Multicentric Castleman's disease manifesting in the lung: Clinical, radiographic, and pathologic findings and successful treatment with corticosteroid and cyclophosphamide
Multicentric Castleman's disease manifesting in the lung: Clinical, radiographic, and pathologic findings and successful treatment with corticosteroid and cyclophosphamide
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DOI：
10.2169/internalmedicine.42.182
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发表时间：
2003-02-01
影响因子：
1.2
通讯作者：
Suga, M
中科院分区：
文献类型：
--
作者：
Iyonaga, K;Ichikado, K;Suga, M
多中心Castleman病（MCD）是一种罕见的，往往无法治愈的淋巴组织增生性疾病。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不清楚。我们报告这样一个活检证实的MCD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性间质性肺炎，主要是B细胞和浆细胞浸润。此外，支气管肺泡灌洗液中B细胞的异常出现具有诊断价值。虽然MCD往往是难治性的治疗，包括皮质类固醇，化疗和免疫治疗，我们显示成功的治疗与皮质类固醇和环磷酰胺和4年的完全缓解。
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.