Natural History Study of the KSHV Inflammatory Cytokine Syndrome (KICS)

Inclusion Criteria

• •Age greater than or equal to18 Years.
• •Any HIV status.
• •At least two manifestations drawn from at least two of the categories (clinical symptoms, laboratory abnormalities and/or radiographic abnormalities), which are at least possibly attributable to KICS and are not readily explicable from known medical conditions in the participant:
• •Clinical symptoms (each at least grade 1 by CTCAE definitions)
• •Fever (\>38 degrees C), chills or rigors
• •Fatigue or lethargy
• •Cachexia or edema
• •Cough, dyspnea, airway hyperreactivity, or nasal inflammation
• •Nausea, anorexia, abdominal pain or altered bowel habit
• •Athralgia or myalgia
• •Altered mental state
• •Neuropathy with or without pain
• •Laboratory abnormalities
• •Anemia (hemoglobin\<12.0g/dL)
• •Thrombocytopenia (platelets\<100,000 cells/microL)
• •Leukopenia (white cell count\<4,000 cells/microL)
• •Hypoalbuminemia (albumin\<3.5g/dL)
• •Hyponatremia (sodium\<135mmol/L)
• •Coagulopathy (PT or PTT \>1.5 times upper limit of normal)
• •Radiographic Abnormalities
• •Pathologic lymphadenopathy (at least five discrete nodes each \>1cm in their longest dimension)
• •Splenomegaly (\>12 cm in the longest dimension)
• •Hepatomegaly (\>17cm in the longest dimension)
• •Body cavity effusions not caused by primary effusion lymphoma nor chylous effusions directly related to lymphatic infiltration by KS
• •C-reactive protein (CRP) \>3mg/L.
• •Exposure risk for KSHV infection (including being a first or second generation immigrant from an endemic area, or male-to-male sexual activity) or evidence of KSHV infection demonstrated by one of:
• •* Molecular evidence of KSHV in whole blood, or KSHV VL levels within circulating PBMCs as determined by the Whitby laboratory
• •* Immunohistochemical evidence of KSHV in tissues (for example by staining for LANA or vIL-6) confirmed in the Laboratory of Pathology (LP), CCR, NCI.
• •* Presence of KS or PEL (KSHV-associated malignancies), confirmed in the LP, CCR, NCI.
• •Women of child-bearing potential (WOCBP) and men must agree to use adequate contraception (hormonal or barrier method of birth control; abstinence) prior to study entry, for the duration of study participation, and after treatment (if received), according to drug requirements. Should a woman become pregnant or suspect she is pregnant while she or her partner is participating in this study, she should inform her treating physician immediately.