Longitudinal, natural history study reveals the disease burden of idiopathic multicentric Castleman disease

Abstract

Idiopathic multicentric Castleman disease (iMCD) is a rare hematologic disorder with heterogeneous presentations ranging from moderate constitutional symptoms to life-threatening multiorgan system involvement. iMCD patients present with vastly different clinical subtypes, with some patients demonstrating thrombocytopenia, anasarca, fever/elevated C-reactive protein, reticulin fibrosis/renal failure, and organomegaly (TAFRO) and others demonstrating more mild/moderate symptoms with potential for severe disease (not otherwise specified, NOS). Due to its rarity and heterogeneity, the natural history and long-term burden of iMCD are poorly understood. We investigated real-world medical data from ACCELERATE, a large natural history registry of Castleman disease patients, to better characterize the long-term disease burden experienced by these patients. We found that iMCD-TAFRO patients face significant hospitalization burden, requiring more time in the hospital than iMCD-NOS patients during the year surrounding diagnosis (median [IQR] 36 [18, 61] days vs. 0 [0, 4] days; p

TAFRO Syndrome: Guidance for Managing Patients Presenting Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis, Renal Insufficiency, and Organomegaly

“…TAFRO syndrome has an acute or subacute onset, and the patient’s general condition usually deteriorates quickly, whereas most iMCD cases have a relatively chronic course. Thus, life-threatening multi-organ involvement and hospitalization burdens are more common in TAFRO syndrome [ 24 ]. Another aspect that differentiates these two conditions is that polyclonal hypergammaglobulinemia, as definitively seen in iMCD without TAFRO, is rarely observed in TAFRO syndrome.…”