Case Report

Two Cases of Hemophagocytic Lymphohistiocytosis Associated with Disseminated Histoplasmosis Presented with Transient Pancytopenia

Table 1

HLH-2004 diagnostic criteria.

The diagnosis of HLH can be established if criterion 1 or 2 is fulfilled

(1) A molecular diagnosis consistent with HLH
(2) Diagnostic criteria for HLH fulfilled (5 of the 8 below)
 Fever
 Splenomegaly
 Cytopenias (affecting ≥ 2 of 3 lineages in the peripheral blood)
    Hemoglobin < 90 g/L (hemoglobin < 100 g/L in infats < 4 weeks)
    Platelets < 100 × 109/L
    Neutrophils < 1.0 × 109/L
 Hypertriglyceridemia and/or hypofibrinogenemia
    Fasting triglycerides ≥ 3.0 mmol/L (ie, ≥265 mg/dL)
    Fibrinogen ≤ 1.5 g/L
 Hemophagocytosis in bone marrow or spleen or lymph nodes. No evidence of malignancy
 Low or no NK cell activity (according to local laboratory reference)
 Ferritin ≥ 500 μg/L
 sCD25 (ie, soluble IL-2 receptors) ≥2400 U/mL
La Rosée et al. [10]