Postpartum hemophagocytic lymphohistiocytosis: A case report

Table 1 Diagnostic criteria of hemophagocytic lymphohistiocytosis: Hemophagocytic lymphohistiocytosis-2004

Criteria	Diagnosis will be established if one of either 1 or 2 below is fulfilled
A	A molecular diagnosis consistent with HLH: Pathogenic mutations of PRF-1, UNC13D, STXBP2, Rab27a, STX11, SH2D1A, or XIAP
B	Diagnostic criteria for HLH fulfilled (5 of 8 criteria below)
	1	Fever ≥ 38.5 C for ≥ 7 d
	2	Splenomegaly ≥ 3 finger breadth below left subcostal margin
	3	Cytopenia affecting ≥ 2 of 3 lineages in peripheral blood: Hemoglobin < 9 g/L; platelets < 100 × 109/L; absolute neutrophil count < 1.0 × 109/L
	4	Hypertriglyceridemia (> 265 mg/dL) and/or hypofibrinogenemia (< 150 mg/dL)
	5	Hemophagocytosis in bone marrow or spleen or lymph nodes or liver
	6	Low or absent natural killer cell activity
	7	Ferritin > 500 ng/mL
	8	Elevated soluble CD25 (sIL-2 receptor) ≥ 2400 U/mL

CD25: Cluster of differentiation 25; HLH: Hemophagocytic lymphohistiocytosis; PRF-1: Perforin 1; SH2D1A: SH2 domain-containing 1A; sIL-2: Soluble interleukin 2; STXBP2: Syntaxin binding protein 2; STX11: Syntaxin 11; UNC13D: Unc-13 homolog D; XIAP: X-linked inhibitor of apoptosis protein.