Bittencourt Achiléa L, da Graças Vieira Maria, Brites Carlos R, Farre Lourdes, Barbosa Helenemarie S
Department of Pathology, Hospital Universitário Prof Edgard Santos, Federal University of Bahia, Brazil.
Am J Clin Pathol. 2007 Nov;128(5):875-82. doi: 10.1309/2YGD1P0QCVCWBLDX.
The purpose of this study was to evaluate whether subdivision of adult T-cell leukemia/lymphoma (ATL) on the basis of clinical types, skin involvement, histologic features, cell size, and proliferative index (PI) was clinically relevant. Skin lesions were present in 47 cases (67%). Five cases were classified as primary cutaneous tumoral (PCT) type not included in the Shimoyama classification and characterized by skin tumors and absence of systemic involvement, lymphocytosis, and hypercalcemia. Mortality was high (61/70 [87%]). The overall median survival time (MST) was 12 months. The following variables were adversely related to survival: acute, lymphoma, and PCT types; absence of skin lesions; large cells; and PI more than 18%. The longer MST observed in cases with skin lesions was probably due to prolonged survival of the smoldering type (58 months). The MST of the PCT type (21 months) was shorter than that of the smoldering type, confirming the importance of clearly defining these 2 types of ATL.
本研究的目的是评估根据临床类型、皮肤受累情况、组织学特征、细胞大小和增殖指数（PI）对成人T细胞白血病/淋巴瘤（ATL）进行细分是否具有临床相关性。47例（67%）存在皮肤病变。5例被分类为原发性皮肤肿瘤（PCT）型，该型未包括在岛山分类中，其特征为皮肤肿瘤且无全身受累、淋巴细胞增多和高钙血症。死亡率很高（61/70 [87%]）。总体中位生存时间（MST）为12个月。以下变量与生存呈负相关：急性、淋巴瘤和PCT型；无皮肤病变；大细胞；以及PI超过18%。有皮肤病变的病例中观察到的较长MST可能是由于冒烟型的生存时间延长（58个月）。PCT型的MST（21个月）短于冒烟型，证实了明确区分这两种ATL类型的重要性。
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