Hairy cell leukemia variant - Fact or fiction
Hairy cell leukemia variant - Fact or fiction
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DOI：
10.1309/8qytyq1clqmhq9cl
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发表时间：
2005-01-01
影响因子：
3.5
通讯作者：
Bahler, DW
中科院分区：
文献类型：
--
作者：
Cessna, MH;Hartung, L;Bahler, DW
毛细胞白血病变异型(HCL-V)是一种少见的B细胞淋巴增生性疾病，通常表达CD103和CD11c，但缺乏CD25。脾边缘带淋巴瘤(SMZL)在15%-25%的病例中也有这种罕见的表型，有其他重叠的临床或形态特征，并且比HCL-V更常见。我们研究的目的是更好地描述HCL-V，并确定大多数病例是否能与SMZL鉴别。从我们的流式细胞仪服务中确定了HCL-V表型的病例，并根据骨髓或脾组织的可用性选择了10例进行进一步研究。所有病例的细胞学特征均符合HCL-V，10例中有9例有淋巴细胞增多症。骨髓受累多为间质和/或窦状，无淋巴结节。转换前重链亚型与转换后重链亚型共表达，4例中有2例出现异常的HCL特征。这项研究更好地定义了HCL-V，并确定大多数病例不代表SMZL。
Hairy cell leukemia variant (HCL-V) is a poorly described, rare B-cell lymphoproliferative disorder typically positive, for CD103 and CD11c, while lacking CD25. Splenic marginal zone lymphomas (SMZL) also have this unusual phenotype in 15% to 25% of cases, have other overlapping clinical or morphologic features, and are more common than HCL-V The purpose of our study was to better characterize HCL-V and determine whether most cases could be distinguished from SMZL. Cases with an HCL-V phenotype were identified from our flow cytometry service, and 10 were selected for further study based on bone marrow or splenic tissue availability. All cases had cytologic features consistent with HCL-V, and 9 of 10 patients had lymphocytosis. Bone marrow involvement was mostly interstitial and/or sinusoidal without lymphoid nodules. Coexpression of preswitched with postswitched heavy chain isotypes, an unusual feature of HCL was seen in 2 of 4 cases. This study better defines HCL-V and establishes that most cases do not represent SMZL.