Soma Lorinda, Cornfield Dennis B, Prager David, Nowell Peter, Bagg Adam
Department of Pathology and Laboratory Medicine, University of Pennsylvania Medical Center, 3400 Spruce Street, Philadelphia, PA 19104, USA.
Am J Hematol. 2002 Nov;71(3):224-6. doi: 10.1002/ajh.10221.
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.
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。