Undiagnosed Chronic Granulomatous Disease, Burkholderia cepacia complex Pneumonia, and Acquired Hemophagocytic Lymphohistiocytosis: A Deadly Association

Table 2

Clinical, laboratory, and autopsy findings corresponding to acquired hemophagocytic lymphohistiocytosis diagnostic criteria.

Hemophagocytic lymphohistiocytosis diagnostic criteria; (diagnosis is established by fulfilling 5 out of 8 of the following)	Patient’s findings
Fever	Yes
Splenomegaly	Yes
Cytopenia (affecting ≥ 2 cell lineages, hemoglobin < 9 g/dL; platelets < 100 G/L; neutrophils < 1.0 G/L)	Hemoglobin 6.4 g/dL; Platelets 80 G/L
Hypertriglyceridemia (≥265 mg/dL) and/or hypofibrinogemia (≤1.5 g/L)	yes
Low or absent natural killer cell cytotoxicity	NI
Hyperferritinemia (>500 ng/mL)	NI
Elevated sCD25 (>2.400 U/mL)	NI
Hemophagocytosis in the bone marrow, spleen, or lymph nodes without malignancy	Bone marrow and spleen

NI: not investigated.
Note: adapted from the 2nd International HLH study, http://www.histio.org/page.aspx?pid=389.