Idiopathic multicentric Castleman disease presenting as generalized lymphadenopathy: A case report

Abstract

A 48-year-old male presented with easy fatigability, and on evaluation, he was found to have generalized lymphadenopathy and elevated erythrocyte sedimentation rate (ESR-70 mm/hr). Neck ultrasound revealed enlarged left paratracheal lymph nodes. He had positive Mantoux test, a diagnosis of tuberculous lymphadenopathy was made and started on antitubercular medication. However, the patient’s condition deteriorated over the next month with new onset of dyspnea on exertion, dysphagia, generalized pruritus, and progressive persistent lymphadenopathy. Laboratory results indicated elevated ESR (72 mm/hr), gamma-glutamyl transpeptidase (GGTP 370 U/L), and AST (95 U/L) levels, although alkaline phosphatase levels were normal. The hemogram and liver, renal, and thyroid function test results were normal. CRP, autoantibody tests, and Serum Protein Electrophoresis results were also negative. CT thorax revealed cervical, Axillary, Hilar and Mediastinal Lymphadenopathy. The left axillary lymph node biopsy was negative for tuberculosis (AFB and PCR) but exhibited histological characteristics indicative of Castleman disease. His viral serology was negative for VDRL, HIV, HBSAG, and HCV. Immunohistochemistry (IHC) on lymph node biopsy was negative for the lymphoma panel and HHV 8. The diagnosis of idiopathic multicentric Castleman disease (iMCD) was confirmed using clinical, laboratory, and histological evaluations after the elimination of viral and neoplastic etiologies. He was initially treated with intravenous steroids, followed by oral steroids and immunosuppressive drugs.