Objective: To investigate the clinical characteristics and therapeutic responte of patients with B-CLPD mainly manifested as cytopenia, so as to deeply understand this disease.
Methods: The clinical data of 13 B-CLPD patients with hematocytopenia as main manifestation, and the absolute count of lymphocytes＜5×109/L, absence of hepatosplenic lymph-nodes and extramedullary invasion tin our department fron 2003 to 2018 were analyzed retrospectively. The clinical characteristics, therapeutic efficacy and adverse reactions of 3 patients were summarized.
Results: The median age of patients was 59 (43-76) years old, the median of lymphocyte was 1.86 (0.69-4.8) ×109/L, the levels of LDH and β2-microglubulin were normal in most patients, the monolineage and multilencage hematopoietic failure of different degrees existed in most all patients. The lymphocyte ratio in patients was 18.5%-94.0%, CD20 was positive in all patients, and yet the CD5-positive and CD-negative existed in 7 and 6 cases respectively. There was no significant difference in ratio of lymphocyte invasion among different immunophemtype. The FISH detection showed that there were no high risk genetic types. 92.3% of patients received rituximab treatment, most of them received chemotherapy of rituximab combined with C0P/CHOP like regimen, only 2 patients received fludarabine for comparatively short course. The analysis indicated that 8 out of 13 patients showed a certain theropeutic efficacy, however the drug-related hematopoietic suppression occurred in both 2 patients treated with fludarabin.
Conclusions: The B-CLPD accompanied with hematocytopenia often displays bone marrow hypohematopoiesis of different degree and easily confuses with the congenital and acquired hemotopoietic faiture diseases. The rituximab treatment may be more appropreate for these patients, but for patients received chemotherapy containing fludarabin, the persistant hematopoietic failure must be especially watched out.
题目: 以血细胞减少为特征的B-CLPD患者临床分析.
目的: 探讨以血细胞减少为主要特征的慢性B淋巴增殖性疾病（B-CLPD）患者临床特征及治疗反应，以加深对该类疾病的认识.
方法: 回顾性分析2003年至2018年我科收治的13例以血细胞减少为主要表现，淋巴细胞绝对值小于5×109/L，影像学上无任何肝脾淋巴结及髓外侵犯B-CLPD的患者的临床资料，总结该类患者临床特征、疗效及不良反应.
结果: 患者中位年龄59（43~76）岁，淋巴细胞中位数1.86（0.69~4.8）×109/L；大部分患者LDH及β2微球蛋白水平正常。几乎所有患者均存在不同程度单系及多系骨髓造血衰竭。淋巴细胞比例18.5%~94%不等。所有患者的CD20均阳性，其中CD5阳性7例，CD5阴性6例。不同类别免疫表型中，淋巴细胞侵犯比例无统计学差异。FISH检测显示无高危遗传型。92.3%患者接受利妥昔单抗的治疗，绝大部分还联合COP/CHOP样方案，仅2例患者短暂应用氟达拉滨。13例患者中8例有效。 2例应用氟达拉滨的患者，发生了药物相关造血恢复延迟.
结论: 伴有血细胞减少的B-CLPD往往合并不同程度骨髓造血低下,容易与先天性及获得性造血衰竭性疾病混淆。对这类患者在利妥昔单抗治疗或许更为合适，但对联合氟达拉滨的化疗的患者需警惕续持续性造血抑制.