A multicenter retrospective clinical study of CD5/CD10-negative chronic B cell leukemias
A multicenter retrospective clinical study of CD5/CD10-negative chronic B cell leukemias
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DOI：
10.1002/ajh.21065
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发表时间：
2008-05-01
影响因子：
12.8
通讯作者：
Baldini, Luca
中科院分区：
文献类型：
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作者：
Goldaniga, Maria;Ferrario, Andrea;Baldini, Luca
白血病期CD 5阴性慢性B细胞淋巴增生性疾病（B-CLPD）是异质性的且相对罕见的病理，由于缺乏可接近的病理组织，其通常缺乏组织病理学定义。我们描述了156例CD 5/CD 10阴性B-CLPD患者（中位年龄66岁，范围25-86）的临床特征和进展相关变量。中位随访时间为51个月（范围6-216），总3年和5年生存率分别为87%和76%; 50例患者在诊断时需要治疗，56例在随访期间需要治疗，50例在末次控制前未接受治疗。结合临床、组织学、细胞形态学、免疫表型和细胞遗传学诊断方法，仅允许少数患者被完全分类为脾边缘区或淋巴浆细胞性淋巴瘤;大多数病例仍无法分类。多因素分析显示，与总生存率呈负相关的临床血液学变量是血清LDH水平和年龄，而高血清LDH水平、血红蛋白水平、
CD5-negative chronic B cell lymphoproliferative disorders in leukemic phase (B-CLPD) are heterogeneous and relatively uncommon pathologies that often lack a histopathological definition because of the absence of accessible pathological tissue. We describe the clinical features and evolution-related variables of 156 patients with CD5/CD10-negative B-CLPD (median age 66 years, range 25-86). The median follow-up was 51 months (range 6-216), and overall 3- and 5-year survival was respectively 87 and 76%; 50 patients needed therapy at diagnosis, 56 during follow-up, and 50 remained untreated until the last control. A combined clinical, histological, cytomorphological, immunophenotypical, and cytogenetic diagnostic approach allowed the complete classification of only a minority of patients as being affected by splenic marginal zone or lymphoplasmacytic lymphoma; the majority of cases remained unclassifiable. Multivariate analysis showed that the clinicohematological variables adversely related to overall survival were serum LDH levels and age, whereas high serum LDH levels, hemoglobin levels of