Malpica Luis, Castro Denisse, Enriquez Daniel J, Oviedo-Pecho Roberto, Peña Camila, Idrobo Henry, Fiad Lorena, Prates Maria, Valcarcel Bryan, Paredes Antonio, Sánchez Gadwyn, Moisés Celia, Castillo Jorge J, Villela Luis, Ramos Juan C, Biglione Mirna, Beltran Brady E
Department of Lymphoma and Myeloma, Division of Cancer Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Departamento de Oncología y Radioterapia, Hospital Nacional Edgardo Rebagliati Martins, Lima, Peru.
Leuk Lymphoma. 2022 Feb;63(2):315-325. doi: 10.1080/10428194.2021.1984455. Epub 2021 Sep 29.
Adult T-cell leukemia/lymphoma (ATLL) is a largely incurable disease. Cutaneous involvement is common and could be first symptom of the disease. We analyzed 169 patients with ATLL of whom 63 had cutaneous involvement. Cutaneous involvement was found in 48, 27, 17, and 60% of acute, lymphomatous, chronic and smoldering ATLL cases, respectively. Eight cases had primary cutaneous tumoral variant. Erythroderma (24%) and plaques (22%) were the most frequent skin lesions. The presence of cutaneous involvement was associated with better overall survival compared to non-cutaneous involvement (aHR 0.55 [95% CI: 0.37-0.82], < 0.01; 1-year OS 53 vs. 27%, respectively, = 0.012). Combination zidovudine and interferon-alpha (AZT-IFN) yielded high response rates (overall response, OR = 100%, = 8; complete response 62.5%) compared to chemotherapy (OR = 33.3%, = 12/36). In conclusion, cutaneous involvement was associated with better survival in Latin American patients with ATLL. AZT-IFN demonstrated encouraging responses in ATLL patients with cutaneous involvement.
成人T细胞白血病/淋巴瘤（ATLL）是一种基本上无法治愈的疾病。皮肤受累很常见，可能是该疾病的首发症状。我们分析了169例ATLL患者，其中63例有皮肤受累。在急性、淋巴瘤性、慢性和冒烟型ATLL病例中，皮肤受累的比例分别为48%、27%、17%和60%。8例为原发性皮肤肿瘤变异型。红皮病（24%）和斑块（22%）是最常见的皮肤病变。与无皮肤受累相比，皮肤受累患者的总生存期更好（调整后风险比0.55 [95%置信区间：0.37 - 0.82]，P < 0.01；1年总生存率分别为53%和27%，P = 0.012）。与化疗相比（总缓解率 = 33.3%，n = 12/36），齐多夫定与α干扰素联合治疗（AZT - IFN）产生了较高的缓解率（总缓解率，OR = 100%，n = 8；完全缓解率62.5%）。总之，在拉丁美洲ATLL患者中，皮肤受累与更好的生存率相关。AZT - IFN在有皮肤受累的ATLL患者中显示出令人鼓舞的反应。
