Somasundaram Venkatesan, Purohit Abhishek, Aggarwal Mukul, Manivannan Prabhu, Mishra Pravas, Seth Tulika, Tyagi Seema, Mahapatra Manoranjan, Pati Hara P, Saxena Renu
Department of Hematology, All India Institute of Medical Sciences, New Delhi, India.
Indian J Med Paediatr Oncol. 2014 Oct;35(4):271-5. doi: 10.4103/0971-5851.144988.
Hairy cell leukemia is a rare chronic B-cell disorder that follows an indolent but progressive course. This disorder is characterized by pancytopenia, splenomegaly, bone marrow fibrosis and the presence of atypical lymphoid cells with hairy projections in peripheral blood, bone marrow and spleen. Treatment is mainly with nucleoside analog cladribine, which induces complete remission in up to 85% cases.
This is a retrospective analysis of Hairy cell Leukemia cases diagnosed and treated in the Department of Hematology, All India Institute of Medical Sciences, New Delhi between 2002 and 2013. Various parameters such as clinical features, laboratory parameters including complete blood cell count, bone marrow findings, cytochemistry, immunophenotyping by flowcytometry or immunohistochemistry, treatment protocol and complications secondary to treatment and relapse were reviewed.
A total of 35 cases were diagnosed during this period of 12 years of which 27 received cladribine and went in to remission. Median follow-up duration was 26 months. 5 (18%) cases had a relapse and all relapsed cases achieved second remission with cladribine; however, there was no case of second malignancy in our cohort.
Cladribine has emerged as the treatment of choice for hairy cell leukemia given that the overwhelming majority of patients achieve long-lasting complete remissions. Upon relapse, these patients could be successfully salvaged with cladribine retreatment.
毛细胞白血病是一种罕见的慢性B细胞疾病，病程进展缓慢但呈进行性。该疾病的特征为全血细胞减少、脾肿大、骨髓纤维化，以及外周血、骨髓和脾脏中存在带有毛状突起的非典型淋巴细胞。治疗主要使用核苷类似物克拉屈滨，高达85%的病例可实现完全缓解。
这是一项对2002年至2013年期间在新德里全印度医学科学研究所血液科诊断和治疗的毛细胞白血病病例的回顾性分析。回顾了各种参数，如临床特征、包括全血细胞计数在内的实验室参数、骨髓检查结果、细胞化学、通过流式细胞术或免疫组织化学进行的免疫表型分析、治疗方案以及治疗继发的并发症和复发情况。
在这12年期间共诊断出35例病例，其中27例接受了克拉屈滨治疗并实现缓解。中位随访时间为26个月。5例（18%）病例复发，所有复发病例再次使用克拉屈滨治疗后均实现二次缓解；然而，我们的队列中没有出现第二原发性恶性肿瘤病例。
鉴于绝大多数患者可实现持久的完全缓解，克拉屈滨已成为毛细胞白血病的首选治疗药物。复发时，这些患者可通过再次使用克拉屈滨成功挽救。
