Mateos-Mayo Ana, Sánchez-Herrero Alejandro, Nieto-Benito Lula María, Parra-Blanco Verónica, Suárez-Fernández Ricardo, Pulido-Pérez Ana
Universidad Complutense de Madrid, Hospital General Universitario Gregorio Marañón, Servicio de Dermatología.
Universidad Complutense de Madrid, Hospital General Universitario Gregorio Marañón, Servicio de Anatomía Patológica. Madrid, España.
Rev Med Inst Mex Seguro Soc. 2020 Sep 1;58(5):628-633. doi: 10.24875/RMIMSS.M20000093.
T-cell prolymphocytic leukemia (T-PLL) is a T-cell lymphoproliferative disorder that frequently involves the skin. The objective was to describe two cases of T-PLL with cutaneous involvement and to present a review of the literature concerning the clinical characteristics, differential diagnosis and treatment of these patients.
T-cell prolymphocytic leukemia accounts for 2% of mature leukemias in adults. Skin involvement is reported in 20-50% of the patients. The characteristic features are facial involvement, purpuric lesions and symmetry of the rash, although there are atypical manifestations as well. Differential diagnosis includes other T-cell lymphoproliferative disorders with hematologic and skin involvement, such as Sézary syndrome. Patients with T-PLL may show cutaneous infiltration at the moment of debut or relapse of the disease. The skin is an accessible organ for taking samples to study and diagnose these patients.
T 细胞原淋巴细胞白血病(T-PLL)是一种常累及皮肤的 T 细胞淋巴增殖性疾病。目的是描述两例伴有皮肤受累的 T-PLL 病例,并对有关这些患者的临床特征、鉴别诊断和治疗的文献进行综述。
1)一名 79 岁男性,此前基于实验室偶然发现被诊断为 T-PLL。他曾接受阿仑单抗治疗,但因反复感染不得不中断治疗。中断治疗后,患者四肢出现对称性皮疹。皮肤活检显示有 T-PLL 浸润。2)一名 28 岁男性,表现为乏力和淋巴细胞增多。他还在躯干出现紫癜性皮疹和面部红斑。皮肤和骨髓的组织病理学证实诊断为伴有皮肤受累的 T-PLL。
T 细胞原淋巴细胞白血病占成人成熟白血病的 2%。据报道,20%至 50%的患者有皮肤受累。其特征性表现为面部受累、紫癜性损害和皮疹对称性,不过也有非典型表现。鉴别诊断包括其他伴有血液学和皮肤受累的 T 细胞淋巴增殖性疾病,如 Sézary 综合征。T-PLL 患者在疾病初发或复发时可能出现皮肤浸润。皮肤是获取样本用于研究和诊断这些患者的一个可及器官。