Multicentric Castleman disease of hyaline vascular variant presenting with unusual systemic manifestations: a case report.

Autor: Basnayake BMDB; Department of Medicine, Teaching Hospital Kandy, Kandy, Sri Lanka. bmdbbasnayake@yahoo.com., Wazil AWM; Department of Nephrology, Teaching Hospital Kandy, Kandy, Sri Lanka., Kannangara T; Department of Medicine, Teaching Hospital Kandy, Kandy, Sri Lanka., Ratnatunga NVI; Department of Pathology, Faculty of Medicine, University of Peradeniya, Peradeniya, Sri Lanka., Hewamana S; Department of Hematology and Hemato-oncology, Lanka Hospitals, Colombo, Sri Lanka., Ameer AM; Department of Medicine, Teaching Hospital Kandy, Kandy, Sri Lanka.
Jazyk: angličtina
Zdroj: Journal of medical case reports [J Med Case Rep] 2017 May 14; Vol. 11 (1), pp. 135. Date of Electronic Publication: 2017 May 14.
DOI: 10.1186/s13256-017-1294-3
Abstrakt: Background: Castleman 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 Presentation: A 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.
Conclusions: Castleman 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.
Databáze: MEDLINE
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