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近年来 ,移植后淋巴组织增生性疾病 (PTLD)的发病率呈上升趋势 ,其发病危险因素与免疫缺陷、EBV感染有关。大多数PTLD起源于B细胞单克隆恶性增殖。典型的病理改变为淋巴组织中有大量浆细胞样B细胞 ,并常有局灶性坏死。分子生物学检查显示ras或p5 3基因突变和c myc、bcl 6基因重排。重度免疫抑制患者可出现多脏器弥漫性浸润、功能障碍 ,病情进展快 ,预后差。治疗上除免疫抑制剂减量外 ,抗病毒和干扰素治疗、抗B细胞单抗、自身EBV特异性毒性T淋巴细胞输注和联合化疗已取得一定疗效
In recent years, the incidence of post-transplant lymphoproliferative disease (PTLD) is on the rise, and its risk factors are related to immunodeficiency and EBV infection. Most PTLDs originate from B cell monoclonal malignant proliferation. A typical pathological change is a large number of lymphoid plasmacytoid B cells, and often focal necrosis. Molecular biology examination revealed ras or p5 3 gene mutations and c-myc, bcl-6 gene rearrangements. Patients with severe immunosuppression may appear multiple organ diffuse infiltration, dysfunction, the disease progresses rapidly, the prognosis is poor. In addition to immunosuppressive therapy, antiviral and interferon treatment, anti-B cell monoclonal antibody, its own EBV-specific T lymphocyte infusion and chemotherapy have achieved some effect