Fava S, De Paoli A, Grimi E, Luoni M, Tocci A, Tosi A, Cassi E
Day-Hospital di Ematologia Oncologica e Oncologia Medica, Medicina 2a, Ospedale, Legnano.
Recenti Prog Med. 1994 Oct;85(10):496-501.
Prolymphocytic leukemia (PLL) is a malignant lymphoproliferative disorder, characterized by massive splenomegaly, predominance of prolymphocytes in the peripheral blood and bone marrow, minimal lymph nodes enlargement and poor prognosis. It accounts for a 5-10% case of chronic lymphocytic leukemia (CLL). Patients age is usually over the fifth decade, the disease is 4.1 more common in males. More than 80% are B-lymphocytic derived cells showing a post-thymic phenotype. Median survival of B-PLL patients is 3 years, while only 7 months in T-PLL. Standard therapy of CLL with alkylating agents and prednisone have been not much effective in the treatment of PLL with a response rate of about 20%. Up to date no ideal treatment is available for PLL. A realistic goal is probably to achieve a clinical course transformation, from aggressive to mild, thus changing from short to long term prognosis. For this purpose the initial therapeutic approach cannot be limited to a single agent only. Splenic irradiation, intensive anthracyclines-based regimens, leukapheresis combine together represent the best therapeutic choice. Alkylating agents with or without prednisone may play a role in keeping indolent clinical course. Fludarabine has shown antileukemic activity against PPL even in patients resistant.
幼淋巴细胞白血病（PLL）是一种恶性淋巴细胞增殖性疾病，其特征为脾肿大明显、外周血和骨髓中幼淋巴细胞占优势、淋巴结肿大不明显且预后较差。它占慢性淋巴细胞白血病（CLL）病例的5%-10%。患者年龄通常在50岁以上，该疾病在男性中更为常见，是女性的4.1倍。超过80%的病例源自B淋巴细胞，呈现胸腺后表型。B-PLL患者的中位生存期为3年，而T-PLL患者仅为7个月。CLL的标准治疗方案，如烷化剂和泼尼松，对PLL的治疗效果不佳，缓解率约为20%。目前尚无针对PLL的理想治疗方法。一个现实的目标可能是实现临床病程从侵袭性向轻度的转变，从而改善预后，从短期变为长期。为此，初始治疗方法不能仅限于单一药物。脾区放疗、基于蒽环类药物的强化方案以及白细胞单采术联合应用是最佳治疗选择。烷化剂加或不加泼尼松可能有助于维持疾病的惰性病程。氟达拉滨即使在耐药患者中也显示出对PLL的抗白血病活性。
