A HLH is a rare fatal condition easily misdiagnosed as infectious disease


  • Md. Khaja Moinuddin Department of Pediatrics, Bangalore Medical College and Research Institute (BMCRI), Bangalore, Karnataka, India
  • Premalatha R. Department of Pediatrics, Bangalore Medical College and Research Institute (BMCRI), Bangalore, Karnataka, India




Bone marrow, Hemophagocytic lymphohistiocytosis, Macrophages


Hemophagocytic lymphohistiocytosis (HLH) is characterized by clonal proliferation of the morphologically normal antigen processing cells, i.e. macrophages and CD8 lymphocytes in the involved tissue, due to uncontrolled activation of inflammatory cytokines. Two major forms include Familial HLH and infection associated hemophagocytic syndrome. Both these forms of HLH proceed with a generalized disease process i.e. fever, rashes and weight loss. Hence it is easily misdiagnosed as any infectious disease. So, a high index of suspicion is required for the diagnosis. With the help of clinical and laboratory criteria we can easily diagnose it without any genetic testing. 


Kliegman RM, Stanton BF, St. Geme JW, Nelson N, Textbook of Paediatrics First South Asia Edition, Chapter 507, 2016:2484-249.

Castillo L, Carcillo J. Secondary hemophagocytic lymphohistiocytosis and severe sepsis/ systemic inflammatory response syndrome/ multiorgan dysfunction syndrome/macrophage activation syndrome share common intermediate phenotypes on a spectrum of inflammation. Pediatr Crit Care Med. 2009;10:387-92.

Jan-Inge H, AnnaCarin H, Maurizio A, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: Diagnostic and Therapeutic Guidelines for Hemophagocytic Lymphohistiocytosis, 2006 Wiley-Liss, Inc.

Scott R, Robb Smith A. Histiocytic medullary reticulocytosis. Lancet 1939;2:194-8.

Fisman DN, Hemophagocytic Syndromes and Infection.Emerging infectious diseases. 2000;6:601-8.

Raschke RA, Garcia-Orr R. Hemophagocytic Lymphohistiocytosis A Potentially Underrecognized Association With Systemic Inflammatory Response Syndrome, Severe Sepsis, and Septic Shock in Adults. Chest. 2011;140:933-8.

Henter JI, Horne A, Arcio M. HLH 2004, Diagnostic and therapeutic guidelines for Hemophagocytic Lymphohistiocytosis. Pediatric Blood Cancer. 2007;48:124-31.






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