub 2022 Dec 27 doi: 10.1007/s12098-022-04394-8. PMID: 36574088Free PMC Article
Consensus-Based Guidelines for the Recognition, Diagnosis, and Management of Hemophagocytic Lymphohistiocytosis in Critically Ill Children and Adults.
Hines MR, von Bahr Greenwood T, Beutel G, Beutel K, Hays JA, Horne A, Janka G, Jordan MB, van Laar JAM, Lachmann G, Lehmberg K, Machowicz R, Miettunen P, La Rosée P, Shakoory B, Zinter MS, Henter JI
Crit Care Med 2022 May 1;50(5):860-872. Epub 2021 Oct 5 doi: 10.1097/CCM.0000000000005361. PMID: 34605776
Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Childhood Inflammatory Disorders: Diagnosis and Management.
Henderson LA, Cron RQ
Paediatr Drugs 2020 Feb;22(1):29-44. doi: 10.1007/s40272-019-00367-1. PMID: 31732958Free PMC Article

Recent clinical studies

Etiology

Risk factors and prognosis of early death in secondary hemophagocytic lymphohistiocytosis.
Zhang Q, Zhu L, Zhou D, Li L, Xie W, Tan Y, Ye X
Ann Hematol 2023 Sep;102(9):2301-2308. Epub 2023 Jun 2 doi: 10.1007/s00277-023-05153-1. PMID: 37266671
Virus-triggered secondary hemophagocytic lymphohistiocytosis.
Imashuku S, Morimoto A, Ishii E
Acta Paediatr 2021 Oct;110(10):2729-2736. Epub 2021 Jun 22 doi: 10.1111/apa.15973. PMID: 34096649
The use of anakinra in the treatment of secondary hemophagocytic lymphohistiocytosis.
Bami S, Vagrecha A, Soberman D, Badawi M, Cannone D, Lipton JM, Cron RQ, Levy CF
Pediatr Blood Cancer 2020 Nov;67(11):e28581. Epub 2020 Jul 29 doi: 10.1002/pbc.28581. PMID: 32725881
Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Childhood Inflammatory Disorders: Diagnosis and Management.
Henderson LA, Cron RQ
Paediatr Drugs 2020 Feb;22(1):29-44. doi: 10.1007/s40272-019-00367-1. PMID: 31732958Free PMC Article
Viral etiology, clinical and laboratory features of adult hemophagocytic lymphohistiocytosis.
Chen J, Wang X, He P, Li Y, Si M, Fan Z, Chang X, Xie Q, Jiao X
J Med Virol 2016 Mar;88(3):541-9. Epub 2015 Oct 19 doi: 10.1002/jmv.24359. PMID: 26287378Free PMC Article

Diagnosis

Management of Dengue: An Updated Review.
Tayal A, Kabra SK, Lodha R
Indian J Pediatr 2023 Feb;90(2):168-177. Epub 2022 Dec 27 doi: 10.1007/s12098-022-04394-8. PMID: 36574088Free PMC Article
Consensus-Based Guidelines for the Recognition, Diagnosis, and Management of Hemophagocytic Lymphohistiocytosis in Critically Ill Children and Adults.
Hines MR, von Bahr Greenwood T, Beutel G, Beutel K, Hays JA, Horne A, Janka G, Jordan MB, van Laar JAM, Lachmann G, Lehmberg K, Machowicz R, Miettunen P, La Rosée P, Shakoory B, Zinter MS, Henter JI
Crit Care Med 2022 May 1;50(5):860-872. Epub 2021 Oct 5 doi: 10.1097/CCM.0000000000005361. PMID: 34605776
Virus-triggered secondary hemophagocytic lymphohistiocytosis.
Imashuku S, Morimoto A, Ishii E
Acta Paediatr 2021 Oct;110(10):2729-2736. Epub 2021 Jun 22 doi: 10.1111/apa.15973. PMID: 34096649
Macrophage Activation Syndrome in Children: Diagnosis and Management.
Bagri NK, Gupta L, Sen ES, Ramanan AV
Indian Pediatr 2021 Dec 15;58(12):1155-1161. Epub 2021 Mar 26 doi: 10.1007/s13312-021-2399-8. PMID: 33772536Free PMC Article
Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Childhood Inflammatory Disorders: Diagnosis and Management.
Henderson LA, Cron RQ
Paediatr Drugs 2020 Feb;22(1):29-44. doi: 10.1007/s40272-019-00367-1. PMID: 31732958Free PMC Article

Therapy

Anakinra versus etoposide-based therapy added to high-dose steroids for the treatment of secondary hemophagocytic lymphohistiocytosis.
Lee BJ, Cao Y, Vittayawacharin P, É'Leima G, Rezk S, Reid J, Brem EA, Ciurea SO, Kongtim P
Eur J Haematol 2023 Sep;111(3):477-484. Epub 2023 Jun 29 doi: 10.1111/ejh.14030. PMID: 37385631
Management of Dengue: An Updated Review.
Tayal A, Kabra SK, Lodha R
Indian J Pediatr 2023 Feb;90(2):168-177. Epub 2022 Dec 27 doi: 10.1007/s12098-022-04394-8. PMID: 36574088Free PMC Article
Is COVID-19-associated cytokine storm distinct from non-COVID-19 secondary hemophagocytic lymphohistiocytosis?
Liu JM, Chi J
Exp Biol Med (Maywood) 2022 Feb;247(4):330-337. Epub 2022 Jan 22 doi: 10.1177/15353702211068840. PMID: 35068219Free PMC Article
The use of anakinra in the treatment of secondary hemophagocytic lymphohistiocytosis.
Bami S, Vagrecha A, Soberman D, Badawi M, Cannone D, Lipton JM, Cron RQ, Levy CF
Pediatr Blood Cancer 2020 Nov;67(11):e28581. Epub 2020 Jul 29 doi: 10.1002/pbc.28581. PMID: 32725881
Macrophage Activation Syndrome.
Sen ES, Clarke SL, Ramanan AV
Indian J Pediatr 2016 Mar;83(3):248-53. Epub 2015 Sep 24 doi: 10.1007/s12098-015-1877-1. PMID: 26400031

Prognosis

Genetic and clinical characteristics of primary hemophagocytic lymphohistiocytosis in children.
Zhao C, Zhang Q, Zhang R, Lian H, Ma H, Zhao X, Li Z
Ann Hematol 2024 Jan;103(1):17-28. Epub 2023 Oct 18 doi: 10.1007/s00277-023-05499-6. PMID: 37851074
Risk factors and prognosis of early death in secondary hemophagocytic lymphohistiocytosis.
Zhang Q, Zhu L, Zhou D, Li L, Xie W, Tan Y, Ye X
Ann Hematol 2023 Sep;102(9):2301-2308. Epub 2023 Jun 2 doi: 10.1007/s00277-023-05153-1. PMID: 37266671
Characteristics and prognostic value of pleural effusion in secondary hemophagocytic lymphohistiocytosis.
Cheng W, Gao X, Yin G, Huang J, Man C, Qiu H
Int J Hematol 2022 Jul;116(1):102-109. Epub 2022 Mar 25 doi: 10.1007/s12185-022-03333-9. PMID: 35338447Free PMC Article
The use of anakinra in the treatment of secondary hemophagocytic lymphohistiocytosis.
Bami S, Vagrecha A, Soberman D, Badawi M, Cannone D, Lipton JM, Cron RQ, Levy CF
Pediatr Blood Cancer 2020 Nov;67(11):e28581. Epub 2020 Jul 29 doi: 10.1002/pbc.28581. PMID: 32725881
Viral etiology, clinical and laboratory features of adult hemophagocytic lymphohistiocytosis.
Chen J, Wang X, He P, Li Y, Si M, Fan Z, Chang X, Xie Q, Jiao X
J Med Virol 2016 Mar;88(3):541-9. Epub 2015 Oct 19 doi: 10.1002/jmv.24359. PMID: 26287378Free PMC Article

Clinical prediction guides

Comprehensive evaluation of immune dysregulation in secondary hemophagocytic lymphohistiocytosis.
Wang Y, Yuan X, Wang T, Wei W, Wu S, Hou H
Virulence 2024 Dec;15(1):2342276. Epub 2024 Apr 17 doi: 10.1080/21505594.2024.2342276. PMID: 38629410Free PMC Article
Genetic and clinical characteristics of primary hemophagocytic lymphohistiocytosis in children.
Zhao C, Zhang Q, Zhang R, Lian H, Ma H, Zhao X, Li Z
Ann Hematol 2024 Jan;103(1):17-28. Epub 2023 Oct 18 doi: 10.1007/s00277-023-05499-6. PMID: 37851074
Hemophagocytic lymphohistiocytosis: A scourge for the physician and bane to the bone marrow.
Krishnan G, Gosavi S, Gujral M, Basheer N, Kumar B, Jain P
Ann Afr Med 2023 Oct-Dec;22(4):532-536. doi: 10.4103/aam.aam_11_23. PMID: 38358157Free PMC Article
Current evidence on the use of anakinra in COVID-19.
Khani E, Shahrabi M, Rezaei H, Pourkarim F, Afsharirad H, Solduzian M
Int Immunopharmacol 2022 Oct;111:109075. Epub 2022 Jul 20 doi: 10.1016/j.intimp.2022.109075. PMID: 35905562Free PMC Article
Primary and secondary hemophagocytic lymphohistiocytosis: clinical features, pathogenesis and therapy.
Gupta S, Weitzman S
Expert Rev Clin Immunol 2010 Jan;6(1):137-54. doi: 10.1586/eci.09.58. PMID: 20383897

Recent systematic reviews

Immunopathology of and potential therapeutics for secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome: a translational perspective.
Nguyen TTT, Kim YT, Jeong G, Jin M
Exp Mol Med 2024 Mar;56(3):559-569. Epub 2024 Mar 6 doi: 10.1038/s12276-024-01182-6. PMID: 38448692Free PMC Article
Ferritin in the coronavirus disease 2019 (COVID-19): A systematic review and meta-analysis.
Cheng L, Li H, Li L, Liu C, Yan S, Chen H, Li Y
J Clin Lab Anal 2020 Oct;34(10):e23618. Epub 2020 Oct 19 doi: 10.1002/jcla.23618. PMID: 33078400Free PMC Article
Kawasaki Disease Complicated With Macrophage Activation Syndrome: A Systematic Review.
García-Pavón S, Yamazaki-Nakashimada MA, Báez M, Borjas-Aguilar KL, Murata C
J Pediatr Hematol Oncol 2017 Aug;39(6):445-451. doi: 10.1097/MPH.0000000000000872. PMID: 28562511
Secondary hemophagocytic lymphohistiocytosis in zoonoses. A systematic review.
Cascio A, Pernice LM, Barberi G, Delfino D, Biondo C, Beninati C, Mancuso G, Rodriguez-Morales AJ, Iaria C
Eur Rev Med Pharmacol Sci 2012 Oct;16(10):1324-37. PMID: 23104648

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