1例意义不明的单克隆γ病并检测到费城染色体

Q3 Medicine
T. Tran, Jennifer Cai, P. Ji, Xin Qing
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引用次数: 0

摘要

背景:未确定意义单克隆γ病(MGUS)和慢性髓系白血病(CML)是不同谱系的疾病。在同一患者中同时诊断MGUS和CML是一种罕见的情况,在很多文献中没有报道。我们描述了一个56岁的男性与类风湿关节炎的历史偶然发现有增加的IgA副蛋白。骨髓活检中单克隆浆细胞少于10%,且无高钙血症、肾功能衰竭、贫血和骨骼病变,诊断为MGUS。当时的常规细胞遗传学显示费城染色体存在于30%的中期。然而,在外周血或骨髓中没有CML的形态学证据。患者未接受任何治疗并失去随访,直到3年后常规CBC显示白细胞增多和血小板增多。慢性粒细胞白血病,慢性期诊断后骨髓穿刺和活检,费城染色体在中期观察到100%。患者先用伊马替尼治疗,后来改用达沙替尼,并继续获得完全的分子缓解。讨论与结论我们在此报告一例白血病前CML是在MGUS诊断过程中偶然发现的。尽管共存的确切原因尚不清楚,但仍讨论了这种关联的可能潜在机制。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A Patient with Monoclonal Gammopathy of Undetermined Significance and Detected Philadelphia Chromosome
Background Monoclonal gammopathy of undetermined significance (MGUS) and chronic myeloid leukemia (CML) are diseases of different lineages. The diagnosis of both MGUS and CML in the same patient is a rare occurrence and has not been reported in much literature. Case Presentation We describe a 56-year-old man with a history of rheumatoid arthritis incidentally found to have an increase in IgA paraprotein. With less than 10% monoclonal plasma cells on the bone marrow biopsy and absence of hypercalcemia, renal failure, anemia and bone lesions, MGUS was diagnosed. The conventional cytogenetics at the time showed the presence of the Philadelphia chromosome in 30% of metaphases. However, there was no morphologic evidence of CML in the peripheral blood or bone marrow. Patient received no treatment and lost follow-up until 3 years later when a routine CBC showed leukocytosis and thrombocytosis. CML, chronic phase was diagnosed following a bone marrow aspiration and biopsy with Philadelphia chromosome observed in 100% of metaphases. Patient was treated with imatinib and later switched to dasatinib and complete molecular remission was continued to be achieved. Discussion and Conclusion Here we report a case of pre-leukemic CML as an incidental finding during the diagnosis of MGUS. The possible underlying mechanisms of the association are discussed although the exact cause of the coexistence is unclear.
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来源期刊
CiteScore
1.30
自引率
0.00%
发文量
32
审稿时长
12 weeks
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