Abdominal Castleman disease with mixed histopathology in a patient with iron deficiency anemia, growth retardation and peliosis hepatis

Saritas U., Ustundag Y., Isitan G., Bastugrul S., Erekul S.

AMERICAN JOURNAL OF THE MEDICAL SCIENCES, vol.331, no.1, pp.51-54, 2006 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 331 Issue: 1
  • Publication Date: 2006
  • Doi Number: 10.1097/00000441-200601000-00015
  • Journal Name: AMERICAN JOURNAL OF THE MEDICAL SCIENCES
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.51-54
  • Keywords: Castleman disease, peliosis hepatis, iron deficiency anemia, growth retardation, LYMPH-NODE HYPERPLASIA, NODULAR REGENERATIVE HYPERPLASIA, PERISINUSOIDAL FIBROSIS, KAPOSIS-SARCOMA
  • Ankara University Affiliated: No

Abstract

In Castleman disease, also known as giant lymph node hyperplasia, lesions are usually located in the chest. Peliosis hepatis is the presence of multiple blood-filled cysts in the liver. The combination of these two disorders in one patient is rare, with only three cases reported to date. We report the case of a 21-year-old man who presented with growth retardation and iron deficiency anemia. Work-up revealed hepatomegaly and multiple conglomerated giant lymph nodes near the splenic hilum. Once the mass was surgically removed, the patients anemia rapidly resolved and he showed catch-up growth. The lymphoid tissue exhibited mixed histologic type, so the atient was diagnosed with both hyaline vascular type and plasma cell type Castleman disease. A liver biopsy obtained at surgery showed sinusoidal dilatation and blood-filled cysts, indicating peliosis hepatis. This is the fourth documented case of peliosis hepatis associated with Castleman disease. In addition to describing the case features, we discuss the possible pathogenesis of peliosis hepatis in the setting of Castleman disease.