Keywords
Castleman disease, TAFRO syndrome
Abstract
A 31-year-old Caucasian woman of South-American descent was diagnosed with a variant of multicentric Castleman disease (MCD) that has been reported in Japan as Castleman-Kojima disease. This is a systemic inflammatory disorder known as TAFRO Syndrome which includes thrombocytopenia, polyserositis (ascites/pleural effusion), microcytic anemia, myelofibrosis, fever, renal dysfunction and organomegaly, with immunologic disorder, polyclonal hypergammaglobulinemia, and elevated levels of interleukin-6 (IL-6) and the vascular endothelial growth factor present in serum and/or effusions. Optimal therapies are not well established. The patient was treated with methylprednisolone and rituximab. Following the start of treatment, the patient has been asymptomatic for over 8 months.
This is one of only a few reports of TAFRO Syndrome in a non-Japanese patient.
References
Views: 2359
HTML downloads: 430
PDF downloads: 664
Untitled downloads: 0
Published:
2015-07-14
Issue:
Vol. 2 No. 4 (2015)
(view)