Hyaline-Vascular Type Castleman’s Disease, Sarcoidosis, and Crohns Disease

• Published in: Indian journal of hematology & blood transfusion 2016-06, Vol.32 (Suppl 1), p.335-339
• Main Authors: Gupta, Arjun, Ayyar, Balaji, Zia, Hamid, Chen, Weina, Harris, Samar, Naina, Harris V.
• Format: Article
• Language: English
• Subjects: Blood Transfusion Medicine; Case Report; Crohn's disease; Hematology; Human Genetics; Medicine; Medicine & Public Health; Oncology; Pathology; Surgery
• ISSN: 0971-4502; 0974-0449; 0974-0449; 0971-4502
• DOI: 10.1007/s12288-015-0580-8

Sarcoidosis and Crohns disease have been associated with increased long term risk of lymphoproliferative disorders, including lymphomas. Newly developed lymphadenopathy in a patient with these disorders should prompt pathological evaluation. Castleman’s disease is a lymphoproliferative disorder characterized by enlarged hyperplastic lymph nodes with regressed follicles surrounded by expanded mantle zones of small lymphocytes, and interfollicular vascular proliferation in the hyaline-vascular type. Similar to sarcoidosis and Crohns disease, its etiology is incompletely understood, although immune dysregulation, genetic factors and infectious and environmental factors are thought to play a role in all three diseases. Interleukin-6 is a possible pathological common factor between these three disease processed. Unicentric, hyaline-vascular type Castleman’s disease can be treated successfully with complete surgical resection. We report a patient with long history of sarcoidosis and Crohns disease with newly developed lymphadenopathy which was found to be due to Castleman’s disease.