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Hemophagocytic lymphohistiocytosis in children with chronic granulomatous disease
Author(s) -
Parekh Chintan,
Hofstra Thomas,
Church Joseph A.,
Coates Thomas D.
Publication year - 2011
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.22830
Subject(s) - hemophagocytosis , chronic granulomatous disease , hemophagocytic lymphohistiocytosis , medicine , macrophage activation syndrome , immunology , disease , arthritis , bone marrow , pancytopenia
Hemophagocytic lymphohistiocytosis (HLH) is characterized by fever, cytopenias, splenomegaly, and hemophagocytosis by macrophages activated by high cytokine levels. Chronic granulomatous disease (CGD) is characterized by recurrent infections, hyperinflammation, and excessive cytokine release. This may predispose patients with CGD to developing HLH during an infection. We conducted a retrospective review of patients with CGD, treated at our institution between 1999 and 2008. Three out of 17 patients developed HLH. Patients with CGD may be at increased risk for developing HLH. Remission of HLH was achieved after treatment with antimicrobials, steroids, and intravenous immunoglobulin This approach to treatment appears to be effective. Pediatr Blood Cancer 2011;56:460–462. © 2010 Wiley‐Liss, Inc.