Unusual presentation of systemic lupus erythematosus as hemophagocytic lymphohistiocytosis in a female patient: A case report

Table 1 Baseline characteristics and hemophagocytic lymphohistiocytosis diagnosis of patient

Variables	Patient
Baseline characteristics
Age (yr)	36
Etiology/Trigger	SLE and multiple infections
HLH- directed therapies	VP16 and methylprednisolone
HLH- 2004 criteria at diagnosis (ref.: Henter-2007)
Fever	Y
Splenomegaly	N
Cytopenia, affecting 2 of 3 lineages in the peripheral blood
Hemoglobin concentration < 9 g/dL	Y
Neutrophil count < 1.0 × 109/L	Y
Platelet count < 100 × 109/L	Y
Hypertriglyceridemia (fasting ≥ 3.0 mmol/L) and/or hypofibrinogenemia (≤ 150 mg/dL)	Y
Hemophagocytosis in bone marrow or spleen or lymph nodes (no evidence of malignancy)	N
Low or absent natural killer cell activity	N/A
Ferritin ≥ 500 ng/mL	Y
Soluble cluster of differentiation 25 (i.e. soluble interleukin 2 receptor) ≥ 2400 U/mL	N

HLH: Hemophagocytic lymphohistiocytosis; N/A: Not applicable; Y: Yes; N: No; ref.: References.