免疫母细胞性淋巴结病样T细胞淋巴瘤伴皮肤损害1例

免疫母细胞性淋巴结病样Ｔ细胞淋巴瘤（ＩＢＬ－ＴＣ）是特殊类型的周围Ｔ细胞淋巴瘤。临床特征为发热;淋巴结、肝脾肿大;皮疹及高γ球蛋白血症。皮疹呈多种形态,随其发展主要为二型：丘疹结节型和红皮病型。淋巴结病理示淋巴结结构破坏,有异形淋巴样细胞、免疫母细胞和浆细胞样的所谓淡染细胞（ｐａｌｅｃｅｌｌｓ）浸润。免疫学示Ｔ细胞标记。

Immunoblastic lymphadenopathy-like T-cell lymphoma with cutaneous lesions:a case report

Immunoblastic lymphadenopathy(IBL)-like T-cell lymphoma is a distinct peripheral T-cell lymphoma.The disease is clinically characterized by generalized enlargement of lymph nodes hepatosplenomegaly,fever,skin rash and polyclonal hypergammaglobulinemia.The skin lesions are diffrent pictures and progress to two main forms predominately:papulonodular and erythroderma.Histologically,the involved lymph nedes show an obliteration of it's normal lymph node architecture by a polymorphic cellular infiltrate including atypical lymphoid cells,immunobthets,plasma cells and plasmacytoid celis,so-called "pale cells",together with a proliferation of arborzing small blood vessels.Immunophenotypic studies disclosed that most of the atypical lymphoid cells have T-cell makers.