An Unusual Case of Adult-Onset Still’s Disease with Hemophagocytic Syndrome, Necrotic Leukoencephalopathy and Disseminated Intravascular Coagulation

Table 1

Diagnostic criteria of hemophagocytic syndrome.

(1) Fever
(2) Spleenomegaly
(3) Cytopenia in 2 of 3 cell lines
(4) Hypertriglyceridemia
(5) Hemophagocytosis in either bone marrow, lymph nodes or the spleen
(6) Low/absent NK cell activity
(7) Hyperferritinemia (>500 𝜇g/L)
(8) High levels of soluble IL-2

Courtesy from Henter et al. [4].