Multicentric Castleman's disease manifesting in the lung: clinical, radiographic, and pathologic findings and successful treatment with corticosteroid and cyclophosphamide.
Multicentric Castleman's disease (MCD) is an uncommon and often incurable lymphoproliferative disorder. There has been some recent evidence that rare cases of MCD manifest diffuse lung involvement, but the features in these cases are not well characterized. We report just such a biopsy-proven case of MCD with typical laboratory abnormalities including serum interleukin-6 elevation and characteristic high-resolution CT findings. Immunopathologically, the features of the lung tissue 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.
Iyonaga
Kazuhiro
K
First Department of Internal Medicine, Kumamoto University School of Medicine, Honjo, Kumamoto.
Ichikado
Kazuya
K
Muranaka
Hiroyuki
H
Fujii
Kazuhiko
K
Yamaguchi
Tetsuro
T
Suga
Moritaka
M
D001707:Biopsy, Needle; D001992:Bronchoalveolar Lavage Fluid / Q000166:cytology; D005871:Castleman Disease / Q000188:drug therapy* / Q000473:pathology*; D003520:Cyclophosphamide / Q000008:administration & dosage*; D004305:Dose-Response Relationship, Drug; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D017563:Lung Diseases, Interstitial / Q000188:drug therapy* / Q000473:pathology*; D008875:Middle Aged; D011239:Prednisolone / Q000008:administration & dosage*; D035583:Rare Diseases; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D003520:Cyclophosphamide; D011239:Prednisolone