Department of Internal Medicine, Eulji University College of Medicine, Seoul, Korea.
J Korean Med Sci. 2011 Jun;26(6):824-8. doi: 10.3346/jkms.2011.26.6.824. Epub 2011 May 18.
Waldenstrom macroglobulinemia (WM) is a B-cell lymphoproliferative disorder associated with bone marrow involvement of lymphoplasmacytic lymphoma (LPL) and an IgM monoclonal gammopathy. Generally B-lymphocytes in LPL do not express CD5 that is important for differential diagnosis of B-cell lymphoproliferative disorders. In WM, various renal diseases and type I cryoglobulinemia are well described separately, but cryoglobulinemic glomerulonephropathy is very rarely reported. A 61-yr-old woman complained of generalized edema, cyanosis of the extremities in cold weather, visual disturbance, and pancytopenia. Bone marrow and renal biopsy showed CD5+ expressing B-cells and cryoglobulinemic glomerulonephropathy. With the diagnosis of WM, she received cyclophosphamide, doxorubicin, vincristine and prednisolone chemotherapy and got complete remission. Here, we report a rare case of WM associated with unusual expression of CD5+ B-lymphocytes and cryoglobulinemic glomerulonephropathy, and emphasize the importance of the clinical features in differentiating CD5+ B-cell lymphoproliferative disorders.
华氏巨球蛋白血症(WM)是一种与骨髓受累的淋巴浆细胞淋巴瘤(LPL)和 IgM 单克隆丙种球蛋白病相关的 B 细胞淋巴增殖性疾病。通常,LPL 中的 B 淋巴细胞不表达 CD5,CD5 对 B 细胞淋巴增殖性疾病的鉴别诊断很重要。在 WM 中,各种肾脏疾病和 I 型冷球蛋白血症分别有很好的描述,但冷球蛋白血症性肾小球肾炎很少见报道。一名 61 岁女性因全身水肿、寒冷天气时四肢发绀、视力障碍和全血细胞减少而就诊。骨髓和肾脏活检显示 CD5+表达 B 细胞和冷球蛋白血症性肾小球肾炎。根据 WM 的诊断,她接受了环磷酰胺、多柔比星、长春新碱和泼尼松化疗,并获得完全缓解。在这里,我们报告了一例罕见的 WM 合并不寻常表达 CD5+ B 淋巴细胞和冷球蛋白血症性肾小球肾炎的病例,并强调了临床特征在鉴别 CD5+ B 细胞淋巴增殖性疾病中的重要性。