A Case Report on Idiopathic-Multi-centric Castleman’s Disease Associated POEMS- Syndrome

Abstract

: Castleman’s Disease is a rare lymphoproliferative disorder of idiopathic etio-pathogenesis and was first described by Dr Benjamin Castleman in 1954. It is a rather rare disease entity in clinical practice. However, the incidence cannot be ignored. It may affect a single lymph node (Unicentric CD) or multiple lymph nodes throughout the body (Multicentric CD). Management depends upon the type and severity of the disease and early recognition of the condition. Our case report is that of a 60-year-old female patient with underlying comorbidities including type 2 diabetes mellitus, hypothyroidism who presented with complaints of generalized weakness, weight loss, hair loss, non-resolving muco-purulent rhinorrhea, bilateral pedal edema and multiple, non-tender, firm and mobile left sided Level I and II ‘AJCC’ cervical lymphadenopathy of >2cm which was later biopsied and diagnosed to be Castleman’s disease associated POEMS syndrome. The aim of this report is to consider CD as a differential diagnosis in patients presenting with similar clinical picture and early identification and treatment may provide a better prognosis. We would also like to highlight 3 take home points: 1) The significance of histology in differentiating it from other lymph proliferative conditions. 2) LN excision of the bothersome LN can be diagnostic and may be considered therapeutic to some extend even in MCD. 3) Steroid therapy has beneficial effects to control progression of the disease.