Stein H, Foss H D, Dürkop H, Marafioti T, Delsol G, Pulford K, Pileri S, Falini B
Institute of Pathology, University Hospital Benjamin Franklin, Free University, Berlin, Germany.
Blood. 2000 Dec 1;96(12):3681-95.
Anaplastic large cell lymphoma (ALCL) represents a generally recognized group of large cell lymphomas. Defining features consist of a proliferation of predominantly large lymphoid cells with strong expression of the cytokine receptor CD30 and a characteristic growth pattern. With the use of molecular and clinical criteria, 3 entities of ALCL have been identified: primary systemic anaplastic lymphoma kinase (ALK)(+) ALCL, primary systemic ALK(-) ALCL, and primary cutaneous ALCL. ALK expression is caused by chromosomal translocations, most commonly t(2;5). ALK(+) ALCL predominantly affects young male patients and, if treated with chemotherapy, has a favorable prognosis. It shows a broad morphologic spectrum, with the "common type," the small cell variant, and the lymphohistiocytic variant being most commonly observed. The knowledge of the existence of these variants is essential in establishing a correct diagnosis. ALK(-) ALCL occurs in older patients, affecting both genders equally and having an unfavorable prognosis. The morphology and the immunophenotype of primary cutaneous ALCL show an overlap with that of lymphomatoid papulosis. Both diseases have an excellent prognosis, and secondary systemic dissemination is only rarely observed. The described ALCL entities usually derive from cytotoxic T cells. In contrast, large B-cell lymphomas with anaplastic morphology are believed to represent not a separate entity but a morphologic variant of diffuse large B-cell lymphoma. Malignant lymphomas with morphologic features of both Hodgkin disease and ALCL have formerly been classified as Hodgkin-like ALCL. Recent immunohistologic studies, however, suggest that ALCLs Hodgkin-like represent either cases of tumor cell-rich classic Hodgkin disease or (less commonly) ALK(+) ALCL or ALK(-) ALCL. (Blood. 2000;96:3681-3695)
间变性大细胞淋巴瘤（ALCL）是一组普遍公认的大细胞淋巴瘤。其定义特征包括以细胞因子受体CD30强表达的主要大淋巴细胞增殖以及特征性生长模式。根据分子和临床标准，已鉴定出3种ALCL实体：原发性系统性间变性淋巴瘤激酶（ALK）（+）ALCL、原发性系统性ALK（-）ALCL和原发性皮肤ALCL。ALK表达由染色体易位引起，最常见的是t(2;5)。ALK（+）ALCL主要影响年轻男性患者，若接受化疗，预后良好。它表现出广泛的形态学谱，最常观察到的是“常见型”、小细胞变异型和淋巴组织细胞变异型。了解这些变异型的存在对于正确诊断至关重要。ALK（-）ALCL发生于老年患者，男女均可受累，预后不良。原发性皮肤ALCL的形态学和免疫表型与淋巴瘤样丘疹病有重叠。这两种疾病预后均良好，仅很少观察到继发性全身播散。所述的ALCL实体通常来源于细胞毒性T细胞。相比之下，具有间变性形态的大B细胞淋巴瘤被认为不是一个单独的实体，而是弥漫性大B细胞淋巴瘤的一种形态学变异型。以前具有霍奇金病和ALCL形态学特征的恶性淋巴瘤被归类为霍奇金样ALCL。然而，最近的免疫组织学研究表明，霍奇金样ALCL要么是肿瘤细胞丰富的经典霍奇金病病例，要么（较少见）是ALK（+）ALCL或ALK（-）ALCL。（《血液》。2000年；96:3681 - 3695）
