14例髓外浆细胞瘤患者的临床特征分析及治疗

  目的   探讨伴髓外浆细胞瘤（extramedullary plasmacytoma,EMP）的多发性骨髓瘤（multiple myeloma,MM）患者的临床特点,治疗选择及预后。   方法   对14例确诊的伴髓外浆细胞瘤的多发性骨髓瘤进行回顾性分析。   结果   14例患者男女比为1:l,中位年龄56.9岁。9例患者初诊时即出现EMP,5例在治疗过程中发生。治疗选择包括化疗,手术,放疗或自体移植。6例患者单发EMP,8例多发EMP（multiextramedullary plasmacytomas,MEP）;单发患者中5例部分或接近完全缓解,1例病情进展;多发患者中5例死亡,1例病情进展,2例部分缓解。   结论   MM伴有MEP者对常规化疗反应差,预后不良,需要探讨新的治疗策略。      

髓外浆细胞瘤的诊断和治疗

目的 探讨髓外浆细胞瘤（EMP）的诊断和治疗。方法 4例髓外浆细胞瘤的临床资料。结果 1例疗后失访。2例分别随访74月和56月，无局部复发、远处转移，无进展证据。1例治疗后6个月复发，18个月时死于多发性骨髓瘤（MM）。结论 EMP的诊断确立必须有组织学结果，并经系统检查除外MM；放疗和手术是EMP的主要治疗手段。     

孤立性浆细胞瘤12例

目的 探讨孤立性浆细胞瘤(SP)的临床特点及诊治方法.方法 回顾分析2007年1月至2014年6月收治的12例SP的临床资料.结果 12例患者中6例患者为骨孤立性浆细胞瘤(SBP),6例患者为髓外浆细胞瘤(EMP).12例患者中位年龄为52.5岁(31～ 78岁),男性6例,女性6例;6例EMP中I期1例,Ⅱ期5例;12例患者行手术+化疗+放疗l例,手术+放疗6例,化疗+放疗2例,单纯放疗3例.12例患者近期疗效完全缓解7例,部分缓解4例,稳定l例,总有效率为92 ％;1、3、5年总生存分别为8、3、2例,死亡6例,其中肺部感染死亡3例,肿瘤进展死亡2例,死亡原因不详1例,4例随访期间未出现病情进展,3例发展为多发性骨髓病(MM).结论 SP的整体治疗效果好,但部分患者因发展为MM或者发生肺部感染等原因导致生存率降低.     

以肺部占位为首发症状的多发性骨髓瘤1例及文献复习

多发性骨髓瘤(multiple myeloma,MM)是一种恶性浆细胞瘤,特征是骨髓浆细胞克隆性增殖,血、尿中出现单克隆免疫球蛋白及相关的靶器官损害。临床表现为贫血、骨痛和溶骨性破坏、肾功能损害、反复感染等。髓外浆细胞瘤为首发表现的MM较为少见,而以肺部浆细胞瘤为首发表现的MM更加罕见,我科近期收治1例患者,现报道如下。     

髓外多发性浆细胞瘤放、化疗一例报告

髓外浆细胞瘤虽不罕见,但髓外多发性浆细胞瘤放化疗综合治疗后瘤存部活八年十一个月,最后侵犯骨髓者较罕见。患者女性,32岁,煤矿临时工,门诊号108772。患者77年5月初诊时鼻塞鼻(?)月余,检查:右侧鼻粘膜隆起表面高低不平,左侧(一),病理报告:(右鼻腔)浆细胞瘤,经三个月 Co~(60)TD66.6 GY 照射后右鼻腔病     

骨孤立性浆细胞瘤18例临床分析

 目的　通过总结分析骨孤立性浆细胞瘤（SBP）的临床特点及治疗方法来提高其诊治水平。方法　回顾分析5家医院1999年至2009年收治的18例SBP的临床资料,对其临床特点及治疗疗效进行总结。结果　18例SBP患者年龄35～78岁,平均年龄54.2岁,男女比2.6∶1。通过随访治疗1～10年,采用化放疗治疗的10例患者有8例生存,2例死于进展为多发性骨髓瘤（MM）,单纯化疗或单纯放疗的8例患者中有1例因化疗相关并发症死亡,2例死于进展为MM。全部患者目前共有11例处于持续缓解状态（CCS）,2例因进展为MM在积极治疗中,平均CCS时间为47.3个月。结论　SBP是低度恶性肿瘤,化疗和放疗是其治疗的主要手段,化放疗相结合预后满意,部分患者可转化为MM。     

累及中枢神经系统多发性骨髓瘤9例

目的:探讨多发性骨髓瘤累及中枢神经系统的诊治及其预后.方法:回顾性分析9例累及中枢神经系统的多发性骨髓瘤患者的诊治及其预后.结果:418例多发性骨髓瘤患者中有9例并发中枢神经系统侵犯,发生率为2.2%,其中脑膜受累最为常见(5/9),其次为垂体(2/9).所有患者在诊治过程中均伴有多处其他部位软组织的髓外病变.中位随访时间为30个月,9例患者的中位生存时间为29个月,累及中枢神经系统后中位生存时间仅为5个月.结论:多发性骨髓瘤累及中枢神经系统较为少见,受累部位以脑膜最为常见,常合并其他部位的软组织浆细胞瘤.累及中枢神经系统的多发性骨髓瘤患者预后不良,在治疗上尚缺乏有效的干预手段.     

髓外浆细胞瘤四例报道并文献复习

 目的 探讨髓外浆细胞瘤的诊断与治疗。方法 回顾分析4例髓外浆细胞瘤的临床资料及复习相关文献。结果 髓外浆细胞瘤预后相对较好。结论 手术加放疗为有效的治疗手段，有不良预后因素者应予以辅助性化疗。     

骨的孤立性浆细胞瘤和髓外浆细胞瘤

在浆细胞病中，孤立性浆细胞瘤（骨的孤立性浆细胞瘤和髓外浆细胞瘤）比多发性骨髓瘤罕见，然而两者间关系仍不清楚。１９６０～１９９４年间，作者治疗骨的孤立性浆细胞瘤２４例，髓外浆细胞瘤２０例。诊断标准为：（１）临床与放射学检查未发现另外病变；（２）活检证实为浆细胞瘤；（３）骨髓检查阴性（浆细胞＜１０％）；（４）无贫血、高血钙或肾受累证据。随访平均１１２个月，骨的孤立性浆细胞瘤中５４％、髓外浆细胞瘤中４０％发展为多发性骨髓瘤，两者差异无显著意义（Ｐ＞０．０５）。作者建议将孤立性浆细胞瘤分为潜伏型与侵袭型：肿瘤细胞分化良好者病变趋向潜伏静止，低分化者病变具侵袭性，容易发展为多发性骨髓瘤。治疗选择广泛切除或彻底刮除，瘤腔用液氮或酚等灭活后植骨或骨水泥充填，术后辅以放疗。侵袭型者需辅以化疗，以延缓播散为多发性骨髓瘤。     

染色体1q21扩增对多发性骨髓瘤预后及治疗效果的影响

目的 探讨1q21扩增在新诊断的多发性骨髓瘤(MM)患者中对治疗效果的影响和预后价值.方法 收集2008年6月至2010年6月收治的52例初治MM患者,利用荧光原位杂交(FISH)技术对其进行1q21扩增的检测,并分析其与患者临床特征、疗效及预后的关系.结果 在52例初治MM患者中,30例(57.7％)伴有1q21扩增,其临床特征(性别、恶性胸腔积液、髓外浆细胞瘤、骨质破坏、β 2-微球蛋白、清蛋白、血红蛋白、血钙、浆细胞数、分型及临床分期等)与不伴有1q21扩增的患者比较,差异均无统计学意义(均P＞ 0.05).52例患者被分为1q21扩增组(30例)和1q21未扩增组(22例),分别接受4个疗程硼替佐米联合地塞米松方案化疗,两组治疗总有效率差异无统计学意义[80.0％(24/30)比81.8％(18/22),P＞ 0.05].扩增组与未扩增组中位总生存期分别为26个月(6～50个月)和30个月(12～ 85个月),差异无统计学意义(P=0.409);两组中位无进展生存期分别为8个月(1～31个月)和20个月(3～ 48个月),差异有统计学意义(P=0.019).多因素分析结果显示,纳入因素中仅含1q21扩增同时伴有2个以上的复杂染色体核型是疗效的独立影响因素(P=0.031).结论 1q21扩增是影响MM患者的一个不良预后因素,1q21扩增阳性的患者接受硼替佐米的治疗效果与阴性的患者无明显差异.但1q21扩增阳性患者的中位PFS较阴性患者明显缩短,复发较快.     

Myelomatous meningitis

BACKGROUND: The most frequent nervous system complications of multiple myeloma are peripheral neuropathy and epidural spinal cord compression. Myelomatous meningitis (MM) has been considered rare. The current study was performed to characterize the clinical presentation, treatment, and outcome of MM. METHODS: The study was a case series of 14 patients with cerebrospinal fluid (CSF)-positive MM who were treated at a tertiary care university medical center. RESULTS: Fourteen patients with advanced multiple myeloma were treated with involved-field radiotherapy (to the brain in 5 patients and the spine in 6 patients) and intra-CSF chemotherapy (ventricular in 10 patients and lumbar in 4 patients). The best response to treatment included 6 partial responses and 8 patients with progressive disease. The median duration of response was 2.5 months (range, 0-6 months). Cause of death was progressive neurologic disease in 6 patients, combined systemic and neurologic disease in 6 patients, and systemic disease progression in 2 patients. CONCLUSIONS: MM is rare and morbid entity (6-month neurologic disease progression-free survival rate of 7%), and appears to be no more responsive to treatment than solid tumor carcinomatous meningitis.     

Emergence of central nervous system myeloma in the era of novel agents

Although multiple myeloma (MM) remains an incurable disease, considerable improvements in survival have been made with the introduction of autologous stem cell transplantation and new drugs. Central nervous system (CNS) MM is a rare complication associated with poor survival. Historically, CNS disease developed early in the course of MM; however recently, patients often present with CNS disease following multiple lines of therapy. It is hypothesized that exposure to novel agents (thalidomide, lenalidomide and bortezomib) changes the natural history of MM, increasing the lifetime risk of CNS disease. We analysed the baseline characteristics, treatment and outcome data of patients who presented with CNS MM at Peter MacCallum Cancer Centre between 2001 and 2010. Seven patients were identified, from 2005 onwards. All patients were Durie–Salmon stage IIIA or IIIB and International Staging System Scores I to III at baseline. All had received at least three lines of therapy, including high‐dose chemotherapy with autologous stem cell transplantation and a novel agent, prior to developing CNS MM. Median time from diagnosis to CNS disease was 24 months (range 10–42). All patients died after developing CNS disease with median survival post‐CNS disease of 2 months (range 1–23). The incidence of CNS MM is increasing, and time to development of CNS manifestations is prolonging, associated with increased use of high‐dose chemotherapy and novel agents. Whether this is due to improved overall survival or specific characteristics of these therapies is not clear. Despite the availability of novel agents, survival after CNS MM remains poor. Copyright © 2011 John Wiley & Sons, Ltd.     

高危多发性骨髓瘤的现状挑战与思考

多发性骨髓瘤（multiple myeloma,MM）是一种常见的血液系统恶性肿瘤,其发病率居血液系统恶性肿瘤第2位,目前仍不可治愈。不同MM患者生存时间跨度大,短至数月,长至15年以上,这说明MM患者间存在着非常大的生物学异质性,因此根据细胞遗传学异常、临床特征、基因表达谱等因素将MM进行危险度分层,对判断该病的疾病进程、选择治疗方案、评估预后等具有非常重要的临床价值。其中高危多发性骨髓瘤（high risk multiple myeloma,HRMM）对化疗极不敏感、生存时间短、易进展、预后极差。因此,如何改善HRMM的预后已成为临床工作的重点和难点。      

Plasma cell leukemia

Opinion statement Plasma cell leukemia (PCL) is a rare aggressive variant of multiple myeloma (MM) characterized by a fulminant course and poor prognosis. The median survival is measured in months. Therapy and prognosis partially depend on whether the disease presents de novo or as a secondary process involving the leukemic transformation of a previously diagnosed MM. Secondary PCL represents a terminal event for refractory/relapsed MM and is usually not responsive to any treatment modality. The optimal regimens for the treatment of primary PCL have not been firmly established. Induction with combination chemotherapy, followed by high-dose chemotherapy (preferably within the setting of a clinical trial), is the current recommended approach for eligible patients.     

An unusual presentation of extramedullary plasmacytoma in testis and review of the literature

Extramedullary plasmacytoma is a rare plasma cell neoplasm, and it is extremely uncommon in the testicles. We report a 73-year-old man with multiple myeloma presented with testicular plasmacytoma. He complained of left leg pain and scrotal swelling. Ultrasonography revealed testicular masses. Pathologic examination of the orchiectomy specimen showed plasmocytoma with kappa expression. Multiple lytic bone lesions were seen in bone survey scans, serum immunoelectrophoresis and bone marrow aspiration aided to the diagnosis of multiple myeloma. He received chemotherapy, melphalan and prednisolone, and palliative radiotherapy. He succumbed to disease after 8 months.     

Response to first-line chemotherapy and long-term survival in patients with multiple myeloma: results of the MM87 prospective randomised protocol

In this study we evaluated whether a good response to conventional chemotherapy, i.e. a significant tumour reduction, is a prerequisite for improved survival in multiple myeloma (MM). Between January 1987 and March 1990, 341 consecutive previously untreated patients with MM received chemotherapy within the prospective, multicentre, randomised Protocol MM87. Of these, 258 patients were evaluable for both response and long-term survival and 244 (94.6%) have died. The median survival of all patients was 40 months (6-162 months). The median survival did not differ between patients who had complete response (CR) (50 months (9-162 months)), partial response (PR) (46 months (8-147 months)) or stable disease (SD) (41 months (7-135 months)). The median survival was shorter (13.6 months (6-135 months)) (P<0.0001) in patients whose disease progressed while they were receiving first induction chemotherapy. Causes of death were more frequently (P=0.04) related to MM in patients who had progressive disease (PD) than in patients who had a CR or PR or SD. The main clinical and laboratory characteristics were similar in the four groups. These data indicate that patients who maintain SD during first-line chemotherapy have a prognosis similar to that of patients who attain a response. Only patients whose disease progresses have a distinctly worse outcome.     

A cost-utility analysis comparing intensive chemotherapy alone to intensive chemotherapy followed by myeloablative chemotherapy with autologous stem-cell rescue in newly diagnosed patients with stage II/III multiple myeloma; a prospective randomised phase III study

A prospective randomised phase III study in patients < or =65 years old with previously untreated multiple myeloma (MM), intensive chemotherapy followed by myeloablative chemotherapy and autologous stem-cell rescue was compared with intensive chemotherapy alone. This economic evaluation was based on detailed data from patient charts and hospital information systems. In the intention-to-treat analysis, mean total treatment and follow-up costs of the myeloablative treatment arm were 81,643 euros compared to 68,802 euros for the chemotherapy arm (P=0.09). Costs per quality-adjusted life year were 51,357 euros versus 37,328 euros. In the clinical study, no significant differences were found in overall survival after a median follow-up of 33 months from randomisation. Intensive chemotherapy is regarded as standard therapy for younger patients with previously untreated MM. Cost-effectiveness of myeloma therapy after 3 years of follow up seems not to be favoured by myeloablative treatment with autologous stem-cell rescue.     

生存5年以上多发性骨髓瘤患者43例临床观察

目的 分析生存期大于5年多发性骨髓瘤(MM)患者的临床特点及其与预后的关系.方法 收集2005年9月至2009年9月确诊的43例生存期大于5年MM患者的临床资料,总结患者临床特征、实验室指标和治疗情况,并分析其与生存期的关系.结果 43例患者中,男性23例,女性20例,中位年龄59岁,中位生存期80个月,不同的国际ISS分期对应的中位生存期分别为Ⅰ期120个月,Ⅱ期93个月,Ⅲ期80个月.所有患者均接受过硼替佐米化疗;复发患者接受了含硼替佐米方案、DECP方案、来那度胺、三氧化二砷及脂质体多柔比星等药物治疗.化疗中位疗程数25个(15 ～ 46个).结论 MM的预后不仅与肿瘤负荷、宿主因素及肿瘤生物学特征等有关,还与治疗方案及对治疗的反应密切相关.硼替佐米、来那度胺等新型靶向药物对初诊及复发难治MM均有较好疗效.     

Nonsecretory and Light Chain Escape in Patients With Multiple Myeloma

Background

Multiple myeloma (MM) is characterized by the secretion of monoclonal protein by malignant plasma cells in the vast majority of cases. We identified and analyzed patterns of disease relapse and progression associated with disappearance of the paraprotein (“nonsecretory [NS] escape”), or conversion from production of intact Ig molecule to its associated light chain (“LC escape”).

Continuous Administration of Low-Dose Cyclophosphamide and Prednisone as a Salvage Treatment for Multiple Myeloma

Purpose:The purpose of this study was to evaluate the efficacy and tolerability of continuous low-dose cyclophosphamide and prednisone (CP) as a salvage therapy for multiple myeloma (MM).Patients and Methods:A total of 27 consecutive patients with MM received a treatment regimen that consisted of oral cyclophosphamide 50 mg and prednisone 15 mg daily. Nineteen patients had severe comorbid conditions; 8 were unwilling to continue conventional chemotherapy as a result of severe infection associated with the conventional chemotherapy. Patients had received 1 to 4 chemotherapeutic regimens before the enrollment.Results:The overall response rate (complete remission, very good partial response, and partial response) was 66.7%. The median time to response was 2 months. In the patients who responded to the treatment, the median progression-free survival (PFS) has not been reached. In the nonresponding patients, the median PFS was 4 months.Conclusion:Continuous low-dose CP is an effective and well-tolerated salvage therapy for MM.