Absi Ahmed, Hsi Eric, Kalaycio Matt
Taussig Cancer Cancer, The Cleveland Clinic Foundation, 9500 Euclid Avenue R35, Cleveland OH 44195, USA.
Curr Treat Options Oncol. 2005 May;6(3):197-208. doi: 10.1007/s11864-005-0003-4.
Prolymphocytic leukemia is a rare chronic lymphoproliferative disorder that includes two subtypes, B cell and T cell, each with its own distinct clinical, laboratory and pathological features. T-cell prolymphocytic leukemia has an aggressive course with short median survival and poor response to chemotherapy. With the use of the purine analogue pentostatin more than half of patients will have a major response and a minority will have a complete remission, usually lasting months. With the introduction of alemtuzumab, most patients who progressed despite treatment with pentostatin had a major response with a complete remission rate higher than that obtained with pentostatin when used as a first line. Unfortunately, progression still follows shortly. We recommend alemtuzumab as initial therapy and offer stem cell transplant (SCT) to selected young, healthy patients who respond. Although B-cell prolymphocytic leukemia is also a progressive disease, some patients can achieve a prolonged progression-free-survival with fludarabine. Patients presenting with massive splenomegaly may be effectively palliated with splenic irradiation or splenectomy. Rituximab is a promising agent and further investigations are warranted to better define its role in treatment of this disorder.
原淋巴细胞白血病是一种罕见的慢性淋巴细胞增殖性疾病，包括B细胞和T细胞两个亚型，各有其独特的临床、实验室和病理特征。T细胞原淋巴细胞白血病病程侵袭性强，中位生存期短，对化疗反应不佳。使用嘌呤类似物喷司他丁后，超过半数患者会有显著反应，少数患者会完全缓解，通常持续数月。随着阿仑单抗的引入，大多数尽管接受喷司他丁治疗仍进展的患者有显著反应，作为一线治疗时完全缓解率高于使用喷司他丁时。不幸的是，很快仍会进展。我们推荐阿仑单抗作为初始治疗，并为有反应的选定年轻健康患者提供干细胞移植（SCT）。虽然B细胞原淋巴细胞白血病也是一种进行性疾病，但一些患者使用氟达拉滨可实现较长时间的无进展生存期。出现巨脾的患者可通过脾照射或脾切除术有效缓解症状。利妥昔单抗是一种有前景的药物，有必要进一步研究以更好地明确其在该疾病治疗中的作用。
