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伴有复杂核型的异常惰性T细胞幼淋巴细胞白血病:这是T细胞慢性淋巴细胞白血病吗?

Unusually indolent T-cell prolymphocytic leukemia associated with a complex karyotype: is this T-cell chronic lymphocytic leukemia?

作者信息

Soma Lorinda, Cornfield Dennis B, Prager David, Nowell Peter, Bagg Adam

机构信息

Department of Pathology and Laboratory Medicine, University of Pennsylvania Medical Center, 3400 Spruce Street, Philadelphia, PA 19104, USA.

出版信息

Am J Hematol. 2002 Nov;71(3):224-6. doi: 10.1002/ajh.10221.

Abstract

T-cell prolymphocytic leukemia (T-PLL) is typically associated with an aggressive clinical course, with a median survival of less than 1 year. We report a case of T-PLL that displays multiple cytogenetic abnormalities, with the most complex subclone having the following karyotype: 47, Y, -X, +8, inv (10) (p12q26), del (11) (p13p15) +marker. However, despite this genetic complexity, the leukemia has behaved in a remarkably indolent manner, with the patient remaining asymptomatic, without therapeutic intervention, for more than 7 years. The unusually benign behavior of this disease calls into question the validity of grouping such cases under the umbrella of T-PLL and warrants a reconsideration of T-cell chronic lymphocytic leukemia (no longer recognized as a distinct disease) as a bona fide diagnostic entity.

摘要

T 细胞幼淋巴细胞白血病(T-PLL)通常与侵袭性临床病程相关,中位生存期不到 1 年。我们报告 1 例 T-PLL 病例,该病例显示出多种细胞遗传学异常,最复杂的亚克隆具有以下核型:47,Y,-X,+8,inv(10)(p12q26),del(11)(p13p15)+标记物。然而,尽管存在这种遗传复杂性,该白血病的行为却极为惰性,患者在未接受治疗干预的情况下无症状存活了 7 年多。这种疾病异常良性的行为使人质疑将此类病例归为 T-PLL 的有效性,并值得重新考虑将 T 细胞慢性淋巴细胞白血病(不再被视为一种独特疾病)作为一个真正的诊断实体。

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