ALK阴性系统型间变性大细胞淋巴瘤1例

A Case of ALK-Negative Systemic Anaplastic Large Cell Lymphoma

报道1例临床罕见的ALK阴性系统型间变性大细胞淋巴瘤。患者男,79岁,因“全身皮肤丘疹、结节1月”,于2023年2月就诊于我科。皮肤科情况:头顶部、胸腹部、背部、四肢可见大量红色丘疹、结节,大小不等,部分丘疹可相互融合,最大直径4 cm,质地坚实,表面光滑,境界清楚,个别结节顶部破溃、结痂,基底红润。组织病理:(腹部皮损)真皮内可见大量多形肿瘤细胞与淋巴细胞增生,肿瘤细胞体积大,核大深染,异型性明显,可见病理性核分裂像及吞噬现象,未见亲表皮现象。(左腹股沟淋巴结)原有淋巴结结构破坏,肿瘤细胞大片状浸润,肿瘤细胞较腹部皮损处更大,胞质丰富,核大偏位,可见肾形、马蹄形,病理性核分裂及吞噬现象更多及明显,肿瘤细胞间夹杂散在少量淋巴细胞、嗜酸性粒细胞。免疫组化:CD30(>95%+),CD2、CD3、CD4阳性,CD5(部分+),CD8(少数+),ALK、CD20、EMA、TIA-1、Gr-B、CD56、CD7、TdT阴性,Ki67(约90%+)。原位杂交:EBER(-)。诊断:ALK阴性系统型间变性大细胞淋巴瘤Ⅳ期A组。治疗:予CHOP方案分段治疗。

We report a rare clinical case of ALK-negative systemic mesenchymal large cell lymphoma.The patient,a 79-year-old male,presented in February 2023 with generalized skin papules and nodules for 1 month".A large number of red papules and nodules were seen on the top of the head,chest and abdomen,back,and extremities,varying in size,some of which could fuse with each other,with a maximum diameter of 4 cm,firm texture,smooth surface,and clear boundaries,with individual nodules breaking down and crusting on top,and a ruddy base.Histopathological biopsy:(abdominal lesion)A large number of pleomorphic tumor cells and lymphocytic proliferation were seen in the dermis,with large tumor cells,large deep-stained nuclei,obvious heterogeneity,pathological nuclear division and phagocytosis,and no pro-epidermal phenomenon was seen.(Left inguinal lymph node)The original lymph node structure was destroyed,and the tumor cells were infiltrated in large patches.The tumor cells were larger than those in the abdominal lesion,with abundant cytoplasm,large eccentric nuclei,kidney-shaped and horseshoe-shaped,and more and more pathological nuclear division and phagocytosis were evident.Immunohistochemistry suggested:CD30(>95%+),CD2,CD3,CD4 positive,CD5(partial+),CD8(few+),ALK,CD20,EMA,TIA-1,Gr-B,CD56,CD7,TdT negative,Ki67(about 90%+),in situ hybridization:EBER(-).Diagnosis:ALK-negative systemic mesenchymal large cell lymphoma stage IV group A.Treatment:treated with CHOP regimen in segments.