» Articles » PMID: 36631166

Coagulopathy is an Under-recognised Complication of Acquired HLH

Overview
Journal BMJ Case Rep
Specialty General Medicine
Date 2023 Jan 11
PMID 36631166
Authors
Affiliations
Soon will be listed here.
Abstract

Acquired haemophagocytic lymphohistiocytosis (aHLH) is a rare and often fatal process of uncontrolled cytokine release driven by the inability of natural killer cells to eliminate infected or malignant cells. Herein, we report two cases of aHLH complicated by bleeding secondary to coagulopathy due to hypofibrinogenaemia and thrombocytopenia despite appropriate correction with blood products. These cases highlight the effect coagulopathy and thrombocytopenia can have on patient outcomes when trying to confirm and manage the underlying process driving aHLH.

Citing Articles

Hyperferritinemia is Associated with Thrombocytopenia and Increased Mortality Risk in Adult Sepsis Patients: A Retrospective Study of Two Observational Cohorts.

Li D, Li X, Wen B, Li B, Wang Y, Zong Y Clin Appl Thromb Hemost. 2025; 31:10760296251321314.

PMID: 39957136 PMC: 11831685. DOI: 10.1177/10760296251321314.

References
1.
Usmani G, Woda B, Newburger P . Advances in understanding the pathogenesis of HLH. Br J Haematol. 2013; 161(5):609-622. DOI: 10.1111/bjh.12293. View

2.
Henter J, Horne A, Arico M, Egeler R, Filipovich A, Imashuku S . HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2006; 48(2):124-31. DOI: 10.1002/pbc.21039. View

3.
Wikkelso A, Wetterslev J, Moller A, Afshari A . Thromboelastography (TEG) or thromboelastometry (ROTEM) to monitor haemostatic treatment versus usual care in adults or children with bleeding. Cochrane Database Syst Rev. 2016; (8):CD007871. PMC: 6472507. DOI: 10.1002/14651858.CD007871.pub3. View

4.
Valade S, Azoulay E, Galicier L, Boutboul D, Zafrani L, Stepanian A . Coagulation Disorders and Bleedings in Critically Ill Patients With Hemophagocytic Lymphohistiocytosis. Medicine (Baltimore). 2015; 94(40):e1692. PMC: 4616770. DOI: 10.1097/MD.0000000000001692. View

5.
Fardet L, Galicier L, Lambotte O, Marzac C, Aumont C, Chahwan D . Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol. 2014; 66(9):2613-20. DOI: 10.1002/art.38690. View