Sabnani I, Zucker M J, Tsang P, Palekar S
Department of Oncology/Hematology, Newark Beth Israel Medical Center, Newark, New Jersey 07112, USA.
Transplant Proc. 2006 Dec;38(10):3437-40. doi: 10.1016/j.transproceed.2006.10.045.
Large granular lymphocytic (LGL) leukemia is a rare disorder, usually caused by clonal proliferation of CD3+ CD57+ T-LGL cells. T-cell clonality is confirmed by rearrangements of the T-cell receptor (TCR) gene. Characteristic features of T-LGL leukemia include neutropenia, anemia, and constitutional symptoms such as fatigue. Many solid organ transplant recipients experience similar symptoms and have neutropenia and anemia often attributed to immunosuppressive therapy. The purpose of this study was to determine the prevalence of T-LGL proliferation in solid organ transplant recipients and demonstrate its association with leukopenia and anemia. Twenty-three cardiac and renal transplant patients were evaluated by peripheral smear examination, flow cytometry, and TCR gene rearrangement study by polymerase chain reaction. Ten of 14 (71%) cardiac transplant patients and 4 of 9 (44%) renal transplant patients, without evidence of either allograft rejection or a viral syndrome, were found to have clonal expansion of T-LGL cells. Constitutional symptoms were present in 30% of these patients. Anemia of <10 g/dL was seen in 75% of renal transplant and 10% of cardiac transplant patients. None of these patients had significant neutropenia defined as absolute neutrophil count of 1500 mu/L. Most of the patients did not require any specific therapeutic intervention. Although TCR gene rearrangement is considered a hallmark of T-LGL leukemia, we believe that this monoclonality is not a true form of posttransplant lymphoproliferative disorder. Constant antigenic stimulus from the allograft may be the underlying etiology of clonal expansion and may contribute to cytopenias and fatigue seen in transplant patients.
大颗粒淋巴细胞（LGL）白血病是一种罕见的疾病，通常由CD3 + CD57 + T-LGL细胞的克隆性增殖引起。T细胞克隆性通过T细胞受体（TCR）基因重排得以证实。T-LGL白血病的特征包括中性粒细胞减少、贫血以及诸如疲劳等全身症状。许多实体器官移植受者会出现类似症状，且常有归因于免疫抑制治疗的中性粒细胞减少和贫血。本研究的目的是确定实体器官移植受者中T-LGL增殖的患病率，并证明其与白细胞减少和贫血的关联。通过外周血涂片检查、流式细胞术以及聚合酶链反应进行的TCR基因重排研究，对23名心脏和肾脏移植患者进行了评估。在14名心脏移植患者中有10名（71%），9名肾脏移植患者中有4名（44%），在没有同种异体移植排斥或病毒综合征证据的情况下，被发现存在T-LGL细胞的克隆性扩增。这些患者中有30%出现全身症状。75%的肾脏移植患者和10%的心脏移植患者出现血红蛋白低于10 g/dL的贫血。这些患者中没有一人的中性粒细胞绝对计数低于1500 μ/L被定义为严重中性粒细胞减少。大多数患者不需要任何特定的治疗干预。尽管TCR基因重排被认为是T-LGL白血病的一个标志，但我们认为这种单克隆性并非移植后淋巴细胞增殖性疾病的真正形式。来自同种异体移植的持续抗原刺激可能是克隆性扩增的潜在病因，并且可能导致移植患者出现血细胞减少和疲劳。
