播散型Paget样网状细胞增生病国内首报

A case report of disseminated pagetoid reticulosis

报告1例播散型Paget样网状细胞增生病。患者男,68岁。全身泛发浸润性斑块、脱屑30年,多发溃疡半年,伴发热。皮损组织病理检查示,表皮、真皮有异形淋巴样细胞浸润,类似Paget细胞。不典型淋巴样细胞免疫组化染色示CD45RO和CD3阳性。基因重排T细胞受体(TCR)-γ阳性。诊断:播散型Paget样网状细胞增生病。给予患者化疗,皮损迅速消退,但出现胸腔积液和腹水,腹腔淋巴结增大,最后死于多脏器衰竭。

Objective： To report a case of disseminated pagetoid reticulosis （PR）. Methods： Histologic examination, immunohistochemistry analysis and T-cell recepor（TCR） rearrangement study of the lesions were performed respectively. Results： The patient presented with extensive and infiltrating plaques, desquamation, multiple ulcers and verrncous hyperplasia, accompanied by fever. The lesions disappeared soon after chemotherapy. Hydrothorax and hydroperitoneum occurred subsequently with celiac lymphadenopathy, which led to fatal outcome at last. Histopathological examination revealed infiltrations of atypical lymphocytes in the epidermis and dermis, which were pagetoid cells with CD45RO and CD3 positive staining. TCR rearrangement studies showed positive results of TCR-γ. Conclusions： The lesions of disseminated pagetoid reticulosis can resolve spontaneously. The disease frequently invades lymph nodes and internal organs with a poor prognosis, and the longterm therapeutic effects ought to be further observed.