Association of Castleman’s Disease with Poems’ Syndrome in Young Adult

Abstract

SUMMARY Authors report the case of a 37 year old female patient. She presents numbness in her arms and legs, amenorrhea, edema on inferior members, adenomegaly and hyper-pigmented lesions on inferior members. Image exams reveal cardiomegaly and pericardial spill and also hepatomegaly, ascitis and solid images in the kidneys’ parenchyma. The laboratory found an increase of gamma fraction with kappa elevation, DHL and VSH elevation, an increase of TSH and chronic inflammatory disease anemia and a negative serology for HIV. The auxiliary lymph node biopsy evidenced intense angiofolicular hyperplasia with the presence of numerous plasmatic cells and a normal presence of germinative centers, all compatible with Castleman’s disease. In addition to that the patient displays an association with POEMS (Motor-sensitive polyneuropathy with axonal degeneration; hepatomegaly + cardiomegaly + kidney increase; 1 year amenorrhea + galactorhea; monoclonal kappa peak and skin hyperchromia). Castleman’s disease (CD), also called angiofolicular lymphoid hyperplasia or Giant Lymph Node Hyperplasia, among other designations. It is a rare lynphoproliferative disease of unknown cause and pathogenesis but with characteristic histological alterations in this multicentric form with POEMS syndrome association, a very rare entity in women below 40 years of age. DESCRIPTORS Castleman’s Disease. POEMS’ Syndrome Monoclonal Gammopathy