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B细胞和T细胞幼淋巴细胞白血病是一类罕见,且预后不良的淋巴组织肿瘤,二者有着相似的临床表现,均以症状性脾脏肿大和淋巴细胞增多为特征。通过严谨评估其形态学特点,免疫表型和分子遗传学特征,可将二者加以区分,并可与其他T或B细胞白血病相鉴别。尽管部分患者可能会有不同时长的惰性期,但典型的临床表现仍是侵袭性过程。T细胞幼淋巴细胞白血病(T-PLL)的一线治疗为静脉注射阿仑单抗,而B细胞幼淋巴细胞白血病(B-PLL)的一线治疗为以嘌呤类似物为基础的化学免疫治疗。新型B细胞受体抑制剂,如依鲁替尼和艾代拉里斯,可能将在B-PLL的治疗中占有一席之地,尤其对于存在P53缺失的患者可能有效。异基因干细胞移植对符合移植条件的患者仍可考虑,并且可能是此疾病目前唯一的治愈方法。在过去几年中,许多疾病发病和进展背后的分子机制逐渐被揭示,为新型靶向治疗方法的发展提供了机遇。
B cells and T-cell lymphoblastic leukemia are a rare and poor-prognosis lymphoid tumor with similar clinical manifestations characterized by symptomatic splenomegaly and lymphocytosis. By rigorous assessment of its morphological characteristics, immunophenotyping and molecular genetic features, the two can be distinguished and differentiated from other T or B cell leukemias. Although some patients may have different duration of inertia, but the typical clinical manifestations are still invasive process. First-line treatment of T-cell lymphoblastic leukemia (T-PLL) is intravenous alemtuzumab, while first-line treatment of B-cell lymphoblastic leukemia (B-PLL) is a purine analog-based chemical immunotherapy. Novel B cell receptor inhibitors, such as Ibrutinib and Adelaide, may have a place in the treatment of B-PLL, especially in patients with P53 deletions. Allogeneic stem cell transplantation is still considered for patients eligible for transplantation and may be the only cure for the disease so far. The molecular mechanisms behind the onset and progression of many diseases have gradually been revealed over the past few years, providing opportunities for the development of new targeted therapies.