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一例表现为肾病综合征的罕见非典型慢性淋巴细胞白血病病例。

A rare case of atypical chronic lymphocytic leukaemia presenting as nephrotic syndrome.

作者信息

Soldarini Martina, Farina Lucia, Genderini Augusto, Bolli Niccolo

机构信息

Dipartimento di Oncologia ed Onco-Ematologia, Universita' degli Studi di Milano, Milano, Italy.

Dipartimento di Oncologia medica ed Ematologia, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy.

出版信息

BMJ Case Rep. 2017 Jul 14;2017:bcr-2016-218850. doi: 10.1136/bcr-2016-218850.

Abstract

Chronic lymphocytic leukaemia (CLL) is characterised by a lymphocytosis of mature-appearing clonal CD5+, CD23+ B lymphocytes. CLL cells arise from the bone marrow and infiltrate lymphoid tissues such as lymph nodes and spleen. Presentation is usually through discovery of lymphocytosis or lymphadenopathy. Unusual presentations, especially paraneoplastic syndromes are rare. Here, we describe a rare case presenting with severe nephrotic syndrome associated with the presence of a monoclonal protein in serum. Workup for suspected plasma cell dyscrasia led instead to the diagnosis of bone marrow infiltration by atypical CLL without lymphocytosis. Renal biopsy showed a glomerulonephritis that turned out to be paraneoplastic as it went into remission after treatment for CLL. Our case shows an unusual presentation of CLL and prompts for increased awareness of lymphoproliferative disorders in the context of seemingly unrelated conditions that may be paraneoplastic in origin.

摘要

慢性淋巴细胞白血病(CLL)的特征是出现成熟的克隆性CD5 +、CD23 + B淋巴细胞的淋巴细胞增多。CLL细胞起源于骨髓,并浸润淋巴结和脾脏等淋巴组织。通常通过发现淋巴细胞增多或淋巴结病来确诊。不寻常的表现,尤其是副肿瘤综合征很少见。在此,我们描述了一例罕见病例,该病例表现为严重肾病综合征并伴有血清中单克隆蛋白。对疑似浆细胞异常增殖症的检查反而导致诊断为非典型CLL骨髓浸润但无淋巴细胞增多。肾活检显示为肾小球肾炎,经CLL治疗后病情缓解,结果证明是副肿瘤性的。我们的病例显示了CLL的一种不寻常表现,并促使人们在看似无关但可能起源于副肿瘤的情况下提高对淋巴增殖性疾病的认识。

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