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原发性皮肤浆细胞瘤——两例报告并文献复习

Primary cutaneous plasmacytoma--report of two cases and review of the literature.

作者信息

Wong K F, Chan J K, Li L P, Yau T K, Lee A W

机构信息

Department of Pathology, Queen Elizabeth Hospital, Hong Kong.

出版信息

Am J Dermatopathol. 1994 Aug;16(4):392-7. doi: 10.1097/00000372-199408000-00006.

Abstract

Primary cutaneous plasmacytomas are very rare. In this report, we describe two such cases and review the literature on this entity. Both patients presented with a slowly growing, painless, and solitary mass on the chest wall. Histologically, one case was composed of mature-looking plasma cells, while the other was composed of immature and anaplastic plasma cells, infiltrating the dermis. The epidermis was spared. Kappa light-chain restriction was demonstrated by immunohistochemical techniques in both cases. There was no evidence of marrow disease even on repeated marrow biopsies, although extracutaneous lesions were detected in one patient. One remained in complete remission 6 years following local irradiation. The other patient was treated with local irradiation and systemic chemotherapy, with a complete response, but this was followed by multiple cutaneous recurrences and further remissions by treatment with cytotoxic agents. The present cases and those reported in the literature illustrate well the variable clinical course of primary cutaneous plasmacytoma. Although cure can apparently be achieved in some patients by local radiation therapy, more than half of the cases relapse or progress to myelomatosis. The disease-related mortality is at least 40%. Thus cutaneous plasmacytoma appears to be more aggressive than noncutaneous extramedullary plasmacytomas and should be separately categorized from them in future studies.

摘要

原发性皮肤浆细胞瘤非常罕见。在本报告中,我们描述了两例此类病例,并回顾了关于该实体的文献。两名患者均表现为胸壁上缓慢生长、无痛的孤立性肿块。组织学上,一例由外观成熟的浆细胞组成,另一例由不成熟和间变性浆细胞组成,浸润真皮。表皮未受累。两例均通过免疫组化技术证实有κ轻链限制。尽管在一名患者中检测到皮肤外病变,但即使重复进行骨髓活检也没有骨髓疾病的证据。一名患者在局部放疗后6年保持完全缓解。另一名患者接受了局部放疗和全身化疗,获得完全缓解,但随后出现多次皮肤复发,并通过细胞毒性药物治疗进一步缓解。目前的病例以及文献中报道的病例很好地说明了原发性皮肤浆细胞瘤的临床病程多变。虽然在一些患者中通过局部放射治疗显然可以治愈,但超过一半的病例会复发或进展为骨髓瘤。与疾病相关的死亡率至少为40%。因此,皮肤浆细胞瘤似乎比非皮肤性髓外浆细胞瘤更具侵袭性,在未来的研究中应与它们分开分类。

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