U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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 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

Recent clinical studies

Etiology

Management of hematological patients requiring emergency chemotherapy in the intensive care unit.
Lafarge A, Chean D, Whiting L, Clere-Jehl R; Groupe de Recherche en Réanimation Respiratoire du patient d’Onco-Hématologie (GRRR-OH); Clinical Research in Intensive Care and Sepsis - TRIal Group for Global Evaluation and Research in SEPsis (CRICS-TRIGGERSEP)
Intensive Care Med 2024 Jun;50(6):849-860. Epub 2024 May 15 doi: 10.1007/s00134-024-07454-z. PMID: 38748265Free PMC Article
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
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

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

Management of hematological patients requiring emergency chemotherapy in the intensive care unit.
Lafarge A, Chean D, Whiting L, Clere-Jehl R; Groupe de Recherche en Réanimation Respiratoire du patient d’Onco-Hématologie (GRRR-OH); Clinical Research in Intensive Care and Sepsis - TRIal Group for Global Evaluation and Research in SEPsis (CRICS-TRIGGERSEP)
Intensive Care Med 2024 Jun;50(6):849-860. Epub 2024 May 15 doi: 10.1007/s00134-024-07454-z. PMID: 38748265Free PMC Article
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
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
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

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
Can we truly diagnose adult secondary hemophagocytic lymphohistiocytosis (HLH)? A critical review of current paradigms.
Naymagon L
Pathol Res Pract 2021 Feb;218:153321. Epub 2020 Dec 17 doi: 10.1016/j.prp.2020.153321. PMID: 33418346
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
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

Clinical prediction guides

Management of hematological patients requiring emergency chemotherapy in the intensive care unit.
Lafarge A, Chean D, Whiting L, Clere-Jehl R; Groupe de Recherche en Réanimation Respiratoire du patient d’Onco-Hématologie (GRRR-OH); Clinical Research in Intensive Care and Sepsis - TRIal Group for Global Evaluation and Research in SEPsis (CRICS-TRIGGERSEP)
Intensive Care Med 2024 Jun;50(6):849-860. Epub 2024 May 15 doi: 10.1007/s00134-024-07454-z. PMID: 38748265Free PMC Article
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
Can we truly diagnose adult secondary hemophagocytic lymphohistiocytosis (HLH)? A critical review of current paradigms.
Naymagon L
Pathol Res Pract 2021 Feb;218:153321. Epub 2020 Dec 17 doi: 10.1016/j.prp.2020.153321. PMID: 33418346
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

Human monocytotropic ehrlichiosis-A systematic review and analysis of the literature.
Gygax L, Schudel S, Kositz C, Kuenzli E, Neumayr A
PLoS Negl Trop Dis 2024 Aug;18(8):e0012377. Epub 2024 Aug 2 doi: 10.1371/journal.pntd.0012377. PMID: 39093857Free PMC Article
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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Related information

    • GTR
      Related information in GTR
    • GTR(Clinical)
      Clinical tests in GTR
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...