| 宋玉臻.利妥昔单抗联合奥布替尼治疗变异型毛细胞白血病一例[J].安徽医药,待发表. |
| 利妥昔单抗联合奥布替尼治疗变异型毛细胞白血病一例 |
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| 投稿时间：2023-04-16 录用日期：2023-05-25 |
| DOI： |
| 中文关键词: 毛细胞白血病 变异型 诊断 利妥昔单抗,奥布替尼 |
| 英文关键词: |
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| 中文摘要: |
| 目的 探讨变异型毛细胞白血病（HCL-V）的诊断、临床表现及治疗方案。方法 回顾性分析胜利油田中心医院收治的1例HCL-V患者的临床资料，并复习相关文献。结果 患者为71岁男性，伴有脾大及淋巴结肿大，根据骨髓形态、免疫学分型、免疫组化及分子生物学特点，患者符合HCL-V的诊断。患者应用1疗程利妥昔单抗联合克拉屈滨化疗方案后，脾脏明显缩小，但疗效评价为疾病稳定。后改用利妥昔单抗联合奥布替尼化疗方案5疗程，尽管疗效评价为部分缓解，但已接近完全缓解。至截稿前正在治疗中。结论 HCL-V临床罕见，诊断应结合骨髓形态、免疫学分型、免疫组化及分子生物学特点，临床上要和经典型毛细胞白血病、B幼淋巴细胞白血病、脾边缘区淋巴瘤、慢性淋巴细胞白血病将鉴别，目前尚无标准治疗方案。与其他布鲁顿型酪氨酸激酶抑制剂相比，奥布替尼提高了激酶的选择性和特异性，对布鲁顿型酪氨酸激酶以外的靶点作用极低。利妥昔单抗联合奥布替尼化疗在HCL-V患者的治疗中取得了良好的疗效，但具体远期疗效还需经后期随访观察。 |
| 英文摘要: |
| Objective To investigate the diagnosis,clinical manifestations and treatments of variant hairy cell leukemia (HCL-V). Methods The clinical data of 1 case of HCL-V in Shengli Oilfield Central Hospital were retrospectively analyzed,and the related literatures were reviewed. Results The patient was a 71-year-old male with splenomegaly and lymphadenectasis.According to the characteristics of bone marrow morphology,immunological classification,immunohistochemical and molecular biology,the patient conformed to the diagnosis of HCL-V. After one course of rituximab combined with cladribine chemotherapy, the spleen was significantly reduced,but the therapeutic evaluation was stable diease.Then rituximab combined with orelabrutinib chemotherapy was used for 5 courses.Although the efficacy evaluation was partial response,it was close to complete response.He was under treatment by the deadline. Conclusion HCL-V is rare in clinic, and its diagnosis should be combined with bone marrow morphology, immunotype, immunohistochemical and molecular biological characteristics. Clinically, HCL-V should be differentiated from typical hair-cell leukemia, B-lymphoblastic leukemia, splenic marginal zone lymphoma, and chronic lymphoblastic leukemia. Currently, there is no standard treatment plan. Compared with other Bruton-type tyrosine kinase inhibitors, obutinib improves kinase selectivity and specificity, and has very little effect on targets other than Bruton-type tyrosine kinase. Rituximab combined with aubutinib chemotherapy has achieved good efficacy in the treatment of HCL-V patients, but the specific long-term efficacy needs to be followed up at a later stage. |
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