Tuberculosis-associated hemophagocytic lymphohistiocytosis misdiagnosed as systemic lupus erythematosus: A case report

Table 2 Diagnostic criteria for hemophagocytic lymphohistiocytosis

A diagnosis of HLH can be made if either 1 or 2 is met	Our case
1. Molecular diagnosis consistent with HLH (e.g., pathologic mutations of PRF1, UNC13D or STX11)	N
2. Clinical and laboratory criteria (at least 5/8 should be fulfilled)	Y (5/8)
Fever ≥ 38.5°C	Y (39.7°C)
Splenomegaly	N
Cytopenia ≥ 2-3 cell lines in peripheral blood (hemoglobin < 90 g/L, platelets < 100 x 109/L, neutrophils < 1.0 x 109/L)	Y (hemoglobin 53 g/L, platelets 64 x 109/L)
Hypertriglyceridemia (fasting triglycerides > 265 mg/dL) and/or hypofibrinogenemia (fibrinogen < 150 mg/dL)	Y (fibrinogen 1.26 g/L)
Reduced or absent NK cell activity	N
Hemophagocytosis in bone marrow, spleen, CSF or lymph nodes	Y (hemophagocytosis in bone marrow)
Ferritin ≥ 500 mg/L	Y (ferritin 679.93 ng/mL)
Elevated soluble CD 25	N

HLH: Hemophagocytic lymphohistiocytosis.