Anaplastic large cell lymphoma with TP63 rearrangement: A dismal prognosis.
Anaplastic large cell lymphoma with TP63 rearrangement: A dismal prognosis.
复制标题
伴有 TP63 重排的间变性大细胞淋巴瘤：预后不佳。
作者：
Yamashita T;Higashi M;Kawano R;Momose S;Tokuhira M;Kizaki M;Tamaru JI
伴有TP 63重排的间变性大细胞淋巴瘤（ALCL）是一种新的恶性肿瘤，在所有类型的ALCL中预后最差。这可能是由于所得融合蛋白具有高致癌能力的N末端截短的p63。由于该N末端结构域具有肿瘤抑制活性的功能，因此高致癌能力的机制被认为是显性负功能。在这里，我们报告了两例伴有TP 63重排的ALCL病例，尽管进行了强化治疗，但每个病例的预后都太短（病例1和2：4个月和6个月）。免疫组化，p63高表达，并检测到sprit信号，使用aTP 63分离荧光原位杂交（FISH）在每种情况下。此外，ALCL的一种不良预后标志物，所有细胞毒性分子（TIA-1、颗粒酶B和穿孔蛋白）也在几乎所有ALCL细胞中表达。综上所述，我们认为，不仅是N-截短型p63的显性负性功能，而且细胞毒性分子的作用可能会影响TP 63重排的ALCL的不良预后。
Anaplastic large cell lymphoma (ALCL) withTP63rearrangement is a new entity and has the most dismal prognosis in all types of ALCL. This might be due to the resulting fusion protein having N‐terminal truncated p63 with high oncogenic ability. Since this N‐terminal domain has the function of tumor suppressor activity, the mechanism for high oncogenic capacity is thought to be the dominant negative function. Here, we report two ALCL cases withTP63rearrangement that was each given too short a prognosis (Case 1 and 2: four and six months) in spite of intensive treatment. Immunohistochemically, p63 was highly expressed, and a sprit signal was detected using aTP63break apart fluorescence in situ hybridization (FISH) in each case. Additionally, a poor prognostic marker of ALCL, all cytotoxic molecules (TIA‐1, Granzyme B, and Perforin) were also expressed in almost all ALCL cells. Taken together, we suggest that not only the dominant negative function of N‐truncated p63 but also the effect of cytotoxic molecules may influence the dismal prognosis of ALCL withTP63rearrangement.