Fever, Splenomegaly, and Pancytopenia: Histoplasma-Associated Hemophagocytic Lymphohistiocytosis
KEY WORDSinfectious diseases hematology clinical images
HLH is a hyperinflammatory disorder caused by uncontrolled proliferation of activated lymphocytes and macrophages secreting inflammatory cytokines.1 Although a primary genetic form exists, secondary HLH is often associated with immunodeficiency, malignancy, and infection, commonly in patients with AIDS with histoplasmosis.2 , 3 Current diagnostic criteria require fulfillment of five out of eight criteria: fever, splenomegaly, cytopenia, hyperferritinemia, hypertriglyceridemia/hypofibrinogenemia, pathological evidence of hemophagocytosis, low natural killer cell activity, and elevated levels of soluble CD25.1 , 2 Treatment of HLH involves treating the underlying cause; additional therapy with corticosteroids, intravenous immunoglobulin (IVIG), etoposide, or cyclosporine may be required.1
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The authors declare that they do not have a conflict of interest.