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[与单克隆丙种球蛋白病(IgA,κ型)相关的慢性中性粒细胞白血病]

[Chronic neutrophilic leukemia associated with monoclonal gammopathy (IgA, kappa type)].

作者信息

Kubo A, Kawanami M, Matsuyama E, Tamura T, Kanoh T

出版信息

Rinsho Ketsueki. 1989 Jun;30(6):858-62.

PMID:2507802
Abstract

A 62-year-old woman with chronic neutrophilic leukemia (CNL) is described. She presented in February 1988 for evaluation of leukocytosis of 3 years' duration with no complaint. Physical examination was normal. The leukocyte count was 20,100/microliters with 70% segmented neutrophils and 12% band forms. A myelogram showed marked myeloid hyperplasia and plasmacytosis (5.9%). Neutrophil alkaline phosphatase score, serum lysozyme and vitamin B12 levels were elevated. Cytogenetic analysis of the marrow aspirate showed normal karyotype, with no Philadelphia chromosome. Total serum protein (TP) was 7.5 g/dl with increased beta-globulin (23.5%), identified as monoclonal IgA kappa (3.3 g/dl) on immunoelectrophoresis. No activity of G-CSF was detected in the serum. A retrospective study revealed that the beta-globulin level was normal (6.3%, TP 6.9 g/dl) in 1980 and that it was slightly increased (11.6%, TP 7.0 g/dl) without leukocytosis (5,900/microliter) in 1981. In 1985, when leukocytosis obviously existed (9,900/microliter), the percentage of beta-globulin was increased to 17.5% (TP 7.2 g/dl). The possibility that monoclonal gammopathy preceded the leukocytosis must be admitted. On the basis of our observation, it is assumed that CNL and monoclonal gammopathy may be blood dyscrasias derived from a common precursor cell or that the immunological abnormality associated with monoclonal gammopathy may be implicated in the development of CNL.

摘要

本文描述了一名62岁的慢性中性粒细胞白血病(CNL)女性患者。她于1988年2月因持续3年的白细胞增多症前来评估,无任何不适主诉。体格检查正常。白细胞计数为20,100/微升,其中70%为分叶核中性粒细胞,12%为杆状核细胞。骨髓象显示明显的髓系增生和浆细胞增多(5.9%)。中性粒细胞碱性磷酸酶评分、血清溶菌酶和维生素B12水平升高。骨髓穿刺液的细胞遗传学分析显示核型正常,无费城染色体。血清总蛋白(TP)为7.5 g/dl,β球蛋白增加(23.5%),免疫电泳鉴定为单克隆IgA κ(3.3 g/dl)。血清中未检测到G-CSF活性。一项回顾性研究显示,1980年β球蛋白水平正常(6.3%,TP 6.9 g/dl),1981年略有升高(11.6%,TP 7.0 g/dl),但无白细胞增多(5,900/微升)。1985年,当明显存在白细胞增多(9,900/微升)时,β球蛋白百分比增加至17.5%(TP 7.2 g/dl)。必须承认单克隆丙种球蛋白病先于白细胞增多症出现的可能性。根据我们的观察,推测CNL和单克隆丙种球蛋白病可能是源自共同前体细胞的血液系统疾病,或者与单克隆丙种球蛋白病相关的免疫异常可能与CNL的发生有关。

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