Au Wing Y, Lam Clarence C, Lie Albert K, Pang Annie, Kwong Yok L
University Department of Medicine, Queen Mary Hospital, Hong Kong, People's Republic of China.
Am J Clin Pathol. 2003 Oct;120(4):626-30. doi: 10.1309/VA75-5A03-PVRV-9XDT.
A 39-year-old man with chronic myeloid leukemia in accelerated phase underwent allogeneic bone marrow transplantation (BMT). At 6 months after BMT, lymphocytosis (WBC count, 23,100/microL [23.1 x 10(9)/L]; 80% (0.80) large granular lymphocytes [LGLs]) occurred. The LGLs were CD3+CD4-CD8+, with clonally rearranged T-cell receptor gamma gene, and of donor origin, as shown by analysis of polymorphic microsatellite markers. Epstein-Barr virus was not present. The diagnosis, therefore, was consistent with T-cell large granular lymphocytic (T-LGL) leukemia. Corticosteroids controlled the LGL count, but progressive pancytopenia led to death 4 months later. Retrospective analysis showed that the T-LGL leukemia apparently had arisen as early as 3 months after BMT. The distinguishing features of this case included donor origin, neoplastic nature, and the aggressive fatal outcome.
一名处于加速期的慢性髓性白血病39岁男性接受了异基因骨髓移植(BMT)。BMT后6个月,出现淋巴细胞增多症(白细胞计数,23,100/μL [23.1×10⁹/L];80%(0.80)为大颗粒淋巴细胞[LGLs])。LGLs为CD3⁺CD4⁻CD8⁺,T细胞受体γ基因发生克隆性重排,且通过多态性微卫星标记分析显示为供体来源。未检测到爱泼斯坦-巴尔病毒。因此,诊断符合T细胞大颗粒淋巴细胞(T-LGL)白血病。皮质类固醇控制了LGL计数,但进行性全血细胞减少导致4个月后死亡。回顾性分析显示,T-LGL白血病显然早在BMT后3个月就已出现。该病例的显著特征包括供体来源、肿瘤性质以及侵袭性致命结局。