B细胞型幼淋巴细胞白血病1例并文献复习

目的:提高对B细胞型幼淋巴细胞白血病（B-PLL）的诊治认识。方法:回顾分析了我院1例B-PLL患者的临床表现、骨髓细胞形态学、免疫分型、不良基因检测等临床资料以及治疗过程,并结合文献进行学习讨论。结果:B-PLL患者初诊时白细胞总数较高,脾肿大；骨髓形态学呈体积中等的幼稚淋巴细胞，核圆、核仁清晰、核染色质浓密、核浆比例低的特点；成熟B细胞免疫表型，免疫球蛋白轻链限制性表达，排除其他淋巴增殖性疾病，诊断为B细胞型幼淋巴细胞白血病。该患者TP53缺失，给予R-CHOP方案治疗，治疗反应差。结论:形态学检查和免疫表型是诊断B-PLL的重要手段，同时需整合临床表现、细胞遗传学等结果综合判断，TP53基因缺失或突变会导致这类患者传统化疗的预后不良。

B-cell lymphocytic leukemia:One case report and literature review

Objective:To improve the diagnosis and treatment of B-cell lymphocytic leukemia (B-PLL).Methods:The clinical features,morphology,immunophenotyping,genetic testing and treatment of one case of B-PLL patients in our hospital were retrospectively analyzed,and the related literatures were reviewed．Results:B-PLL patients had a higher count of white blood cells and massive splenomegaly at the initial diagnosis.The bone marrow morphology is characterized by immature lymphocytes of medium volume which has a round and prominent central nucleolus with condensed chromatin and decreased nuclear to pulp ratio.Combining with mature B cell immune phenotype,restricted expression of immunoglobulin light chain,it was finally diagnosed as B-cell lymphoblastic leukemia while excluding other lymphoid proliferation.The patient was deficient in TP53 and treated with r-chop regimen whose response was poor.Conclusion:The morphology and the immune phenotype is an important methods for the diagnosis of B-PLL,but need to integrate the results of clinical manifestations,cytogenetics,and other comprehensive judgments.Deletion or mutation of the TP53 gene may result in poor prognosis of conventional chemotherapy in these patients.