Cover Image

Hemophagocytic Lympho Histiocytosis (HLH)

Nijwm Mahilary, Rajib Ray, Rameswar Prasad Mishra, Raj Kumar Paul*


Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyper-inflammatory condition caused by a highly stimulated, but ineffective immune response, characterized by a dys-regulated activation and proliferation of macrophages leading to uncontrolled phagocytosis of platelets, erythrocytes, lymphocytes and their hematopoietic precursors throughout the reticulo-endothelial system. HLH is a distinct clinical entity characterized by fever, pancytopenia, splenomegaly and hemophagocytosis in bone marrow, liver, or lymph nodes. HLH also occurs as a complication of rheumatic diseases and of malignancies. Awareness of the clinical symptoms and of diagnostic criteria for HLH (Histiocyte society; HLH study 2004) is crucial to starting life-saving therapy in time. We have reported a case of 7-year-old male child presenting with respiratory distress, distension of abdomen and deep jaundice. This case report may be an eye opener to the pediatricians and other physicians to recognize this rare entity of infection triggering fatal HLH and thus proper treatment may be instituted in those affected with this disease at the earliest.


Fever; hepatosplenomegaly; lymphadenopathy; hemophagocytes in bone marrow; pancytopenia

Full Text:



Henter JI, Elinder G. Incidence in sweden and c/f hemophagocytic lymphohistiocytosis. Acta paediatr Scand. 1991; 80:428-435).

Janka G. Hemophagocytic lymphohistiocytosis, Klin Padiatr: 2009;221:278-285

Stephan Ladisch, 501 Class II Histiocytosis Syndrome of Childhood: Nelson textbook of pediatrics 19th edition volume II:pg:1776-1777 : pg:1774

Nathan DG, Orkin SH, Ginsburg D, et al., editors: Nathan Oskis hematology of infancy and childhood, ed 6; Philadelphia 2003, WB Saunders, p 1381.

David N. Fisman; Hemophagocytic Syndomes and Infection: vol.6, no.6, November-December 2000; pg: 604.

Ravelli A. MAS. Curr opin Rheumatol 2002; 14:548-552

Emmenegger U Reimers A, Frey U, et al., reactive macrophage activation syndrome: a simple screening strategy and its potential in early treatment initiation. Swiss Med Wkly 2002; 132:230-236

Henter Jl, Arico M Egeler M, et al., HLH 94: a treatment protocol for hemophagocytic lymphohistiocytosis. Med Pediatr Oncol 1997; 28:342-347.

Henter Jl, Arico M Egeler M, et al., Samuelsson-Horne A: Treatment protocol for hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood 2002; 100:2367-2373.

Seidel Mg, Kastiner U, Minkow M, Gadner H. IVIG treatment of adenovirus infection associated macrophage activation syndrome in a two-year-old boy: case report and review of the literature. Pediatr Hematol 2002; 44: 259-272.

Matsumoto Y, Naniwa D, Banno S, Sugiura Y. The efficacy of the therapeutic plasmapheresis for the treatment of fatal hemophagocytic syndrome: two case report. The Apher 1998; 300-304.

Henter Jl, Samuelsson- Horne A, Arico M, et al., treatment of Hemophagocytic Lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood. Oct 1 2002; 100 (7): 2367-73.



  • There are currently no refbacks.

Copyright (c) 2015 International Journal of Bioassays

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

International Journal of Bioassays is a member of the Publishers International Linking Association, Inc. (PILA), CROSSREF and CROSSMARK (USA). Digital Object Identifier (DOI) will be assigned to all its published content.