Table 2.
Proposed working case definition of KSHV inflammatory cytokine syndrome.
| 1. CLINICAL MANIFESTATIONS* | |
|---|---|
| a. Symptoms | b. Laboratory abnormalities |
| Fever | Anemia |
| Fatigue | Thrombocytopenia |
| Edema | Hypoalbuminemia |
| Cachexia | Hyponatremia |
| Respiratory symptoms (including cough, dyspnea, airway hyperreactivity) | c. Radiographic abnormalities |
| Gastrointestinal disturbance (including nausea, anorexia, abdominal discomfort, altered bowel habit) | Lymphadenopathy |
| Athralgia and myalgia | Splenomegaly |
| Altered mental state | Hepatomegaly |
| Neuropathy with or without pain | Body cavity effusions |
| 2. EVIDENCE OF SYSTEMIC INFLAMMATION | |
| Elevated C-reactive protein | |
| 3. EVIDENCE OF KSHV LYTIC ACTIVITY | |
| Elevated KSHV viral load in peripheral blood mononuclear cells (≥100 copies/106 cells) | |
| 4. NO EVIDENCE OF KSHV-ASSOCIATED MULTICENTRIC cASTLEMAN DISEASE | |
| Exclusion of MCD requires pathologic assessment lymph node, bone marrow, or spleen | |
The working case definition of KICS requires the presence of at least two clinical manifestations drawn from at least two categories (1a, b, and c), together with each of the criteria in 2, 3, and 4. Clinical manifestations for the working definition are drawn from the initial case series and from findings commonly seen in KSHV-MCD.