International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease

Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G, Simpson D, Liu AY, Menke D, Chandrakasan S, Lechowicz MJ, Wong RS, Pierson S, Paessler M, Rossi JF, Ide M, Ruth J, Croglio M, Suarez A, Krymskaya V, Chadburn A, Colleoni G, Nasta S, Jayanthan R, Nabel CS, Casper C, Dispenzieri A, Fosså A, Kelleher D, Kurzrock R, Voorhees P, Dogan A, Yoshizaki K, van Rhee F, Oksenhendler E, Jaffe E, Elenitoba-Johnson KS, Lim MS. Blood. 2017 Jan 13. pii: blood-2016-10-746933. doi: 10.1182/blood-2016-10-746933. [Epub ahead of print]


More than six decades after Castleman disease (CD) was first described, a group of experts from Penn Medicine and other institutions around the world has established the first set of diagnostic criteria for a life-threatening subtype of the condition, idiopathic multicentric CD (iMCD), which is often misdiagnosed as other illnesses. Diagnosis of iMCD has been challenging, with no standard diagnostic criteria to guide physicians and significant overlap with cancer and autoimmune, or infectious disorders.
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