Hemophagocytic Lymphohistiocytosis Complicating T-Cell Lymphoma in a Patient with HIV Infection

Table 1

Diagnostic criteria for hemophagocytic lymphohistiocytosis.

(A) Molecular diagnosis consistent with HLH

(B) Five of the eight criteria listed below

Pathologic mutations of PRF1, UNC13D, Munc18-2, Rab-27A, STX11, SH2D1A, or BIRC4

(1) Fever > 38.5°C; (2) Splenomegaly; (3) Cytopenias (affecting at least 2 of 3 lineages in the peripheral blood); (a) Hemoglobin < 9 g/dL; (b) Platelets < 100 × 10^3/mL; (c) Neutrophils < 1 × 10^3/mL; (4) Hypertriglyceridemia (fasting > 265 mg/dL) and/or hypofibrinogenemia (< 150 mg/dL); (5) Hemophagocytosis in bone marrow, spleen, lymph nodes, or liver; (6) Low or absent NK cell activity; (7) Ferritin > 500 ng/mL; (8) Elevated sCD25