多发性骨髓瘤致心肌淀粉样变1例

1病例资料患者,男性,70岁,以反复活动后气喘2月余为主诉于2020年7月19日入院.入院前2个月快步走约50 m后感气喘不适,休息可稍缓解,症状反复发作,无水肿及夜间阵发性呼吸困难,就诊当地医院,考虑肥厚型心肌病,后气喘、乏力症状加重转诊福建医科大学附属第一医院(我院).既往史:否认高血压、糖尿病;个人史:...     

多发性骨髓瘤合并胃淀粉样变性一例

多发性骨髓瘤合并胃淀粉样变性一例晏发超胡卞新谢幼荣高萍任兴昌患者男，５０岁。因上腹部隐痛，饱胀、嗳气、食欲减退，间断性排柏油样大便半个月。于１９９２年１１月１８日就诊，当时查体除轻度贫血貌及脾肋下２ｃｍ外余无异常。血象：Ｈｂ９３ｇ／Ｌ，白细胞、血小板...     

多发性骨髓瘤(轻链λ型)致心力衰竭一例

正心力衰竭是由各种心脏疾病导致的以心功能下降为表现的临床综合征,临床表现为心输出量不足及体循环和(或)肺循环淤血。心肌病是导致心力衰竭的常见原因,包括原发性心肌病和继发性心肌病。继发性心肌病是有已知原因或继发于其他疾病的心肌病变,由于其病因多种多样,有些易被原发病所掩盖而出现误诊和漏诊。现将连云港市第一人民医院收治的1例多发性骨髓瘤(轻链λ型)致心力衰竭患者的诊治过程报告如下。1 临床资料患者女性,63岁,主因"间断胸闷、气短2个     

多发性骨髓瘤合并心肌淀粉样变性诊治进展

多发性骨髓瘤（MM）是一种浆细胞异常增殖的恶性肿瘤,也叫浆细胞骨髓瘤,是由骨髓中异常增殖的浆细胞增生并分泌单克隆免疫球蛋白和（或）轻链（M蛋白）而导致相关的组织器官损伤。异常单克隆免疫球蛋白的轻链片段所形成的淀粉样物质在组织器官沉积而导致淀粉样变性。其中多发性骨髓瘤所致淀粉样变性中最重要的损害是心肌淀粉样病变,此类患者的发病机制复杂,心脏受损的临床表现具有多样性,常合并有其它多种组织及器官受累表现且缺乏特异性,容易误诊。本文拟对多发性骨髓瘤合并心肌淀粉样变性诊治进展做一综述。     

多发性骨髓瘤引发淀粉样管型肾病一例

<正>患者,男,61岁,因“腰痛伴尿中泡沫增多1周”于2020年11月10日入院。患者入院前1周无诱因出现腰部胀痛,伴尿泡沫增多,无其他不适,查尿蛋白1+,血肌酐145 μmol/L(57～111 μmol/L,括号内为正常参考值范围,以下相同),为求进一步治疗遂入我科。患者起病后饮食、睡眠欠佳,大便3～4次/日,体重近半年下降30斤。既往史：高血压病20年,2型糖尿病4年,痛风1年。个人史、家族史无特殊。入院体格检查：     

多发性骨髓瘤合并淀粉样变性3例报告

目的 分析多发性骨髓瘤合并淀粉样变性的临床特征，提高对此病诊治的认识。方法 回顾性分析3例多发性骨髓瘤合并淀粉样变性患者的临床资料。结果 多发性骨髓瘤本身临床症状复杂多样，合并淀粉样变性后，受累及的组织器官更广泛，舌、腮腺、皮肤、心脏、胃肠道、周围神经、肝、脾、肾、肺等均可受累，治疗效果差，预后差。结论 多发性骨髓瘤合并淀粉样变性虽发病率低，表现呈多样性而缺乏特异性，常导致误诊，确诊靠病理活检及刚果红染色，治疗效果差。     

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。     

多发性骨髓瘤与肾功能衰竭

多发性骨髓瘤并发肾功能衰竭（肾衰）的发生率在５０％以上。肾衰发生机制主要为轻链和／或高钙血症的肾毒性作用，其他包括并发轻链肾病、增生性肾炎、肾淀粉样变性、急性尿酸肾病以及瘤细胞直接侵犯肾实质等。肾衰在充分输液后大部分可以逆转。并发肾衰的治疗主要是在充分输液同时尽早化疗，血浆置换是去除大量轻链的最佳方法。     

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…     

Rapidly progressive heart failure in patient with restrictive cardiomyopathy secondary to newly diagnosed multiple myeloma

Restrictive cardiomyopathy is a rare disease of the myocardium characterized by elevated filling pressures, and limited left ventricle filling with usually normal systolic function. The most common cause in our geographic region is AL amyloidosis. This paper describes a case of a patient in whom multiple myeloma was diagnosed in an advanced stage of the disease, with organ damages and progressive heart failure, which was gradually reducing the quality of life and which despite the initiation of intensive treatment led to patient's death.This case proves that the disease gets little attention, is diagnosed late, and therefore it is important to be reminded of this diagnose. Early detection of the aetiology of heart failure has a significant impact on the effectivity and success of the treatment.     

Pleural amyloidosis as the first sign of IgD multiple myeloma

We describe a case of IgD myeloma with amyloid and plasmocytic pleural localisations. At the onset of the disease it mimicked rheumatoid arthritis, which can be the first presentation of both AL amyloidosis and multiple myeloma. Pleural effusion can happen first in IgD myeloma, but our observation is of interest in that it confirms the very rare possibility of pleural amyloid and plasmocytic localisations devoid of pleural effusion.     

Rectal bleeding as a presenting symptom of AL amyloidosis and multiple myeloma

Amyloidosis of the gastrointestinal tract is a rare disease that presents with common, nonspecific signs and symptoms. It may affect any part of the gastrointestinal tract from mouth to anus. The clinical and endoscopic features are diverse and may mimic other diseases, such as inflammatory bowel disease, malignancy, ischemic colitis and, at times, collagenous colitis. We describe an uncommon case of rectal bleeding and anemia with polypoid lesions and ulcerations in the colon, as the presenting symptom of AL amyloidosis and light chain multiple myeloma.     

Fulminant hepatic failure due to secondary amyloidosis

Systemic amyloidosis frequently involves liver, however, clinically apparent liver disease is rare and, if observed, is usually mild. Rarely, primary amyloidosis may present with evidence of portal hypertension or hepatic failure. Although secondary amyloidosis may involve the liver and rarely causes liver pathologies such as cholestatic hepatitis and portal hypertension, to our knowledge, hepatic failure due to secondary amyloidosis has been reported once before. Herein, we presented a woman with subacute fulminant hepatic failure who had no known history of a chronic disease other than episodes of urinary tract infection, and secondary amyloidosis was diagnosed with post-mortem liver biopsy.     

Approach to acute renal failure with multiple myeloma: role of plasmapheresis

Multiple myeloma is commonly complicated by renal failure, which limits therapeutic options and aggravates the prognosis. In myeloma the most common cause of advanced renal failure is myeloma kidney, where light chains produced by tumor cells precipitate and impede kidney function. We suggest that plasma exchange is an appropriate intervention in advanced renal failure from multiple myeloma, and support this notion with two case reports. The two elderly individuals had advanced chronic and acute renal failure, and recovered after the removal of large quantities of light chains by existing membrane separation techniques. We also present an algorithm to approach renal failure and myeloma.     

Hepatic failure caused by plasma cell infiltration in multiple myeloma

Although plasma cell infiltration is not rare in autopsy of patients with multiple myeloma （MM）, it is very rarely detected in living patients. This is because MM rarely causes significant liver dysfunction that requires further evaluation. A 49-year-old man presented with acute renal failure and was diagnosed with kappa light chain MM stageⅡB. Thalidomide and dexamethasone were initiated. The patient developed a continuous increase in bilirubin that led to severe cholestasis. A liver biopsy revealed plasma cell infiltration. He then rapidly progressed to liver failure and died. Treatment options are limited in MM with significant liver dysfunction. Despite new drug therapies in MM, those patients with rapidly progressive liver failure appear to have a dismal outcome.     

多发性骨髓瘤合并肾功能不全38例临床和预后分析

目的研究多发性骨髓瘤合并肾功能不全的临床特点及肾功能可逆性的相关因素和生存相关因素。方法回顾性分析1999-01~2003-12北京协和医院收治的91例初治多发性骨髓瘤及其中38例多发性骨髓瘤合并肾功能不全患者的临床资料。结果91例初治多发性骨髓瘤中有38例(41·8%)合并肾功能不全。合并肾功能不全的患者病程进展更快,贫血更严重,血乳酸脱氢酶(LDH)和β2-微球蛋白(β2-MG)质量浓度更高,以及尿蛋白量更多,同时对化疗的反应更差及生存时间更短。其中34·2%的患者肾功能不全是可逆的,血肌酐和LDH是肾功能可逆的独立预测因素。肾功能可逆患者的生存时间与肾功能正常患者的生存时间比较差异无显著性意义。Cox生存多因素分析发现肾功能可逆性(B=1·294,EXP(B)=3·647,P=0·001)是与生存惟一相关的因素。结论肾功能不全是多发性骨髓瘤常见并发症,相当一部分患者的肾功能可以恢复正常,血肌酐水平和LDH水平是肾功能可逆的重要因素,肾功能可逆性是独立的生存预后因素。     

Cardiac amyloidosis: a practical approach to diagnosis and management

Cardiac amyloidosis, the primary determinant of prognosis in systemic amyloidoses, is characterized by infiltration of myocardium by amyloid protein resulting in cardiomyopathy and conduction disturbances. Cardiac involvement is primarily encountered in immunoglobulin (AL) and transthyretin-associated (hereditary/familial and senile) amyloidoses. Although the latter variants could be indolent, untreated AL amyloidosis with clinical cardiac involvement is a rapidly fatal disease. The management decisions of cardiac amyloidosis are based on the underlying cause. Although treatment of senile systemic amyloidosis is largely supportive, the therapeutic approaches for AL amyloidosis include chemotherapy, autologous stem cell transplantation, and, rarely, cardiac transplantation. The familial variant is potentially curable with a liver ± cardiac transplantation. This narrative review outlines a practical approach to these challenging diagnoses in the face of rapidly evolving management strategies.     

Cutaneous light chain amyloidosis with multiple myeloma: A concise review

Objective/BackgroundCutaneous immunoglobulin (Ig) amyloid light-chain (AL) amyloidosis associated with overt multiple myeloma (MM) is rare and optimal treatment is not well defined. The recently developed highly efficacious MM therapy has brought on a new set of challenges to this field for consideration. The goal of this paper is to describe the characteristics of cutaneous manifestations of systemic AL amyloidosis associated with MM according to age, sex, race, Ig type, plasma cell percentage, and cytogenetic and fluorescent in situ hybridization studies along with their outcomes.MethodsAn electronic search of the PubMed database was performed to obtain key literature in AL amyloidosis and MM, using the following search terms: multiple myeloma, immunoglobulin light chain amyloidosis, and cutaneous amyloidosis. The search results were narrowed by selecting studies in English. Results were confined to the following articles types: case reports, case series, and systematic reviews.ResultsWe identified 32 cases from the PubMed database search and examined their potential relevance. We found the following: (a) higher prevalence in women (two-thirds) and white population; (b) IgG and IgA were equally distributed with lambda (λ) light chain occurring in 53–66% of cases; (c) majority of cases (56%) presented as hemorrhagic bullous lesions, followed by purpura/ecchymosis in 25% of cases; and (d) majority (64%) died within 6 months since diagnosis.ConclusionsWe reviewed the constellation of the cutaneous manifestations of AL amyloidosis with concurrent MM. We found a female predominance, and more than half presented as hemorrhagic bullous lesions. There is a preponderance of λ light chains over kappa (κ) light chains, both as a free light chain (15% vs. 4%) and as an intact Ig (38% vs. 24%; absolute number of 14 vs. 7 patients, respectively). In the subgroup of patients with bullous skin lesions, λ light chain was present in eight cases and κ light chain in seven cases. All κ light chain subtypes presented with bullous lesions and no other cutaneous types of lesions. They carried very poor prognosis with majority of cases surviving only 6 months, much worse than overall patients with AL amyloidosis without myeloma or myeloma without amyloidosis.