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[多发性骨髓瘤终末期多发性皮肤播散性髓外浆细胞瘤]

[Gross spreading multiple extramedullary plasmacytomas to the skin in the terminal stage of multiple myeloma].

作者信息

Kanno M, Nakamura S, Danno D, Uotani C, Kotani T, Yamanaka S, Terasaki Y, Matano S, Okumura H

机构信息

Department of General Medicine and Clinical Investigation, Nara Medical University.

出版信息

Rinsho Ketsueki. 2001 Jul;42(7):554-8.

Abstract

A 71-year-old woman with an 8-year history of IgG-kappa type multiple myeloma was admitted because of severe lumbago and bone destruction. Her serum IgG level was elevated to 5,565 mg/dl at admission. Despite treatment with doxorubicin, vincristine, dexamethasone, melphalan and interferon-alpha, the response was transient. Nine months later, multiple skin nodules appeared on her chest, abdominal wall and right thigh accompanied by elevation of the serum IgG level. Response to combination chemotherapy with cyclophosphamide, ranimustine, vincristine and prednisolone was also transient. The skin tumors on the bilateral thighs, especially on the left side, acquired chemotherapy resistance and gradually enlarged. Although the serum IgG level was maintained by chemotherapy within the range 1, 790-2,676 mg/dl, the skin tumors on the left thigh had spread very rapidly and appeared "rock-like". The enlarged tumors caused necrosis with erosions and oozing hemorrhage. A skin biopsy from the tumors on the left thigh showed plasmacytoma in which infiltration of large anaplastic plasma cells was observed. The patient died of sepsis 8 months after the skin tumors initially developed. This is a very rare case of multiple myeloma in which multiple large plasmacytomas of the skin developed and grew aggressively at the terminal stage after a long-term indolent course.

摘要

一名患有IgG-κ型多发性骨髓瘤8年的71岁女性因严重腰痛和骨质破坏入院。入院时她的血清IgG水平升至5565mg/dl。尽管接受了阿霉素、长春新碱、地塞米松、美法仑和α干扰素治疗,但反应是短暂的。9个月后,她的胸部、腹壁和右大腿出现多个皮肤结节,同时血清IgG水平升高。对环磷酰胺、司莫司汀、长春新碱和泼尼松龙联合化疗的反应也是短暂的。双侧大腿,尤其是左侧的皮肤肿瘤产生了化疗耐药性并逐渐增大。尽管化疗将血清IgG水平维持在1790 - 2676mg/dl范围内,但左大腿的皮肤肿瘤迅速扩散并呈现“岩石样”。增大的肿瘤导致坏死,伴有糜烂和渗血。左大腿肿瘤的皮肤活检显示为浆细胞瘤,其中观察到大量间变性浆细胞浸润。皮肤肿瘤最初出现8个月后,患者死于败血症。这是一例非常罕见的多发性骨髓瘤病例,在长期惰性病程后的终末期出现了多个皮肤大浆细胞瘤并呈侵袭性生长。

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