Alsop Skylar, Sanger Warren G, Elenitoba-Johnson Kojo S J, Lim Megan S
Department of Pathology, Penn State University, Hershey, PA 16802, USA.
Hum Pathol. 2007 Oct;38(10):1576-80. doi: 10.1016/j.humpath.2007.05.018.
The development of Philadelphia chromosome-positive chronic myelogenous leukemia (CML) in the adolescent population is very rare. CML occurring as a secondary malignancy in individuals treated for anaplastic large cell lymphoma (ALCL) is also rare. We present the case of a 16-year-old adolescent boy who developed a right orbital mass that was diagnosed as ALCL with the t(2;5)(p23;q25) chromosomal aberration. Four years after receiving treatment for ALCL, he presented with a swollen leg and a white cell count of 431,000. Peripheral blood and bone marrow evaluation revealed a myeloproliferative disorder. Cytogenetic and molecular studies demonstrated the presence of t(9;22). We present the histopathologic, molecular, and cytogenetic findings of this patient's initial presentation with systemic ALCL as well as his secondary presentation with CML 4 years later. Therapy-related CML and non-therapy-related secondary CML are discussed as potential explanations of this highly unusual clinical presentation.
费城染色体阳性慢性粒细胞白血病（CML）在青少年人群中的发生极为罕见。CML作为间变性大细胞淋巴瘤（ALCL）治疗个体中的继发性恶性肿瘤也很罕见。我们报告了一例16岁青少年男性病例，该患者出现右眼眶肿物，经诊断为伴有t(2;5)(p23;q25)染色体畸变的ALCL。在接受ALCL治疗四年后，他出现腿部肿胀，白细胞计数达431,000。外周血和骨髓评估显示为骨髓增殖性疾病。细胞遗传学和分子研究证实存在t(9;22)。我们展示了该患者最初系统性ALCL表现以及四年后CML继发表现的组织病理学、分子和细胞遗传学结果。讨论了与治疗相关的CML和与治疗无关的继发性CML作为这种高度不寻常临床表现的潜在解释。
