一例儿童幼稚T细胞型急性淋巴细胞白血病的临床和生物学特征  

Clinical and Biological Features in A Case of Child Pre-T-Cell Leukemia

作　　者：肖昕[1,2] 陈少华[1,2] 郭志强[1,2] 何国玲[1,2] 李扬秋[1,2] 

机构地区：[1]暨南大学医学院第一附属医院儿科 [2]暨南大学医学院血液病研究室

出　　处：《暨南大学学报（自然科学与医学版）》1998年第6期84-85,共2页Journal of Jinan University(Natural Science & Medicine Edition)

摘　　要：患儿，男，１４岁，因发热、乏力、皮肤出血点入院。查体：Ｔ３７５℃、Ｐ１２６次／ｍｉｎ，Ｒ３６次／ｍｉｎ、ＢＰ１５／１１ｋＰａ；中度贫血外貌，四肢及躯干有散在性瘀点、瘀斑，双下肢踝部水肿，颈部、耳后、颌下、锁骨上窝、腋窝和腹股沟可触及１５ｃｍ×０．２ｃｍ的淋巴结，心肺无异常。腹膨隆，腹围６５ｃｍ，腹水征（＋），肝、脾肋缘下均１２ｃｍ。克氏征（－）。Ｘ线提示纵膈增宽和少量胸腔积液。Ｈｂ８０ｇ／Ｌ，ＷＢＣ３２５×１０９／Ｌ，幼稚细胞占０９，ＢＰＣ６１×１０９／Ｌ。骨髓增生活跃，原始＋幼稚淋巴细胞占０７８。细胞化学染色：ＰＯＸ和ＮＡＥ染色阴性。颈淋巴结活检示淋巴结构破坏，被大量弥漫分布的幼稚淋巴细胞取代。血ＬＤＨ１４５９Ｕ／Ｌ、ＡＳＴ４９Ｕ／Ｌ、γ－ＧＴ１４４Ｕ／Ｌ、总蛋白５５４ｇ／Ｌ和白蛋白２８８ｇ／Ｌ。白血病细胞免疫标记：ＣＤ７＋占０９９、ＣＤ５＋０９９，未见ＣＤ２、ＣＤ３、ＣＤ４、ＣＤ８和ＣＤ１ａ表达（外周血）；ＣＤ３４＋ＣＤ７＋０２７、ＣＤ３４＋ＣＤ５＋０２９（骨髓）。ＰＣＲ检测：白介素受体－２（ＩＬ－２Ｒ）基因和Ｔ细胞特异性转录因子ＧＡＴＡ－３阳性表达。根据外周血、骨髓涂片和颈淋巴结活?CASE: A 15-year-old Chinese child with precursor T cell acute lymphoblastic leukemia(pre-T-ALL)is reported.He had a fever,weakness,cutaneous hemorrhage,cervical lymph node swelling,hepatosplenomegaly,ascites,adema in low extremities and mediastinal enlargement.Peripheral leukocyte counts were 325×10 9/L,of those,juvenile lymphocytes account for 0.9.The specimens of the bone marrow and cervical lymph node showed the presence of diffuse lymphoblast and juvenile lymphocytes,and POX,-,ANE- The lymphoblasts had immunophenotype CD7+99 2% and CD5+99 0% in the peripheral blasts,and CD34+CD7+27 0%,CD34+CD5+29 0% in bone marrow blasts;but lacked CD2,CD3,CD4,CD8 and CD1a.Serum LDH level was 1 459 U/L,AST 49 U/L,γ-GT 144 U/L,total protein and albumin 55 4g/L and 28 8g/L,respectively.Molecular genetic studies of lymphocytes demonstrated both positvie expression of interleukin-2 receptor(IL-2R) gene and T cell specific transcription factor GATA-3. DISCUSSION Diagnose of ALL(FAB L2 morphology)is based on the presence of leukemic cells in peripheral blood,bone marrow,lymph node,immunohistochemical stains,as well as clinical features.Pre-T-ALL is further classified by expression of T cell antigens. Pre-T-ALL is relatively rare in childhood.Positive expression of CD7 and CD5 has a high sensitivity and specificity for determination of pre-T-ALL.The child with pre-T-ALL has a poor prognosis due to his age of about 15 years,WBC>100×10 9/L,increasing LDH level,FAB L2 morphology,blast cell expression of IL-2R gene,hepatosplenomegaly and damaged liver function.High peripheral leukocytes can cause the infiltration and thrombopoiesis in brain blood vessel due to the aggressive feature of leukemic cells and increasing viscosity. It has been showed that a competition between IL-2R from leukemic cells and IL-2R on normal lymphocytes for IL-2 makes host-anti-leukemia immunity to diminish and cancer growth to accelerate.Hence,A careful consideration should be given to administration of IL-2 for pre-T-ALL with IL-2R expression.Exp

关 键 词：急性 淋巴细胞白血病 儿童 白血病 生物学特征 

分 类 号：R733.71[医药卫生—肿瘤]