Working case definition of KSHV inflammatory cytokine syndrome
| Criterion 1: At least two manifestations drawn from at least two of the categories listed below (clinical symptoms, laboratory abnormalities and radiographic abnormalities) . | ||
|---|---|---|
| Symptoms . | Laboratory abnormalities . | Radiographic abnormalities . |
| Fever (>38°C), chills or rigors | Anemia (hemoglobin <12.0 g/dL) | Pathologic lymphadenopathy (at least five discrete nodes each >1cm in their longest dimension) |
| Fatigue or lethargy | Thrombocytopenia (platelets <100,000 cells per μL) | Splenomegaly (>12 cm in the longest dimension) |
| Cachexia or edema | Leukopenia (white cell count <4,000 cells per μL) | Hepatomegaly (>17 cm in the longest dimension) |
| Cough, dyspnea, airway hyperreactivity, or nasal inflammation | Hypoalbuminemia (albumin <3.5 g/dL) | Body cavity effusions (not caused by primary effusion lymphoma nor chylous effusions directly related to lymphatic infiltration by KS) |
| Nausea, anorexia, abdominal pain or altered bowel habit | Hyponatremia (sodium <135 mmol/L) | |
| Athralgia or myalgia | Coagulopathy (PT or PTT >1.5x upper limit of normal) | |
| Altered mental state | ||
| Neuropathy with or without pain | ||
| Criterion 2: Elevated CRP (>3 mg/L) | ||
| Criterion 3: Elevated KSHV VL in whole blood (≥1000 copies/mL) or in PBMCs (≥100 copies/106 cells in blood or body cavity effusion) | ||
| Criterion 4: No evidence of KSHV-associated MCD or primary effusion lymphoma: Exclusion of MCD requires pathologic assessment of lymph node, bone marrow and/or splenic tissue. Exclusion of PEL requires evaluation of effusions and/or lymph nodes or extracavitary masses | ||
| Criterion 1: At least two manifestations drawn from at least two of the categories listed below (clinical symptoms, laboratory abnormalities and radiographic abnormalities) . | ||
|---|---|---|
| Symptoms . | Laboratory abnormalities . | Radiographic abnormalities . |
| Fever (>38°C), chills or rigors | Anemia (hemoglobin <12.0 g/dL) | Pathologic lymphadenopathy (at least five discrete nodes each >1cm in their longest dimension) |
| Fatigue or lethargy | Thrombocytopenia (platelets <100,000 cells per μL) | Splenomegaly (>12 cm in the longest dimension) |
| Cachexia or edema | Leukopenia (white cell count <4,000 cells per μL) | Hepatomegaly (>17 cm in the longest dimension) |
| Cough, dyspnea, airway hyperreactivity, or nasal inflammation | Hypoalbuminemia (albumin <3.5 g/dL) | Body cavity effusions (not caused by primary effusion lymphoma nor chylous effusions directly related to lymphatic infiltration by KS) |
| Nausea, anorexia, abdominal pain or altered bowel habit | Hyponatremia (sodium <135 mmol/L) | |
| Athralgia or myalgia | Coagulopathy (PT or PTT >1.5x upper limit of normal) | |
| Altered mental state | ||
| Neuropathy with or without pain | ||
| Criterion 2: Elevated CRP (>3 mg/L) | ||
| Criterion 3: Elevated KSHV VL in whole blood (≥1000 copies/mL) or in PBMCs (≥100 copies/106 cells in blood or body cavity effusion) | ||
| Criterion 4: No evidence of KSHV-associated MCD or primary effusion lymphoma: Exclusion of MCD requires pathologic assessment of lymph node, bone marrow and/or splenic tissue. Exclusion of PEL requires evaluation of effusions and/or lymph nodes or extracavitary masses | ||
Patients also cannot have other evident causes of inflammation.
PT, prothrombin time; PTT, partial thromboplastin time.