老年IgE-λ型多发性骨髓瘤1例报告

<正>多发性骨髓瘤(MM)是浆细胞的恶性克隆性增生性肿瘤,约占所有恶性肿瘤的1%,约占血液肿瘤的10%〔1〕。本病好发于中老年人,常伴有骨痛、贫血、肾衰竭、感染等各种表现。临床上常见为IgA型和IgG型,IgD型少见,IgM型罕见,IgE型极其罕见。1病例摘要患者,男性,72岁,因"腰痛1月余,腹胀15 d"入院。患者     

IgE型多发性骨髓瘤一例

IgE型多发性骨髓瘤非常罕见, 预后不良。本文结合文献复习报告一例IgE-κ型多发性骨髓瘤诊断及治疗过程, 通过规范治疗, 获得了较好反应。     

IgA型多发性骨髓瘤生物行为与临床特征的研究

报道IgA型多发性骨髓瘤20例，与IgG型多发性骨髓相比较，虽然高粘滞综合征、出血、高钙血症及髓外浸润稍多，但统计学上差异不显著，感染发生率差别也无显著性。然而IgAMM病程进展快，疗效不持久，生存期短，仍具有一定特征性。     

冒烟型多发性骨髓瘤临床新进展

1980年Kyle和Greipp首先描述了6例符合MM诊断标准（即血清M蛋白＞30 g/L和骨髓中骨髓瘤细胞比例＞10%）但没有溶骨性病变或单克隆球蛋白相关症状的病例,并将其命名为“冒烟型多发性骨髓瘤”（smoldering multiple myeloma,sMM）.之后，又陆续报道了一些类似病例。最近20年来，在sMM的诊断标准、发病机理、自然病程和治疗等方面都取得了重大进展。本文将详细地介绍sMM的诊断标准、自然病程和治疗的最新进展。     

IgD型多发性骨髓瘤18例临床特征和生存分析

目的探讨IgD型多发性骨髓瘤(MM)的临床表现与实验室检查特点、对治疗的反应、生存及预后情况。方法回顾性分析中山大学附属第一医院1996-01-01—2010-05-30收治的18例IgD型MM患者的临床资料。结果 IgD型MM占所有MM的4.4%,起病中位年龄54岁(36～70岁)。18例IgD型MM中,IgD-λ型16例(88.9%),IgD-κ型2例(11.1%);ISS分期中,Ⅲ期10例(占55.6%);DS分期中,18例患者均为Ⅲ期;2例(11.1%)合并淀粉样变,4例(22.2%)伴有髓外浸润;2例(11.1%)起病时有高钙血症;10例(55.6%)有肾功能损害。10例接受≥4个疗程方案化疗,总有效率60.0%;达平台中位时间为5.2个月,中位疾病进展时间为7.8个月,中位生存时间为13.0个月。结论 IgD型MM是一种侵袭性疾病,诊断时多处于疾病晚期,临床表现和实验室检查与其他亚型MM有较大差异,对治疗反应差,生存期短,预后差。     

多发性骨髓瘤合并轻链型淀粉样变性患者的临床特征及预后分析

目的：分析多发性骨髓瘤合并轻链型(AL型)淀粉样变性患者的临床特征、治疗和预后。方法：回顾性分析2009年7月至2022年12月在东部战区总医院国家肾脏疾病临床医学研究中心确诊的多发性骨髓瘤合并AL型淀粉样变性患者的临床资料、治疗反应及预后特征。结果：71例患者中主要以水肿(84.5%)起病,以IgG型M蛋白最常见,中位浆细胞比例15%。70例(98.6%)患者累及肾脏,41例(57.7%)累及心脏。本组患者主要接受含硼替佐米(32.4%)或沙利度胺(25.4%)的方案治疗,可评估患者总体血液学缓解率为75.0%,其中完全缓解率为8.3%,非常好的部分缓解率为38.9%,部分缓解率为27.8%;仅1例(6.7%)患者取得心脏缓解,15例(41.7%)患者取得肾脏缓解。中位随访时间为16(1.0～120.0)月,中位生存时间为34月,6月、1年、2年和4年的累积生存率分别为83.6%、75.2%、62.2%和43.0%。年龄、浆细胞比例和氨基末端脑钠肽前体水平与患者预后独立相关。结论：多发性骨髓瘤合并AL型淀粉样变性患者总体预后不佳。抗浆细胞治疗有效,但器官缓解率较低,年龄、心脏受累严重...     

多发性骨髓瘤临床线索分析及细胞学特征

多发性骨髓瘤(multiple myeloma，MM)发病率占造血系统恶性肿瘤的。10％～15％，多见于中老年人。本病在不同人群中所表现的特点和临床经过有所差异。目前国内外对其临床及实验室检查特点的研究自益重视。为进一步了解MM，降低误诊率，本文对我院近年来确诊的41例MM进行综合分析。     

多发性骨髓瘤继发Ⅰ型冷球蛋白血症患者的临床特征与转归

目的:探讨7例多发性骨髓瘤(multiple myeloma, MM)继发Ⅰ型冷球蛋白血症(cryoglobulinemia, CG)患者的临床特征、治疗及转归。方法:回顾性分析2015年1月—2023年3月北京协和医院确诊的7例MM继发Ⅰ型CG患者的临床资料、治疗方案和生存结局。结果:7例患者中,男5例,中位诊断年龄为56(40～71)岁。4例继发于冒烟型骨髓瘤。CG受累器官包括皮肤(n=5)、周围神经(n=4)、关节(n=4)和肾脏(n=1)。中位冷球蛋白水平为13 046.5(693.8～33 988.0) mg/L,所有患者均为IgG单克隆型冷球蛋白,与M蛋白类型一致。7例患者均接受了抗浆细胞治疗,其中1例因CG相关急性肾衰竭同时进行了血浆置换。6例有随访资料的患者均获得了MM血液学缓解,4例有CG相关症状者均达到临床缓解。中位随访37(10～45)个月后,1例失访,1例因治疗相关感染死亡。结论:MM是Ⅰ型CG相对少见病因,对于有皮肤、周围神经、关节等受累表现的MM患者,应考虑到继发性CG的可能。     

轻链型多发性骨髓瘤一例

患者,女性,61岁。因反复头昏,乏力,加重2个月入院。一年前,患者无明显诱因感头昏、乏力,伴活动后心悸,当时未行诊治。2个月前患者因受凉后出现咳嗽、咳痰,并发热,自服感冒药症状无缓解。且头昏、乏力加重,上一层楼即感心悸不适。查体:中度贫血貌,胸骨压痛,浅表淋巴结肿大,心肺(-),肝脾不大。实验室检查:Ｈｂ71ｇ/Ｌ,尿蛋白(),尿本周氏蛋白阳性;ＷＢＣ4.8×109/Ｌ,ＢＰＣ73×1012/Ｌ;ＩｇＧ6.14ｇ/Ｌ(8.00～15.50ｇ/Ｌ),ＩｇＡ0.16ｇ/Ｌ(0.74～2.86ｇ/Ｌ),ＩｇＭ0.21ｇ/Ｌ(0.62～2.26ｇ/Ｌ);抗λ血清和尿之间均出现一条明显沉淀线;尿素4.63ｍ…     

老年多发性骨髓瘤的临床特征及预后因素分析

目的明确老年多发性骨髓瘤(MM)患者的临床特征,探讨影响老年MM预后的因素。方法回顾性分析2004年1月至2011年1月在吉林大学中日联谊医院收治的MM患者300例,其中老年患者134例,通过与非老年组各种临床因素对比观察分析老年组的临床特征;并通过Cox回归模型进行多因素分析,采用KaplanMeier法计算累积生存率,探讨各临床因素对其预后的影响。结果老年组与非老年组比较,感染发生率明显升高,血红蛋白(Hb)、血清白蛋白(ALB)下降明显,骨髓浆细胞比例、β2-微球蛋白(β2-MG)、肌酐、血钙及尿本周蛋白(BJP)阳性率明显升高。ISS分期中Ⅲ期患者在老年患者中所占比例明显升高。多因素分析显示β2-MG、ALB、ISS分期和治疗方法是影响生存的独立预后因素。结论老年MM患者具有相对较重的临床表现,多重因素影响其预后。     

多发性骨髓瘤的心脏损害

多发性骨髓瘤常累及心脏,主要表现为充血性心力衰竭、心绞痛、急性心肌梗死、心律失常和心包积液。心电图、超声心动图、放射性核素扫描、核磁共振显像能帮助诊断。诊断的金标准是心肌活检。多发性骨髓瘤合并心脏受累者的预后很差,但一些新的治疗方法为患者带来了曙光。     

Melphalan-Related Leukemia in Multiple Myeloma

ABSTRACT. Five patients with multiple myeloma ending in acute leukemia are described. The preleukemic phase was characterized by anemia, leukopenia and/or thrombocytopenia. The incidence of acute leukemia in myeloma was calculated to be 6%. Melphalan therapy for more than two years increased the incidence to 14%. All patients who developed leukemia had received a total melphalan dose of at least 1100 mg.     

Presenting Features and Prognosis in 72 Patients With Multiple Myeloma Who Were Younger Than 40 Years

The purpose of this study was to analyse the presenting clinical and laboratory features and the outcome of 72 patients with multiple myeloma (MM) who were younger than 40 years. The records of all Mayo Clinic patients with MM younger than 40 years who were seen between 1 January 1956 and 31 December 1992 were reviewed. Survival was measured from the date when treatment was required to the date of last follow-up or death. The frequency of MM in patients younger than 40 and 30 years in 3278 Mayo Clinic patients was 2.2% and 0.3%, respectively. The main presenting clinical features were bone pain (66%), fatigue (26%), extramedullary plasmacytomas (19%) and bacterial infection (11%). Renal function impairment (creatinine level ≥ 177 μmol/l) and hypercalcaemia (serum calcium value ≥2.75 mmol/l) occurred in 29% and 30% of patients, respectively. Among the 57 patients evaluable for response the objective response rate was 54%. 14/35 patients treated with a single alkylating agent achieved an objective response, whereas 17/22 patients given combination chemotherapy had an objective response ( P  = 0.013). However, this higher response rate did not result in a significantly longer survival. The median survival for the 72 patients was 54 months. Patients with good prognostic features (normal renal function or low β₂-microglobulin level) had a median survival of 8 years. The actuarial survival at 5 and 10 years after initiation of therapy was 43% and 13%, respectively. In summary, survival in very young patients with myeloma is longer than that observed in series of patients of all ages, especially in those with good prognostic factors.     

Osteogenesis Imperfecta Associated with Multiple Myeloma

In a case of osteogenesis imperfecta with multiple fractures already from childhood, myelomatosis was diagnosed at the age of 52 years because of a serum M-component (IgG, lambda), Bence Jones proteinuria, myeloma cells in the bone marrow, and osteolytic skeletal lesions. She died 10 months later. A partial postmortem examination of a larger bone lesion confirmed the diagnosis.     

Multiple Myeloma in Primary Biliary Cirrhosis

A patient who had primary biliary cirrhosis and, simultaneously, multiple myeloma (IgG, lambda) is reported. In chronic liver diseases, polyclonal hypergammaglobulinaemia is common, monoclonal gammopathies have also been found in rare instances, being attributed to chronic antigenic stimulation of the reticuloendothelial system by antigens from the intestinal flora. The mechanisms (as chronic antigenic stimulation, immunologic disturbances and granulomata formation) which apply to explain the development of monoclonal gammopathies in other conditions are also found in primary biliary cirrhosis. It is postulated that, in this disease, the development of multiple myeloma could be not merely coincidental but also the result of chronic stimulation of the reticuloendothelial system.     

Multiple Myeloma after Phenytoin Therapy

We report a case of multiple myeloma in a 48-year-old woman treated with phenytoin for 55 months. This association may be fortuitous. The depressive effect of phenytoin on the immune responses, however, could account for the occurrence of the dysglobulinaemia. This case points out the need for periodic examination of serum proteins in patients chronically treated with hydantoin derivatives.     

Hypertrophic Cardiomyopathy as a Manifestation of Multiple Myeloma

Multiple myeloma （MM） is a malignancy of the plasma cell characterized by migration and localization to the bone marrow where cells then disseminate and facilitate the formation of bone lesions. It is associated with a constellation of disease manifestations, apart from osteolytic lesions, anemia and immuno-suppression due to loss of normal hematopoietic stem cell function, and cardiac amyloidosis due to monoclonal immunoglobulin secretion as well. Amyloid infiltration of the heart may frequently masquerade as hypertrophic cardiomyopathy （HCM）. HCM, of which underlying cause and pathogenesis are largely unknown, is characterized by left and/or right ventricular hypertrophy, with predominant involvement of the interventricular septum in the absence of other causes of hypertrophy, such as hypertension or valvular heart diseases. While excessive hypertrophy of the myocardium is most commonly associated with myocyte hypertrophy, infiltration with amyloid always needs to be considered. In this report we presented two cases of multiple myeloma that mimicked hypertrophic cardiomyopathy so closely that it required bone marrow or endomyocardial biopsy to establish the diagnosis.     

Critical Study of Staging in Multiple Myeloma

130 patients with multiple myeloma were revieved for a retrospective study of their classification according to Durie & Salmon. No significant difference was found in survival between patients in stages I, II and III (median survival 24.8, 32.1, and 17.3 months, respectively). Of the classical criteria affecting survival, only the Hb level showed any significant influence (P < 0.04). In accordance with this finding, the survival time was much shorter (P < 0.001) in patients with renal failure than in patients without (median survival 7.4 and 24.8 months, respectively); in addition, thrombopenia, fever, old age, and above all, the % of bone-marrow plasmocytes present, were shown to be decisive factors.     

Nonsecretory IgD-Kappa Multiple Myeloma in a Patient with Gaucher's Disease

A patient with Gaucher's disease and non-secretory multiple myeloma is described. The presence of typical Gaucher and myeloma cells was confirmed by transmission and scanning electron microscopy. The immunological studies established the diagnosis of ‘non-secretory’ type of multiple myeloma.     

Evaluation of Serial Bone X-ray Examination in Multiple Myeloma

ABSTRACT. Fifteen patients with multiple myeloma stage III were treated with a combination of cytostatics and plasmapheresis in a sequential trial running for 60 weeks. Thirteen patients showed clinical improvement and ten a reduction of their myeloma protein by at least 50%. Bone X-ray examination was performed every 15 weeks. Progression of bone lesions was seen in one patient, whereas the radiographic picture was unchanged in the others. It is concluded that bone X-ray, although essential in the diagnosis and staging of multiple myeloma, is not suitable for the monitoring of patients during treatment.