Multicentric Castleman' s Disease Manifesting in the Lung: Clinical, Radiographic, and Pathologic Findings and Successful Treatment with Corticosteroid and Cyclophosphamide

Kazuhiro IYONAGA, Kazuya ICHIKADO, Hiroyuki MURANAKA, Kazuhiko FUJII, Tetsuro YAMAGUCHI, Moritaka SUGA
2003 Internal medicine (Tokyo. 1992)  
Multicentric Castleman's disease (MCD)is an uncommonand often incurable lymphoproliferative disorder. There has been some recent evidence that rare cases of MCDmanifest diffuse lung involvement, but the features in these cases are not well characterized. Wereport just such a biopsy-proven case of MCDwith typical laboratory abnormalities including serum interleukin-6 elevation and characteristic high-resolution CT findings. Immunopathologically, the features of the lung tissue resembled those of
more » ... resembled those of lymphocytic interstitial pneumonia with predominant infiltration of B cells and plasma cells. In addition, the abnormal appearance of B cells in bronchoalveolar lavage fluid was of diagnostic value. Although MCD is often refractory to treatment including corticosteroid, chemo-and immuno-therapy,we show successful treatment with corticosteroid and cyclophosphamide and 4 years of complete remission.
doi:10.2169/internalmedicine.42.182 fatcat:qffuqvoxrjeb5orhgya3aa3s5q