移植后淋巴组织增生性疾病的诊断和治疗进展

Advanle in the diagnosis and treatment of posttransplantation lymphoproliferative disorder

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。

Recenly,the incidence of posttransplatation lymphoproliferative disorder(PTLD) is rising,Immunodeficiency and EBV-infection are risk factors of pathogenesis related. The majority of PTLD originates from malignant proliferation of B-cell.Typical pathologic findings of PTLD are large number of plasmacytoid B cells in lymphotic tissue and often with necrosis in regional areas. Molecular biology findings are ras or p53 gene mutation and c-myc?bcl-6 gene rearrangement. Highly immunosuppressed patients may present with diffuse infiltration and functional failure of multiple organs,the prognosis is poor. Except for the decrease or discontinuation of the immunosuppressive therapy,antiviral agents and interferon- α,anti B-cell monoclonal antibodies,autologous EBV-specific cytotoxic T cell and combination chemotherapy may achieve better responses.