Abstract
BackgroundCastleman disease is a rare lymphoproliferative disorder presenting with localized or disseminated lymphadenopathy and systemic manifestations. It can be categorized in numerous ways, such as unicentric versus multicentric, histopathological variants (hyaline-vascular, plasma cell, and mixed), or subtypes based on causative viral infections (human immunodeficiency virus, human herpesvirus-8, or Kaposi sarcoma herpesvirus). Presentation ranges from asymptomatic to symptoms involving multiple organs. Even though the exact mechanism of pathogenesis is unknown, treatment is directed toward possible etiologies such as interleukin-6, cluster of differentiation 20, and viral agents.Case presentationA 36-year-old Sri Lankan woman presented with generalized body swelling and foamy urine of 2 weeks’ duration. Examination revealed pallor; generalized edema; axillary, cervical, and inguinal lymphadenopathy; hypertension; and hepatomegaly. Investigations showed bicytopenia, nephrotic range proteinuria with hypoalbuminemia, hypogammaglobulinemia, and features of hyaline-vascular type Castleman disease in a lymph node biopsy. She was managed with rituximab and had good clinical improvement.ConclusionsCastleman disease has a broad spectrum of clinical manifestations, disease pathogeneses, and associations and/or complications. Medical professionals need to be familiar with this spectrum because timely diagnosis and aggressive targeted therapy are the cornerstones of managing these patients.
Highlights
Castleman disease is a rare lymphoproliferative disorder presenting with localized or disseminated lymphadenopathy and systemic manifestations
We report a case of a Sri Lankan patient with the multicentric hyalinevascular variant of Castleman disease (CD) who presented with unusual systemic manifestations, namely nephrotic range proteinuria, marked lymphadenopathy, hepatomegaly, and hematological abnormalities
CD first appeared in the medical literature in 1956 with 13 cases of localized mediastinal lymph node hyperplasia described by Dr Benjamin Castleman
Summary
Hyaline-vascular CD usually has a benign clinical course, our patient presented with multicentric distribution and multisystemic involvement.
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have