大颗粒淋巴细胞白血病临床特征分析

Analysis of the Clinical Characteristics of Large Granular Lymphocytic Leukemia

目的提高对大颗粒淋巴细胞白血病(LGLL)的认识。方法计算机检索中国生物医学文献数据库、中国期刊网全文数据库(CNKI)、万方数据库,并结合文献追溯的方法收集2000年至2015年国内LGLL的文献,从临床特征、免疫表型及治疗等方面进行归纳分析。结果共检索出76篇文献,最终纳入29篇,共50例LGLL患者。除9例患者未报道就诊原因外,LGLL患者首发症状以贫血相关症状、发热为主,分别占68.3%(28/41)和17.1%(7/41),体检发现血象异常就诊者占4.9%(2/41);合并肝、脾、淋巴结肿大者分别占26%(13/50)、60%(30/50)和6%(3/50),合并类风湿因子阳性者占12%(6/50),合并纯红细胞再生障碍性贫血者占46%(23/50)。流式细胞术免疫表型分析结果提示T-LGLL占72.9%(35/48),NK-LGLL占27.1%(13/48),T-LGLL患者免疫表型主要为CD3+,CD4-,CD8+,CD16+,CD56-,CD57+;NKLGLL患者免疫表型主要为CD3-,CD4-,CD8-,CD16+,CD56+,CD57-。惰性T-LGLL目前多采用基于小剂量环孢素(CSA)的免疫抑制治疗,亦有应用小剂量激素联合免疫抑制剂/雄激素类似药物治疗的,预后相对较好,而侵袭性T-LGLL和NK-LGLL多采用高强度的联合方案化疗或者造血干细胞移植治疗,预后较差。结论 LGLL临床罕见,其临床特征、免疫表型和预后等方面有显著不同。惰性T-LGLL治疗方案单一,预后较好,而侵袭性T-LGLL和NK-LGLL多采用联合方案化疗,预后较差。

Objective To deepen the understanding of large granular lymphocytic leukemia （LGLL）. Methods The literature about LGLL in Chinese Bio-medicine Database（CBM） , China National Knowledge Infrastructure （CNKI） and Wan fang DATA were searched by computer retrieval, combined with the literature trace in 2000- 2015 ,and analyzed from aspects of clinical manifestations, immune phenotype and treatment of the disease. Results Total 76 articles were retrieved and 29 of them including a total of 50 patients with LGLL were involved in the study. LGLL patients with anemia, fever as the major initial symptoms, and with abnormal blood in medical examinations were accounted for 68.3% （28/41）, 17.1% （7/41） and 4.9% （2/41） ,respectively. The proportions of hepatomegaly, splenomegaly, and lymphadenectasis associated with LGLL were 26% （13/50）, 60% （30/50） and 6% （3/50）, respectively. In addition, the cases with positive rheumatoid factor, pure red cell aplastic anemia（PRCA）were accounted for 12% （6/50）and 46% （23/50）. The analysis of immune phenotype by flow cytometry indicated that T- LGLL（ CD3 ＋ , CD4 -, CD8 ＋ , CD16 ＋ , CD56 -, CD57 ＋） accounted for 72.9% （ 35/48 ） , whereas NK- LGLL（ CD3 - , CD4 - , CD8 - , CD16 ＋ , CD56＋ , CD57 - ） accounted for 27.1% （ 13/48 ）. At present, the immunosuppressive therapy based on small doses of CsA were usually used in treatment of inert T-LGLL, and other therapy like small doses of the hormone combined with immunosuppressant / androgen analogue were also applied with relatively good prognosis. However, high insensitive combined chemotherapy protocols or hematopoietie stem cell transplantation were generally adopted in treatment of invasive T-LGLL andNK-LGLL with poor prognosis. Conclusion Large granular lymphocytic leukemia which was rarely seen in clinic has significantly different clinical features, immune phenotype and prognosis. Single treatment protocols and combined chemotherapy protocols are usually used in inert T-LGLL with good prognosis and invasive T-LGLL and NK-LGLL with poor prognosis, respectively.