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单克隆丙种球蛋白病和血清免疫球蛋白水平作为慢性淋巴细胞白血病的预后因素。

Monoclonal gammopathy and serum immunoglobulin levels as prognostic factors in chronic lymphocytic leukaemia.

机构信息

Department of Hematology, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.

Department of Hematology, Università Cattolica del Sacro Cuore, Rome, Italy.

出版信息

Br J Haematol. 2020 Sep;190(6):901-908. doi: 10.1111/bjh.16975. Epub 2020 Jul 26.

Abstract

The relationship between chronic lymphocytic leukaemia (CLL) and qualitative/quantitative gammaglobulin abnormalities is well established. Nevertheless, in order to better understand this kind of connection, we examined 1505 patients with CLL and divided them into four subgroups on the basis of immunoglobulin (Ig) aberrations at diagnosis. A total of 73 (4·8%), 149 (10%), 200 (13·2%) and 1083 (72%) patients were identified with IgM monoclonal gammopathy (IgM/CLL), IgG monoclonal gammopathy (IgG/CLL), hypogammaglobulinaemia (hypo-γ) and normal Ig levels (γ-normal) respectively. IgM paraprotein was significantly associated with a more advanced Binet/Rai stage and del(17p)/TP53 mutation, while IgG abnormalities correlated with a higher occurrence of trisomy 12. Patients with any type of Ig abnormality had shorter treatment-free survival (TFS) but no significant impact affecting overall survival (OS) compared to those with normal Ig levels.

摘要

慢性淋巴细胞白血病(CLL)与质/量球蛋白异常之间的关系已得到充分证实。然而,为了更好地理解这种关联,我们检查了 1505 例 CLL 患者,并根据诊断时的免疫球蛋白(Ig)异常将他们分为四个亚组。分别有 73 例(4.8%)、149 例(10%)、200 例(13.2%)和 1083 例(72%)患者被诊断为 IgM 单克隆丙种球蛋白病(IgM/CLL)、IgG 单克隆丙种球蛋白病(IgG/CLL)、低丙种球蛋白血症(hypo-γ)和正常 Ig 水平(γ-正常)。IgM 副蛋白与更晚期的 Binet/Rai 分期和 del(17p)/TP53 突变显著相关,而 IgG 异常与 12 号三体的发生率更高相关。与 Ig 水平正常的患者相比,任何类型的 Ig 异常患者的无治疗生存时间(TFS)更短,但对总生存(OS)没有显著影响。

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