Multicentric Castleman Disease: A Rare Case of Generalised Lymphadenopathy in India

Castleman disease (CD) is a type of hematological disorder, classified based on the number of the regions of enlarged lymph node, histopathological features, and association with human herpes virus 8. It may be unicentric with single region of the lymph node enlarged, or multicentric with multiple regions of lymphadenopathy. Some cases of Multicentric Castleman Disease (MCD) are caused by human herpes virus 8 (75%) while in others it is HHV8 negative. The epidemiology of idiopathic MCD is poorly understood due to lack of diagnosis since it is a difficult clinical diagnosis and also require analysis from a pathologist that is not available worldwide. Diagnosis and treatment are incredibly challenging as it can presents with wide array of manifestation from asymptomatic to systemic manifestation. There is no exact treatment guideline, Anti IL6 monoclonal antibody with or without systemic steroid is the mainstay of treatment. Here, we report a case that was initially suspected to have a lymphoma but later histologically was confirmed to have Multicentric Castleman's Disease. Here we report a case of forty-year-old man with history of chronic fever & constitutional symptoms, anasarca, exertional dyspnea, easy fatiguability, palpitation and generalized lymphadenopathy on physical examination which later diagnosed as Multicentric Castleman Disease. He had bicytopenia, raised inflammatory markers, lymph node biopsy revealed Castleman disease like picture. All the possible differential diagnosis had been ruled out with respective investigations. He was treated with steroid and IL6 inhibitor Tocilizumab and the patient improved remarkably.