Budd-Chiari syndrome, a rare complication of multicentric Castleman disease: A case report.

Abstract

A 39-year-old female presented to The First Affiliated Hospital of Jishou University (Jishou, Hunan) with a fever of unknown origin and progressive abdominal distension. Physical examination revealed generalized lymphadenopathy, multiple non-tender cutaneous nodules, hepatomegaly, splenomegaly and abdominal edema. An axillary lymph node biopsy indicated hyaline vascular type Castleman disease, and color Doppler and computed tomography scans suggested Budd-Chiari syndrome (BCS). Based on the abdominal distension and impairments of the liver and kidneys, an inferior vena cavography and balloon dilatation were performed, confirming the diagnosis of BCS and leading to symptomatic improvement. The patient commenced a combination chemotherapy regimen of cyclophosphamide (0.4 g; days 1-3), vindesine (4 mg; day 1) and prednisolone (100 mg; days 1-5), with no melioration of symptoms. Theprubicin was added to suppress the aggravation of the disease on day six of the chemotherapy cycle. The patient exhibited symptomatic remission for one week, however, she subsequently succumbed to intracranial hemorrhage and infections of the lung and intestine due to long-term myelosuppression following chemotherapy. To the best of our knowledge, this is the first report of BCS in a patient with multicentric Castleman disease without human immunodeficiency virus infection.