皮肤恶性T细胞性淋巴瘤伴反应性噬血组织细胞增生症的临床病理研究

报道6例皮肤T细胞性恶性淋巴瘤伴噬血组织细胞增生症.病变局限于真皮和皮下脂肪组织,灶性的肿瘤性T淋巴细胞浸润和分化良好的噬血组织细胞增生伴各种炎症细胞浸润.4例表现为病情进行性恶化、复发或死亡.并探讨其发病机理、诊断标准及鉴别诊断.

T-cell Lymphoma Associated with Reactive Histiocytic Hyperplasia with Hemophagocytosis

Six cases of T-cell lyrnphoma associated with reactive histiocytic hyperplasia with hemophagocytosis (HHH) localized primarily to the skin and subcutaneous fat were described.Initial biopsy findings may he misinterpreted as benign panniculitis.Four of which were progressive,recurrent,even fatal (follow-up for 8-36 months).Histologically the lesions of all cases were composed of a mixture of small and medium sized atypical lymphoid cells and benign hemophagocytes infiltrated with neutrophils and eosinophils between fat cells.The mechanism and differential diagnosis of the disease were discussed.