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冷球蛋白血症的成功治疗:一例报告

Successful therapy of crystalcryoglobulinemia: a case report.

作者信息

Cummings F J, Park C H, Bogaars H A, Kalderon A E, Melnicoff I, Kaplan S R, Diamond I, Calabresi P

出版信息

Med Pediatr Oncol. 1979;7(2):181-90. doi: 10.1002/mpo.2950070213.

Abstract

Successful therapy for a case of multiple myeloma with a spontaneously crystallizing cryoglobulin of the IgG2-kappa light chain variety was achieved, using both continuous-flow cell centrifugation plasmapheresis to rapidly lower the M component and combination chemotherapy with phenylalanine mustard, prednisone, procarbazine, and vincristine to control the myeloma process. This resulted in resolution of incapacitating large and small necrotic cutaneous ulcerations of the extremities. Physicochemical studies of the crystalcryoprotein demonstrated that cryoprecipitation was rapid and accompanied by the formation of needle-shaped crystals, yet was completely reversible at 37 degrees C. Cryocrit determinations varied depending upon relative centrifugal forces and temperature and did not always relate linearly to the amount of abnormal protein, thus making these alone unreliable in assessing response to therapy.

摘要

采用连续流式细胞离心血浆置换术迅速降低M成分,并联合使用苯丙氨酸氮芥、泼尼松、丙卡巴肼和长春新碱进行化疗以控制骨髓瘤进程,成功治愈了一例患有IgG2-κ轻链型自发结晶冷球蛋白的多发性骨髓瘤患者。这使得四肢严重的大小不等坏死性皮肤溃疡得以痊愈。对冷沉淀蛋白的物理化学研究表明,冷沉淀迅速,伴有针状晶体形成,但在37℃时完全可逆。冷比容测定结果因相对离心力和温度而异,并不总是与异常蛋白量呈线性关系,因此仅靠这些在评估治疗反应时并不可靠。

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