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目的:探讨急性儿童系统性EB病毒阳性T细胞淋巴组织增殖性疾病的发病机制、临床病理特征及鉴别诊断要点,以缩短诊断时间和减少误诊。方法:结合文献分析1例急性儿童系统性EB病毒阳性T细胞淋巴组织增殖性疾病死亡病例的临床症状和体征、病理特征及免疫组织化学、EBER原位杂交、基因克隆重排结果等。结果:急性儿童系统性EB病毒阳性T细胞淋巴组织增殖性疾病临床上主要表现为嗜血综合征,包括发热、淋巴结及肝脾肿大、外周血三系减少,可伴有腹水及胸腔积液,血清EB病毒载量增高、血清铁蛋白明显增高,肝肾功能、凝血、血脂等均异常;骨髓涂片示异型淋巴细胞约占18%,并可见嗜血现象。淋巴结活检示其结构破坏,淋巴滤泡减少,T区明显扩大,可见轻-中度异型淋巴细胞;淋巴窦扩张,组织细胞增生,可见嗜血现象,间质血管增生。免疫组化证实EB病毒感染的细胞毒性T细胞构成病变主体;EBER原位杂交部分淋巴细胞胞核阳性;淋巴结组织标本基因重排示TCR基因发生克隆性重排,患者在发病第27天因多脏器衰竭死亡。结论:急性儿童系统性EB病毒阳性T细胞淋巴组织增殖性疾病是一种系统性病变,部分患者病情急剧恶化死于严重并发症。该病病情多较复杂,且与其它疾病存在重叠或交叉,早期确诊困难,目前证实其中T淋巴细胞增生为克隆性增生,为T细胞淋巴瘤。应提高对其病理认识,并紧密结合临床、检验、免疫表型、基因重排等因素,减少治疗延误。
Objective: To investigate the pathogenesis, clinicopathological features and differential diagnosis of acute Epstein-Barr virus-positive T cell lymphoproliferative diseases in children with acute hepatitis B, in order to shorten the diagnosis time and reduce misdiagnosis. Methods: The clinical symptoms and signs, pathological features, immunohistochemistry, EBER in situ hybridization and gene cloning and rearrangement of 1 case of acute Epstein-Barr virus-positive T cell lymphoproliferative diseases were analyzed in combination with the literature. Results: Acute children with systemic Epstein-Barr virus positive T-cell lymphoproliferative diseases mainly manifested as bloodthirsty syndrome, including fever, lymph nodes and hepatosplenomegaly, decreased peripheral blood three lines, may be associated with ascites and pleural effusion , Serum EBV increased, serum ferritin was significantly higher, liver and kidney function, blood clotting, blood lipids were abnormal; bone marrow smear showed abnormal lymphocytes accounted for about 18%, and can see the bloodthirsty phenomenon. Lymph node biopsy showed its structural destruction, lymphoid follicles decreased, T area was significantly expanded, showing mild to moderate allopathic lymphocytes; lymphatic sinus dilatation, tissue cell proliferation, visible bloodthirsty phenomenon, interstitial vascular proliferation. Immunohistochemistry showed that the EBV-infected cytotoxic T cells formed the main lesion; EBER was positive for some lymphocytes; lymph node tissue gene rearrangement showed clonal rearrangement of TCR gene. On the 27th day, Organ failure and death. Conclusion: Acute systemic lupus erythematosus (EBV) -positive T-cell lymphoproliferative disorder is a systemic disease. Some patients died of serious complications due to their serious deterioration. The disease is more complicated, and overlap with other diseases or cross, early diagnosis difficult, confirmed that T lymphocyte hyperplasia clonal hyperplasia, T cell lymphoma. Should increase their understanding of the pathology, and closely combined with clinical, testing, immunophenotype, gene rearrangement and other factors to reduce the treatment delay.