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.

Table 2 Number of diagnostic criteria met, treatments, and outcomes

Case No	Diagnostic criteria met	Primary therapy	Outcome
1	1,2,3,4,5,7,8	HLH-04	Died
2	1,2,3,4,5,6,7	HLH-04	Survived
3	1,3,4,5,7,8	HLH-04	Survived
4	1,2,4,5,6,7,8	HLH-94	Survived
5	1,2,4,5,6,7,8	HLH-94	Survived
6	1,3,4,5,7	HLH-94	Died
7	1,2,4,6,7,8	FD	Survived
8	1,2,4,5,6,7,8	DEP	Survived
9	1,2,3,4,5,6,7,8	HLH-94 with anakinra	Survived
10	1,2,3,4,5,7	HLH-04	Survived
11	1,2,3,4,7	Methylprednisolone	Survived

Diagnostic criteria met, 1: Fever; 2: Splenomegaly; 3: Cytopenia affecting ≥ 2 of 3; 4: Hypertriglyceridemia and/or hypofibrinogenemia; 5: Hemophagocytosis in bone marrow or spleen or lymph nodes or liver; 6: Low or absent natural killer cell activity; 7: Ferritin elevation; 8: Elevated soluble cluster of differentiation 25. DEP: Doxorubicin + etoposide + methylprednisolone; FD: Fludarabine + prednisolone; HLH: Hemophagocytic lymphohistiocytosis.