CAJ | 학술논문

目的探讨大颗粒淋巴细胞白血病(LGLL)的临床特征及诊疗方法.方法回顾性分析3例LGLL患者的临床资料并复习相关文献.结果 3例患者均以淋巴细胞增多起病,病情进展缓慢,其中2例为T-LGLL,免疫表型特征为CD3+ CD4-CD8+ CD56-CD57+,1例为NK-LGLL,免疫表型特征为CD3-CD4-CD8-CD56+ CD57-.2例患者暂时不需要临床干预,1例患者因粒细胞缺乏及反复感染给予环孢素治疗.结论 LGLL是一组异质性疾病,其临床特征及预后各不相同.流式细胞术免疫表型分析及TCRVβ基因谱型分析、TCR基因重排等检测有利于明确诊断.
목적 탐토대과립림파세포백혈병(LGLL)적림상특정급진료방법.방법 회고성분석3례LGLL환자적림상자료병복습상관문헌.결과 3례환자균이림파세포증다기병,병정진전완만,기중2례위T-LGLL,면역표형특정위CD3+ CD4-CD8+ CD56-CD57+,1례위NK-LGLL,면역표형특정위CD3-CD4-CD8-CD56+ CD57-.2례환자잠시불수요림상간예,1례환자인립세포결핍급반복감염급여배포소치료.결론 LGLL시일조이질성질병,기림상특정급예후각불상동.류식세포술면역표형분석급TCRVβ기인보형분석、TCR기인중배등검측유리우명학진단.
Objective To investigate the clinical characteristics and methods of diagnosis and treatment of granular lymphocytic leukemia (LGLL).Methods Clinical data of 3 patients with LGLL were retrospectively analyzed and relevant literature was reviewed.Results 3 patients were all onset with lymphocytosis,whose conditions progressed slowly.The diagnosis of 2 patients was T-LGLL with immunological characteristics of CD3+ CD4 CD8+ CD56-CD57+.The other patient' s diagnosis was NK-LGLL,whose immunological characteristic was CD3-CD4-CD8-CD56+ CD57-.Two of them didn' t need any treatment.One of them was treated with cyclosporine because of agranulocytosis and recurrent infection.Conclusions LGLL is a group of heterogeneous diseases,which clinical characteristic and prognosis are different.Flow cytometric immunopheotype,TCR Vβ analysis and TCR gene rearrangement are helpful to diagnosis.