The Enigma of Idiopathic Multicentric Castleman Disease: An Elusive Diagnosis.

Autor: Ohemeng-Dapaah J; Internal Medicine, Sisters of Saint Mary (SSM) Health St. Mary's Hospital, St. Louis, USA., Onyechi A; Internal Medicine, Sisters of Saint Mary (SSM) Health St. Mary's Hospital, St. Louis, USA., Kang A; Internal Medicine, Sisters of Saint Mary (SSM) Health St. Mary's Hospital, St. Louis, USA., Lacasse A; Internal Medicine, Sisters of Saint Mary (SSM) Health St. Mary's Hospital, St. Louis, USA., Sinha J; Hematology and Medical Oncology, Sisters of Saint Mary (SSM) Health St. Mary's Hospital, St. Louis, USA.
Jazyk: angličtina
Zdroj: Cureus [Cureus] 2024 Nov 06; Vol. 16 (11), pp. e73156. Date of Electronic Publication: 2024 Nov 06 (Print Publication: 2024).
DOI: 10.7759/cureus.73156
Abstrakt: 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.
Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.
(Copyright © 2024, Ohemeng-Dapaah et al.)
Databáze: MEDLINE