LOCALISED PLASMA-CELL TYPE CASTLEMAN’S DISEASE AND AA-AMYLOIDOSIS CURED BY RESECTION. A CASE REPORT AND REVIEW OF THE LITERATURE

Abstract

Castleman’s disease (CD) is a rare atypical lymphoproliferative disorder with frequent, yet less well known renal involvement. We describe the case of a 58-year-old woman with localised abdominal plasma-cell type CD complicated by nephrotic syndrome due to renal amyloidosis. ¹⁸Fluorodeoxyglucose (FDG) positron emission tomography (PET) confirmed the unicentric nature. Resection of the mass resulted in regression of the signs of inflammation, a negative FDG-PET and complete remission of the nephrotic syndrome. A review of the literature of renal involvement (incidence, clinical and pathological manifestations, treatment and prognosis) in CD is provided.either purely reactive or truly neoplastic. (1). It is characterized by hyperplasia of lymphoid follicles with or without capillary proliferation and endothelial hyperplasia (2). Keller et al distinguished two histological subtypes: a more common hyaline vascular type (80-90%) and a rarer plasma cell type (10-20%) (3). Later, a third transitional or mixed type has been described, sharing aspects of both types. CD has two distinct clinical presentations: a localised form, mostly an asymptomatic mass and a multicentric form characterised by multiple enlarged lymph nodes frequently accompanied with B-symptoms (4).