Heterogeneous Presentations of iMCD: A Single-Institution Case Series

Idiopathic multicentric Castleman disease (iMCD) is a rare lymphadenopathic disorder characterized by hyperplasia of multiple lymph nodes and can be associated with a wide range of symptoms and presentations, from mild disease to life-threatening organ failure. Varied histopathological features and heterogeneous presentation of this rare entity can make the diagnosis quite challenging for both hematologists and other specialists who may encounter patients at various stages of disease progression.

We analyze five different clinical presentations at our institution to demonstrate challenging routes of diagnosis and treatment complexities of iMCD. We aim to raise awareness to the importance of early diagnosis and appropriate management of this rare condition.

All patients in this series presented with symptomatic lymphadenopathy. We highlight one rare instance of thrombocytopenia, anasarca/ascites, fever, reticulin fibrosis or renal dysfunction, and organomegaly (TAFRO) syndrome with elusive iMCD, which illustrates the challenges in the diagnosis of this rare condition and the importance of early recognition of its symptoms to avoid decompensation of patients. We also review established treatment guidelines and response criteria to siltuximab as outlined in the international consensus treatment guidelines.

These cases highlight the heterogeneity and challenging diagnosis of this rare cytokine-driven hematological disorder and the role of siltuximab in the treatment of iMCD.