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Acquired hemophagocytic lymphohistiocytosis associated with malignant disease

MedGen UID:
1683300
Concept ID:
C5190710
Disease or Syndrome
Synonym: acquired hemophagocytic lymphohistiocytosis associated with malignant disease
SNOMED CT: Acquired hemophagocytic lymphohistiocytosis associated with malignant disease (782915004)
 
Monarch Initiative: MONDO:0015544
Orphanet: ORPHA158057

Definition

A rare secondary haemophagocytic lymphohistiocytosis characterised by occurring as either initial presentation of a malignant disease or at any stage during chemotherapy. The common associated malignancies are leukaemias, B-cell, T-cell or NK-cell lymphomas, and Hodgkin lymphoma. Typical clinical manifestation includes fever, hepatosplenomegaly and cytopenias, combined with specific laboratory findings. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAcquired hemophagocytic lymphohistiocytosis associated with malignant disease

Professional guidelines

PubMed

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
Janka GE, Lehmberg K
Hematology Am Soc Hematol Educ Program 2013;2013:605-11. doi: 10.1182/asheducation-2013.1.605. PMID: 24319239

Recent clinical studies

Etiology

Bains A, Mamone L, Aneja A, Bromberg M
Ann Diagn Pathol 2017 Jun;28:37-42. Epub 2017 Feb 20 doi: 10.1016/j.anndiagpath.2017.02.009. PMID: 28648938
Janka GE, Lehmberg K
Hematology Am Soc Hematol Educ Program 2013;2013:605-11. doi: 10.1182/asheducation-2013.1.605. PMID: 24319239
Subramaniam K, D'Rozario J, Pavli P
J Gastroenterol Hepatol 2013 Jan;28(1):24-30. doi: 10.1111/jgh.12015. PMID: 23094824
Janka GE
Eur J Pediatr 2007 Feb;166(2):95-109. Epub 2006 Dec 7 doi: 10.1007/s00431-006-0258-1. PMID: 17151879
Singh ZN, Rakheja D, Yadav TP, Shome DK
Clin Lab Haematol 2005 Oct;27(5):312-5. doi: 10.1111/j.1365-2257.2005.00717.x. PMID: 16178911

Diagnosis

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
Suleman N, Ozdemirli M, Weisman D
BMJ Case Rep 2021 Jan 6;14(1) doi: 10.1136/bcr-2020-238183. PMID: 33408106Free PMC Article
Altook R, Ruzieh M, Singh A, Alamoudi W, Moussa Z, Alim H, Safi F, Duggan J
Am J Med Sci 2019 Jan;357(1):67-74. Epub 2018 Jul 17 doi: 10.1016/j.amjms.2018.07.004. PMID: 30278875
Bhattacharyya M, Ghosh MK
J Assoc Physicians India 2008 Jun;56:453-7. PMID: 18822626
Janka GE
Eur J Pediatr 2007 Feb;166(2):95-109. Epub 2006 Dec 7 doi: 10.1007/s00431-006-0258-1. PMID: 17151879

Therapy

Allahverdi Nazhand H, Sabeti S, Javandoust Gharehbagh F, Nalini R, Babamahmoodi A, Marahemi M, Mahmoudi Chalmiani E, Lotfollahi L, Alavi Darazam I
J Infect Dev Ctries 2023 Nov 30;17(11):1647-1653. doi: 10.3855/jidc.17303. PMID: 38064397
Suleman N, Ozdemirli M, Weisman D
BMJ Case Rep 2021 Jan 6;14(1) doi: 10.1136/bcr-2020-238183. PMID: 33408106Free PMC Article
Subramaniam K, D'Rozario J, Pavli P
J Gastroenterol Hepatol 2013 Jan;28(1):24-30. doi: 10.1111/jgh.12015. PMID: 23094824
Janka GE
Annu Rev Med 2012;63:233-46. doi: 10.1146/annurev-med-041610-134208. PMID: 22248322
Bhattacharyya M, Ghosh MK
J Assoc Physicians India 2008 Jun;56:453-7. PMID: 18822626

Prognosis

Bains A, Mamone L, Aneja A, Bromberg M
Ann Diagn Pathol 2017 Jun;28:37-42. Epub 2017 Feb 20 doi: 10.1016/j.anndiagpath.2017.02.009. PMID: 28648938
Magaki S, Ostrzega N, Ho E, Yim C, Wu P, Vinters HV
Hum Pathol 2017 Jan;59:108-112. Epub 2016 Aug 27 doi: 10.1016/j.humpath.2016.07.033. PMID: 27574808
Smith MC, Cohen DN, Greig B, Yenamandra A, Vnencak-Jones C, Thompson MA, Kim AS
Int J Clin Exp Pathol 2014;7(9):5738-49. Epub 2014 Aug 15 PMID: 25337215Free PMC Article
Price B, Lines J, Lewis D, Holland N
S Afr Med J 2014 May 12;104(6):401-6. doi: 10.7196/samj.7810. PMID: 25214245
Janka G, zur Stadt U
Hematology Am Soc Hematol Educ Program 2005:82-8. doi: 10.1182/asheducation-2005.1.82. PMID: 16304363

Clinical prediction guides

Govindarajan A, Venter F, Chaudhry A, Kaur H, Cobos E, Petersen G
J Investig Med High Impact Case Rep 2024 Jan-Dec;12:23247096241273131. doi: 10.1177/23247096241273131. PMID: 39345128Free PMC Article
Suleman N, Ozdemirli M, Weisman D
BMJ Case Rep 2021 Jan 6;14(1) doi: 10.1136/bcr-2020-238183. PMID: 33408106Free PMC Article
Tangye SG, Palendira U, Edwards ES
J Exp Med 2017 Feb;214(2):269-283. Epub 2017 Jan 20 doi: 10.1084/jem.20161846. PMID: 28108590Free PMC Article
Schneier A, Stueck AE, Petersen B, Thung SN, Perumalswami P
Semin Liver Dis 2016 Feb;36(1):99-105. Epub 2016 Feb 12 doi: 10.1055/s-0036-1571299. PMID: 26870936
Smith MC, Cohen DN, Greig B, Yenamandra A, Vnencak-Jones C, Thompson MA, Kim AS
Int J Clin Exp Pathol 2014;7(9):5738-49. Epub 2014 Aug 15 PMID: 25337215Free PMC Article

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