Kar Rakhee, Kumar Rajat, Tyagi Seema
Department of Haematology, AIIMS 110029 New Delhi, India, Phone: +91-11-26594670 E-mail:
Turk J Haematol. 2008 Sep 5;25(3):149-51.
B-cell prolymphocytic leukemia (B-PLL) comprises 1% of chronic lymphocytic leukemias. CD5 positivity is seen in 1/3rd of cases which generally arise from pre existing CLL. They have longer median survival compared with de novo B-PLL which are commonly CD5 negative and are more aggressive with an older age of presentation. Herewith, we describe a 48-year-old male of de-novo CD5+ B-PLL presenting with minimal lymphadenopathy and massive splenomegaly with 90% atypical lymphoid cells in the peripheral smear and bone marrow. Immunophenotyping was strongly positive for CD5, CD45, CD19, CD22, FMC-7, S-Ig and CD38, moderately positive for CD 11c, weakly positive for CD23 and negative for CD-103 and ZAP 70. The patient responded well to fludarabine and cyclophosphamide and had an uneventful hospital course. Our case illustrates a de-novo B-PLL with aberrant CD5 positivity who had a short duration of illness, younger age at presentation and favourable treatment outcome.
B 细胞幼淋巴细胞白血病（B-PLL）占慢性淋巴细胞白血病的 1%。三分之一的病例可见 CD5 阳性，这些病例通常由先前存在的慢性淋巴细胞白血病（CLL）发展而来。与通常 CD5 阴性且发病年龄较大、侵袭性更强的原发性 B-PLL 相比，它们的中位生存期更长。在此，我们描述一名 48 岁的原发性 CD5 + B-PLL 男性患者，表现为轻度淋巴结病和巨大脾肿大，外周血涂片和骨髓中有 90%的非典型淋巴细胞。免疫表型分析显示 CD5、CD45、CD19、CD22、FMC-7、S-Ig 和 CD38 呈强阳性，CD11c 呈中度阳性，CD23 呈弱阳性，CD-103 和 ZAP 70 呈阴性。该患者对氟达拉滨和环磷酰胺反应良好，住院过程顺利。我们的病例说明了一例原发性 B-PLL，其 CD5 异常阳性，病程短，发病年龄较轻，治疗效果良好。
