Iyonaga Kazuhiro, Ichikado Kazuya, Muranaka Hiroyuki, Fujii Kazuhiko, Yamaguchi Tetsuro, Suga Moritaka
First Department of Internal Medicine, Kumamoto University School of Medicine, Honjo, Kumamoto.
Intern Med. 2003 Feb;42(2):182-6. doi: 10.2169/internalmedicine.42.182.
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.
多中心Castleman病（MCD）是一种罕见且通常无法治愈的淋巴增生性疾病。最近有一些证据表明，罕见的MCD病例表现为弥漫性肺受累，但这些病例的特征尚未得到充分描述。我们报告了一例经活检证实的MCD病例，该病例具有典型的实验室异常，包括血清白细胞介素-6升高和特征性的高分辨率CT表现。免疫病理学上，肺组织的特征类似于淋巴细胞间质性肺炎，以B细胞和浆细胞为主浸润。此外，支气管肺泡灌洗液中B细胞的异常表现具有诊断价值。尽管MCD通常对包括皮质类固醇、化疗和免疫治疗在内的治疗方法难治，但我们展示了皮质类固醇和环磷酰胺治疗成功且完全缓解达4年。
