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[32岁无症状女性吸烟者,伴有持续性多克隆淋巴细胞增多症]

[Asymptomatic 32 year old female smoker with persistent polyclonal lymphocytosis].

作者信息

Chapuy B, Wulf G, Tessen H W, Griesinger F, Trümper L, Schroers R

机构信息

Abteilung für Hämatologie und Onkologie, Universitätsklinikum Göttingen, Göttingen, Deutschland.

出版信息

Internist (Berl). 2007 Mar;48(3):314-8. doi: 10.1007/s00108-007-1800-6.

Abstract

A 32 year old female smoker (20 pack years) presented with an asymptomatic lymphocytosis of 13,000/nl and splenomegaly. The patient's blood smear showed an absolute lymphocytosis with 65% atypical lymphocytes. A total of 1% of the lymphocytes were bilobulated. Bone marrow histology and immunphenotyping of blood and bone marrow excluded leukemia and non-Hodgkin's lymphoma. IgH-CDR-3 PCR analysis revealed a polyclonal pattern. In summary, a persistent polyclonal B-cell-lymphocytosis (PPBL) was diagnosed. The exact etiology of PPBL is still unclear, however, it is associated with a polyclonal raise in the lymphocyte count of CD27+IgD+-memory-B-lymphocytes due to a defect in apoptosis signaling and leukocyte homing to secondary lymphoid tissues. An association with cigarette smoking is obvious since all patients are smokers. From all published cases, only two developed a malignancy with an uncertain association with PPBL. We have been monitoring our patient for 6.5 years without any evidence of the development of a lymphoma.

摘要

一名32岁女性吸烟者(吸烟史20包年)出现无症状性淋巴细胞增多,淋巴细胞计数为13,000/微升,并有脾肿大。患者的血涂片显示绝对淋巴细胞增多,其中65%为非典型淋巴细胞。共有1%的淋巴细胞为双叶核。骨髓组织学检查以及血液和骨髓的免疫表型分析排除了白血病和非霍奇金淋巴瘤。IgH-CDR-3聚合酶链反应分析显示为多克隆模式。综上所述,诊断为持续性多克隆B细胞淋巴细胞增多症(PPBL)。PPBL的确切病因仍不清楚,然而,由于凋亡信号传导缺陷以及白细胞归巢至二级淋巴组织,它与CD27+IgD+记忆B淋巴细胞的淋巴细胞计数多克隆升高有关。吸烟与该病的关联很明显,因为所有患者均为吸烟者。在所有已发表的病例中,只有两例发生了恶性肿瘤,与PPBL的关联尚不确定。我们对该患者进行了6.5年的监测,未发现任何淋巴瘤发生的证据。

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