A Case of Spontaneously Improving Secondary Hemophagocytic Lymphohistiocytosis in an Adult Associated with T-Cell Histiocyte-Rich Large B-Cell Lymphoma

Table 1

Diagnostic criteria for HLH.

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Fever ≥38.5°C

Splenomegaly

Peripheral blood cytopenia, with at least two of the following:

Hemoglobin <9 g/dL (hemoglobin <10 g/dL for infants <4 weeks)

Thrombocyte count <100,000/μL

Absolute neutrophil count (ANC) <1000/μL

Fasting triglycerides >265 mg/dL and/or fibrinogen <150 mg/dL

Hemophagocytosis in bone marrow, spleen, lymph node, or liver

Low or absent NK-cell activity

Ferritin >500 ng/mL

Elevated soluble CD25 (soluble IL-2 receptor alpha) two standard deviations above age-adjusted laboratory-specific norms (or >2400 U/ml, often cited in the literature)

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≥5/8 criteria must be met for the diagnosis. Adapted from Jordan et al. [5].