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IgD型多发性骨髓瘤。三例报告。

IgD multiple myeloma. A report of three cases.

作者信息

Martini R, Santini S, Bottari G

机构信息

Ospedale Misericordia e Dolce UO Medicina ad indirizzo Oncologico, Centro Oncologico Sandro Pitigliani Azienda USL n. 4, Prato.

出版信息

Minerva Med. 1996 Mar;87(3):109-12.

PMID:8668286
Abstract

Three patients suffering from IgD myeloma, which a rare variant of multiple myeloma which often has an aggressive course, were studied retrospectively in order to elucidate the existence of clinical or laboratory features in relationship to survival time. The patients were monitored in follow-up for a time variable for 8 to 52 months. All patients received courses of chemotherapy using an association of Melphalan and Prednisone (MP); one patient also received recombinant interferon alpha in association. Response to chemotherapy, with a > 50% reduction of serum M component, disappearance of Bence Jones proteinuria and permanent control of the disease was achieved in all patients. The median duration of survival in IgD myeloma is shorter than that currently observed in patients with other myeloma types: in our series one patient died 8 months after diagnosis but other two patients are still alive 8 and 52 months after diagnosis, respectively. Great difficulty was encountered in analysis of unfavourable prognostic clinical and laboratory data: in our series, in spite of the small number of cases, the Authors observe that only the relief of increased serum levels of Lactate Dehydrogenase (LDH) seem to be in relationship with a trend of shorter survival. The authors, confirming the particular clinical and laboratory aspects of this myeloma, stress that there may coexist cases in which standard chemotherapy failed to control the diseases: these seem to indicate neoplasia with fast growth kinetics. Further studies are necessary in order to identify new prognostic index which allows the identification of selected groups of patients who can profit from a combination chemotherapy regimen other than the standard MP association.

摘要

对三名患有IgD骨髓瘤(一种多发性骨髓瘤的罕见变体,通常病程侵袭性强)的患者进行了回顾性研究,以阐明与生存时间相关的临床或实验室特征的存在情况。对患者进行了8至52个月的随访监测,时间为可变因素。所有患者均接受了美法仑和泼尼松联合化疗(MP)疗程;一名患者还联合使用了重组干扰素α。所有患者化疗后均有反应,血清M成分降低>50%,本周氏蛋白尿消失,疾病得到长期控制。IgD骨髓瘤的中位生存期短于目前观察到的其他骨髓瘤类型患者:在我们的系列研究中,一名患者在诊断后8个月死亡,但另外两名患者分别在诊断后8个月和52个月仍存活。在分析不良预后的临床和实验室数据时遇到了很大困难:在我们的系列研究中,尽管病例数量较少,但作者观察到只有血清乳酸脱氢酶(LDH)水平升高的缓解似乎与生存期缩短趋势有关。作者在证实这种骨髓瘤特殊的临床和实验室特征时强调,可能存在标准化疗无法控制疾病的病例:这些病例似乎表明肿瘤生长动力学较快。有必要进行进一步研究,以确定新的预后指标,从而识别出能从标准MP联合方案以外的联合化疗方案中获益的特定患者群体。

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