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瓦尔登斯特伦巨球蛋白血症并发膜增生性肾小球肾炎。

Membranoproliferative glomerulonephritis complicating Waldenström's macroglobulinemia.

机构信息

Department of Nephrology, University Hospital Essen, University of Duisburg-Essen, Hufelandstr. 55, 45122 Essen, Germany

出版信息

BMC Nephrol. 2012 Dec 21;13:172. doi: 10.1186/1471-2369-13-172.

Abstract

BACKGROUND

Lymphoproliferative disorders causing paraproteinemia can be associated with various kidney injuries including the deposition of monoclonal immunoglobulins (Ig). A known glomerular manifestation of Waldenström's macroglobulinemia is characterized by prominent intracapillary hyaline thrombi and lack of conspicuous glomerular proliferation. The present case was special in 2 aspects: 1. the diagnosis of glomerulonephritis was unexpected before renal biopsy, 2. the prominent glomerular proliferation paired with large intracapillary hyaline thrombi is uncommon in Waldenström's macroglobulinemia-associated glomerulonephritis.

CASE PRESENTATION

A 73-year-old Caucasian woman with a long-standing history of rheumatoid arthritis and Waldenström's macroglobulinemia was admitted for acute renal failure (ARF), which initially was presumed to be the consequence of extrarenal causes. Proteinuria and hematuria were only mild. In renal core biopsy, a membranoproliferative glomerulonephritis (MPGN) and prominent intracapillary hyaline monoclonal IgM thrombi were found in addition to acute tubular necrosis. Of note, the patient's history was positive for purpuric skin changes, suspicious for cryoglobulinemia. However, serological tests for cryoglobulins were repeatedly negative. The ARF resolved before the start of immunomodulatory therapy for Waldenström's macroglobulinemia.

CONCLUSION

The presence of MPGN with prominent hyaline thrombi in the context of Waldenström's macroglobulinemia is uncommon and can be oligosymptomatic. We discuss this case in the context of previous literature and classifications suggested for monoclonal Ig-related renal pathologies.

摘要

背景

导致副蛋白血症的淋巴增生性疾病可引起各种肾脏损伤,包括单克隆免疫球蛋白(Ig)的沉积。瓦尔登斯特伦巨球蛋白血症的一种已知肾小球表现为特征性的毛细血管内透明血栓形成,且无明显的肾小球增殖。本病例有两个特点:1. 在肾活检之前,肾小球肾炎的诊断出乎意料;2. 瓦尔登斯特伦巨球蛋白血症相关肾小球肾炎中,大量毛细血管内透明血栓形成与明显的肾小球增殖并存并不常见。

病例介绍

一名 73 岁的白人女性,患有长期的类风湿关节炎和瓦尔登斯特伦巨球蛋白血症,因急性肾衰竭(ARF)入院,最初认为是肾外原因所致。蛋白尿和血尿仅为轻度。在肾活检中,除急性肾小管坏死外,还发现膜增生性肾小球肾炎(MPGN)和明显的毛细血管内透明单克隆 IgM 血栓。值得注意的是,患者有紫癜性皮肤改变史,疑诊为冷球蛋白血症。然而,冷球蛋白的血清学检查反复呈阴性。在开始用免疫调节剂治疗瓦尔登斯特伦巨球蛋白血症之前,ARF 已得到缓解。

结论

在瓦尔登斯特伦巨球蛋白血症的背景下,存在 MPGN 伴明显透明血栓形成并不常见,且可能症状不明显。我们结合之前的文献和建议的单克隆 Ig 相关肾脏病理分类讨论了这个病例。

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