边缘区淋巴瘤的诊断与治疗进展

Diagnosis and treatment advance of marginal zone lymphoma

边缘区淋巴瘤是起源于边缘区记忆淋巴细胞的惰性肿瘤,属于小B细胞淋巴瘤。边缘区淋巴瘤分为脾边缘区淋巴瘤、黏膜相关淋巴组织淋巴瘤和淋巴结边缘区淋巴瘤。它们有各自不同的临床特征、形态学和病理学特征、免疫表型特征及分子遗传学特征,对其综合分析有助于各型边缘区淋巴瘤的诊断,以及与其他小B细胞淋巴瘤的鉴别诊断。近年来,随着对边缘区淋巴瘤发病机制的研究不断深入,临床治疗不断进展,许多新药物逐渐应用到临床试验中,为边缘区淋巴瘤患者的治疗提供新的希望。

The marginal zone lymphoma (MZL) is an invasive tumor originating from the memory cells in the marginal zone and belongs to small B-cell lymphoma. MZL is divided into spleen marginal zone lymphoma (SMZL), mucosa-associated lymphoid tissue (MALT) lymphoma,and lymph node marginal zone lymphoma (NMZL). They have different clinical characteristics, morphological and pathological features, immunophenotypic characteristics and molecular genetic characteristics. Comprehensive analysis is helpful for the diagnosis of marginal lymphoma subtypes and differential diagnosis with other small B cell lymphomas.In recent years, with further study of the pathogenesis of MZL, clinical treatment has been progressing, and many new drugs have been used in clinical trails, promising new hope for MZL patients.