A CASE OF MULTICENTRIC CASTLEMANS-DISEASE ASSOCIATED WITH RENAL AMYLOIDOSIS AND PURE RED-CELL APLASIA

This is the first reported case of multicentric Castleman's disease (M CD) associated with renal amyloidosis and pure red cell aplasia (PRCA) . Inguinal lymph node biopsy showed follicular hyperplasia with promin ent germinal centers and plasma cell proliferation in the interfollicu lar areas. Renal biopsy specimens revealed intraglomerular amyloid dep osits, defined as AA amyloidosis. Since amyloid deposits were not pres ent in the gastric and rectal mucosal tissues, his renal disorder was found to be an unusual secondary amyloidosis associated with MCD. Foll owing treatment by plasma exchange, there was progressive deterioratio n of anemia due to PRCA detected by bone marrow aspiration. Subsequent ly he was successfully treated with steroid pulse therapy not only for anemia but also for renal function.