The Enigma of Idiopathic Multicentric Castleman Disease: An Elusive Diagnosis

Castleman disease (CD) is a rare lymphoproliferative disorder encompassing a spectrum of conditions with distinct histopathological findings and varied clinical presentations. Diagnostic challenges are often encountered due to overlapping features with other malignancies, infections, and autoimmune disorders. Idiopathic multicentric Castleman disease (iMCD) is a subtype of CD, characterized by generalized lymphadenopathy, polyclonal lymphoproliferation, systemic inflammation, and a cytokine storm that can be life-threatening. Here, we present a case of iMCD in a 70-year-old male with constitutional symptoms, dyspnea, and pancytopenia. Imaging demonstrated multifocal lymphadenopathy. Histopathological examination of a cervical lymph node revealed Castleman-like features, meeting the major criteria for a diagnosis of iMCD. Elevated interleukin-6 (IL-6) levels further supported the diagnosis. Treatment with siltuximab was planned but was preempted by the patient's demise following acute heart failure exacerbation. Diagnosing CD necessitates a thorough evaluation to differentiate it from other diseases. Treatment strategies, particularly IL-6 blockade, play a crucial role in the management of iMCD and improve patient outcomes.