Alzamora Meritxell Garcia, Schmidli Markus, Hess Urs, Cathomas Richard, von Moos Roger
Sektion Neuroradiologie, Neurozentrum, Universitätsklinikum Freiburg i.Br., Deutschland.
Onkologie. 2006 Apr;29(4):153-6. doi: 10.1159/000091644. Epub 2006 Mar 22.
So far, only 9 cases of minimal change glomerulonephritis (MCGN) related to chronic lymphocytic leukemia (CLL) have been described.
Our patient presented with severe nephrotic syndrome. Diagnostic biopsies confirmed MCGN and early-stage BCLL (Binet A). In contrast to previously described cases, kappa monoclonal IgM and cryoglobulins were also detected. The patient was treated with chlorambucil and prednisone. 3 weeks later, renal function and white blood cell (WBC) count were completely normal.
Although, in most cases, renal disease and CLL present separately, there is evidence for a causal relationship between the two disorders. Although it is widely approved that early-stage CLL should not be treated by medication, the severe nephrotic syndrome in our patient required immediate intervention. So far, there is no standard therapy for MCGN associated with CLL. Our combination therapy resulted in normalization of the nephrotic syndrome and the WBC count. Hence, we emphasize the importance of early recognition of renal symptoms in CLL and propose a successful therapy for nephrotic syndrome in MCGN associated with early-stage B-CLL. Additionally, the pathophysiological and therapeutic aspects of MCGN associated with CLL are retrospectively discussed.
迄今为止,仅报道过9例与慢性淋巴细胞白血病(CLL)相关的微小病变性肾小球肾炎(MCGN)。
我们的患者表现为严重肾病综合征。诊断性活检确诊为MCGN和早期B细胞慢性淋巴细胞白血病(Binet A期)。与先前报道的病例不同,还检测到κ单克隆IgM和冷球蛋白。患者接受了苯丁酸氮芥和泼尼松治疗。3周后,肾功能和白细胞(WBC)计数完全恢复正常。
尽管在大多数情况下,肾脏疾病和CLL是分别出现的,但有证据表明这两种疾病之间存在因果关系。虽然普遍认为早期CLL不应接受药物治疗,但我们患者的严重肾病综合征需要立即干预。迄今为止,尚无针对与CLL相关的MCGN的标准治疗方法。我们的联合治疗使肾病综合征和WBC计数恢复正常。因此,我们强调早期识别CLL肾脏症状的重要性,并提出了一种针对与早期B细胞慢性淋巴细胞白血病相关的MCGN中肾病综合征的成功治疗方法。此外,还对与CLL相关的MCGN的病理生理和治疗方面进行了回顾性讨论。