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The Enigma of Idiopathic Multicentric Castleman Disease: An Elusive Diagnosis
Jessica Ohemeng-Dapaah1, Afoma Onyechi1, Ayesha Kang1
1Internal Medicine, Sisters of Saint Mary (SSM) Health St. Mary's Hospital, St. Louis, USA.
Insights
Idiopathic multicentric Castleman disease (iMCD) is a rare disorder causing systemic inflammation and lymphadenopathy. Early diagnosis and IL-6 blockade are crucial for managing iMCD, though this case highlights diagnostic challenges and treatment complexities.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Castleman disease (CD) is a rare lymphoproliferative disorder with diverse presentations.
- Idiopathic multicentric Castleman disease (iMCD) involves generalized lymphadenopathy, systemic inflammation, and a dangerous cytokine storm.
- Diagnosis of CD can be challenging due to overlapping symptoms with other conditions.
Abstract:
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.
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