KSHV Inflammatory Cytokine Syndrome (KICS)

Working Case Definition for KICS: Requires at least 2 clinical manifestations (from at least 2 categories of 1a, b, c) together with each of criteria in 2, 3, and 4.

1) Clinical manifestations

   a. Symptoms: Fatigue, Edema, Cachexia, Respiratory symptoms, GI disturbance, Arthralgia/myalgia, Neuropathy +/- pain

   b. Lab abnormalities: Anemia, Thrombocytopenia, Hypoalbuminemia, Hyponatremia

   c. Radiographic abnormalities: Lymphadenopathy, Splenomegaly, Hepatomegaly, Body cavity effusions

2) Evidence of systemic inflammation - Elevated CRP (>3 g/dL)

3) Evidence of KSHV viral activity - Elevated KSHV viral load in plasma 1000 copies/mL) or peripheral mononuclear cells 100 copies/ cells)

4) No evidence of KSHV-multicentric Castleman disease - Exclusion of MCD requires histopathologic assessment of lymphadenopathy if present



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