PDF(Pubmed)
Abstract：
Waldenström\'s disease is a rare lymphoproliferative syndrome in the bone marrow and sometimes in lymphoid organs which secretes high amounts of monoclonal immunoglobulin M into serum. It can remain indolent for years and rarely affects the kidney, with intraglomerular rather than intratubular damage being predominant, in contrast to multiple myeloma. Different studies identified AL amyloidosis as the most frequent renal lesion, followed by cryoglobulinemic glomerulonephritis. Signs and symptoms may be unspecific, as well as renal manifestations, so collaboration between nephrologists, hematologists, and pathologists is crucial to establish the role of paraprotein in the development of renal damage. We present an atypical case of Waldenström\'s disease that had a minimal monoclonal peak and clinically debuted with nephritic and nephrotic syndromes. The diagnosis was cryoglobulinemic glomerulonephritis. Currently, there are numerous treatment options, without enough evidence yet to establish a standardised treatment.
摘要：
Waldenström病是骨髓中的一种罕见的淋巴增生综合征，有时在淋巴器官中会分泌大量的单克隆免疫球蛋白M进入血清。它可以保持惰性多年，很少影响肾脏，以肾小球内而不是肾小管内损伤为主，与多发性骨髓瘤相反。不同的研究确定AL淀粉样变性是最常见的肾脏病变，其次是冷球蛋白血症性肾小球肾炎。体征和症状可能不明确，以及肾脏表现，所以肾病学家之间的合作，血液学家,病理学家对确定副蛋白在肾损害发展中的作用至关重要。我们介绍了Waldenström病的非典型病例，该病例具有最小的单克隆峰，并在临床上首次出现肾病和肾病综合征。诊断为冷球蛋白血症性肾小球肾炎。目前,有许多治疗选择，没有足够的证据来建立标准化的治疗方法。
