Ha Austin Y, Noronha Nicole, Gleason Patrick, Winkler Jonathan N, Butera James N, Henriksen Kammi J, Hu Susie L
Warren Alpert Medical School of Brown University.
Department of Medicine.
Clin Nephrol Case Stud. 2016 Jan 19;4:1-4. doi: 10.5414/CNCS108740. eCollection 2016.
Cryoglobulinemia and mucosa-associated lymphoid tissue (MALT) lymphoma are diseases characterized by B-cell dysregulation and overproduction of antibodies. Vasculitis and cutaneous manifestations are common, but renal involvement is rare. A 65-year-old woman with type 1 cryoglobulinemia and MALT lymphomas of the right lacrimal and parotid glands successfully treated by excision and chemoradiotherapy, presented with dyspnea on exertion, edema, and hematuria. Renal biopsy findings revealed type 1 cryoglobulinemic glomerulonephritis. She underwent treatment with high-dose oral prednisone and intravenous rituximab with subsequent return of creatinine to baseline levels. To our knowledge, this is the first report of a patient in whom type 1 cryoglobulinemia, multiple MALT lymphomas, and MPGN with IgM cryoglobulin deposits coexist. Evidence for rituximab is sparse with widely varying protocols and mixed results. There is a need for high quality evidence in the treatment of these conditions.
冷球蛋白血症和黏膜相关淋巴组织(MALT)淋巴瘤是以B细胞调节异常和抗体过度产生为特征的疾病。血管炎和皮肤表现很常见,但肾脏受累很少见。一名65岁患有1型冷球蛋白血症以及右泪腺和腮腺MALT淋巴瘤的女性,经手术切除和放化疗成功治疗后,出现劳力性呼吸困难、水肿和血尿。肾活检结果显示为1型冷球蛋白血症性肾小球肾炎。她接受了大剂量口服泼尼松和静脉注射利妥昔单抗治疗,随后肌酐恢复到基线水平。据我们所知,这是首例1型冷球蛋白血症、多发MALT淋巴瘤以及伴有IgM冷球蛋白沉积的膜增生性肾小球肾炎共存患者的报告。关于利妥昔单抗的证据稀少,治疗方案差异很大且结果不一。在这些疾病的治疗中需要高质量的证据。