多发性骨髓瘤30例临床分析

多发性骨髓瘤是一种浆细胞系统恶性增生性疾病,典型病例浸润骨髓,伴多发性骨胳溶骨性改变,血清和尿蛋白电泳出现同质单株免疫球蛋白增高,少数病例可在晚期发展为浆细胞性白血病。现将我院1964～1981年的30例多发性骨髓瘤分析如下。     

POEMS 综合征24例临床分析

 【摘要】　目的　分析POEMS综合征的临床特征,提高对其诊断、治疗的认识,探索有效的治疗策略。方法　回顾性分析1997年9月至2011年1月24例POEMS综合征患者的临床特点、实验室检查及治疗方法,并复习相关文献。结果　24例中男18例,女6例;年龄40～73岁,中位年龄56.5岁。24例患者均有周围神经病变,19例患者出现肝大,17例患者出现脾大,15例患者出现内分泌改变,18例患者血清M蛋白阳性,21例患者出现皮肤色素沉着。治疗以MP方案联合沙利度胺为主,有效率达100 %。1例行外周血自体造血干细胞移植（PBSCT）2年,周围神经系统症状达到完全缓解,M蛋白持续阴性。结论　POEMS综合征为少见的多系统损害疾病,对于以周围神经病变为主要首发症状的病因不明患者,应进一步筛查,提高临床检出率。左旋苯丙氨酸氮芥联合泼尼松为最有效的治疗方案,PBSCT为POEMS综合征的治疗提供了新的研究方向。     

POEMS综合征四例并文献复习

 目的　探讨POEMS综合征的临床特征及治疗方法。方法　对4例POEMS综合征患者临床资料、相关检查及治疗方法进行回顾性分析。结果　4例患者均有多发性神经病、异常球蛋白血症及内分泌异常,其他症状还有浆膜腔积液、肝脾大等。患者均给予激素、环磷酰胺及沙利度胺治疗及应用人血免疫球蛋白静脉滴注等。结论　POEMS综合征是一种少见的多脏器损害疾病,较难诊断。临床上对可疑病例需进行密切观察及必要的检查,以免误诊。治疗并无特效药物,尚处于探讨阶段。     

克拉霉素、沙利度胺治疗POEMS综合征三例并文献复习

 目的　分析POEMS综合征患者的临床特征和治疗效果,提高对该病的诊治水平。方法　分析3例POEMS综合征患者的临床表现特点和诊治经过,结合文献对POEMS综合征的病因、诊断及治疗进行复习。结果　3例患者均有多发性周围神经病变、皮肤色素沉着、双下肢水肿、甲状腺功能减退;脾大2例,视乳头水肿1例;蛋白免疫固定电泳可发现M蛋白;骨髓浆样瘤细胞比例不增高。3例患者经克拉霉素、沙利度胺联合地塞米松治疗后症状均缓解,随访2～3年处于CR或稳定状态。结论　POEMS综合征是一组临床罕见的以多发性神经病变为主要表现的多系统损害症候群,首发症状和病程不同时期表现复杂、多变,容易误诊、漏诊,应及时进行相关实验室检查明确诊断。克拉霉素、沙利度胺联合地塞米松治疗有一定疗效。     

多发性骨髓瘤伴非骨旁髓外病变临床分析

目的:探讨多发性骨髓瘤伴非骨旁髓外病变的临床特点。方法:回顾性分析2013 年3月至2016 年3 月在西安交通大学第一附属医院血液科住院的多发性骨髓瘤伴非骨旁髓外病变患者26例（A组）、病例资料及随访较完整的无髓外侵犯患者130例（B组）及伴骨旁髓外病变患者10例（C组）的临床资料,比较三者临床特点及预后。结果:多发性骨髓瘤伴非骨旁髓外病变患者发生率为4.68%（26/556）,发生部位为睾丸、椎管硬膜外、皮肤等。伴骨旁髓外病变患者发病率为1.80％（10/556）,发生部位为肋骨附近胸壁和脊柱旁等。A组中6例为反复治疗不缓解,出现髓外侵犯,自确诊到髓外病变出现中位时间为 3（2~12）个月;9例为完全缓解后在骨髓复发或未复发状态出现髓外侵犯,自确诊到髓外病变出现中位时间为16.5（8~45）个月;11例为初诊时伴有髓外侵犯。A组中位生存时间为17.2个月,B组中位生存时间为37.3个月,C组中位生存时间为35.3个月。结论:多发性骨髓瘤伴非骨旁髓外侵犯在骨髓不缓解和缓解状态均可出现,不依赖骨髓微环境生长,是骨髓瘤预后不良因素。骨髓瘤伴骨旁髓外病变患者预后优于伴非骨旁髓外病变患者。     

唑来膦酸治疗多发性骨髓瘤骨损害临床体会

多发性骨髓瘤（multiple myeloma，MM）系浆细胞恶性增殖引起的恶性肿瘤，主要侵蚀骨髓，也波及淋巴组织和其他组织。多发性骨髓瘤患者会出现明显的骨相关事件，骨痛是其中最常见的症状，多由溶骨性损害引起。我科自2006年2月至2008年1月，应用唑来膦酸联合化疗治疗多发性骨髓瘤骨损害患者15例，取得较好的临床疗效，现报告如下。     

POEMS综合征患者就诊行为特征及临床误诊漏诊分析

  目的  了解POEMS综合征患者就诊行为特征,分析POEMS综合征临床误诊、漏诊原因。  方法  回顾性分析在中国医学科学院血液病医院就诊的30例POEMS综合征患者的临床资料,包括患者初诊年龄、性别、首发症状及就诊过程等。  结果  30例POEMS综合征患者的中位发病年龄为51（26～67）岁,男女之比为 1.1∶1,自发病至明确诊断期间的中位就诊次数和时间分别为3（1～6）次和13（1.5～132）个月。患者就诊科室多为神经内科、肾内科、内分泌科、消化科,但确诊科室集中在血液内科。患者临床误诊、漏诊现象普遍存在,误诊及漏诊率高达87%。常见的首诊诊断包括格林巴利综合征、周围神经病和诊断不明。近一半患者在此后多次就诊的过程中更改了原有诊断,部分患者曾疑诊POEMS综合征但均未得到进一步证实。确诊前患者接受的治疗方式包括对症支持、丙种球蛋白+糖皮质激素冲击治疗、糖皮质激素治疗和观察随访。73%（19/26）的患者在治疗过程中出现病情加重。误诊及漏诊的主要原因为POEMS综合征临床表现多样且非特异,患者和医务工作者不易识别;此外,接诊医师专注于本学科范围相关的症状体征,忽略了专科之外的临床表现,也是导致误诊及漏诊的主要原因。  结论  由于POEMS综合征较为罕见且临床表现高度异质性,临床误诊、漏诊较为常见。若接诊医师能够从“一元论”角度探究病因,将本学科范围内症状体征与专科外临床表现联系在一起,加强多学科合作,可有效减少漏诊和误诊的发生。      

骨科知识介绍

1．Albers—Schonberg综合征 [同义名]全身性脆性骨硬化症 大理石骨症 石骨症 本征常有两种类型：一种为良性显性型（Henck—Assmann综合征），临床上多无明显的体征，仅在x线片上可见到弥漫性骨硬化征象。另一型为恶性隐性型，隐性遗传。临床上表现较复杂，多有血液系统的改变，如：贫血、血小板减少等。     

重视浆细胞疾病的临床研究

 浆细胞病（包括多发性骨髓瘤、意义未明的单克隆免疫球蛋白血症、华氏巨球蛋白血症、淀粉样变性、POEMS综合征等）是血液系统常见的恶性疾病,发病率逐年增高。深入地研究该病的临床过程,制订合理的治疗方法是延长患者生存期,改善预后的重要途径。     

POEMS综合征患者内分泌改变特点及临床意义

摘 要：［目的］ 探讨POEMS综合征患者内分泌异常的特点、治疗后的变化规律及其临床意义。［方法］ 收集收治的14例初治POEMS综合征患者(男性9例,女性5例,中位年龄49岁)的临床资料,回顾性分析不同化疗方案对患者内分泌改变的影响。［结果］ 所有患者均具有内分泌异常,其中毛发增多5例、皮肤色素沉着13例、甲状腺功能异常8例、血糖异常7例、皮质醇节律异常4例。9例男性患者中,出现性功能异常8例、乳腺发育5例。经过化疗后,甲状腺功能、性激素水平和皮质醇节律恢复较早,最快者经过2个疗程即可恢复正常;经过2个疗程后即恢复正常比例最高的是甲状腺功能和皮质醇节律。与非硼替佐米组相比,应用硼替佐米化疗患者的性激素水平恢复快,但差异无统计学意义（P=0.486）;在性激素中,硼替佐米对雌二醇水平恢复作用最为明显。［结论］ POEMS综合征内分泌改变涉及到多个内分泌轴,化疗可以有效改善患者的内分泌异常;含硼替佐米的化疗方案相较于传统的化疗方案,可以更有效的改善患者的内分泌异常。     

POEMS Syndrome: an Enigma

POEMS syndrome is a paraneoplastic disorder secondary to an underlying plasma cell dyscrasia. By definition, all patients with POEMS syndrome must display polyneuropathy and monoclonal plasma cell disorder. In addition, at least one major criterion (Castleman’s disease, sclerotic bone lesions, or vascular endothelial growth factor elevation) and one minor criterion (organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema, thrombocytosis, or polycythemia) are required for diagnosis. Treatment is based on extent of the disease. Radiotherapy is used for localized disease. Systemic therapy is required for disseminated disease, with bone marrow involvement by clonal plasma cells, or in patients who progress shortly after radiation. Upfront autologous stem cell transplantation is the treatment of choice for transplant-eligible patients. Outcomes are typically superior to that of standard myeloma. Herein, using a case vignette, we outline the latest evidence regarding the prognostication and management of POEMS syndrome, with a focus on its relapsing-remitting course.     

Up-date on solitary plasmacytoma and its main differences with multiple myeloma

Solitary plasmacytoma is plasma cell neoplasm. It is a localized bone disease and for this reason it is different from multiple myeloma (systemic plasma cell neoplasm). Sometimes, solitary plasmacytoma precedes a following multiple myeloma. Clinical findings of solitary plasmacytoma are related to the univocal localization on damaged bone, while laboratory findings could be similar to multiple myeloma (i.e. M component, kidney dysfunction, blood calcium alterations, increased beta-2-microglobulin). However, during a solitary plasmacytoma, laboratory findings could not be present contemporaneously such clinical complications (i.e. kidney failure, immunological disorders with a trend toward infectious disease and/or autoimmunity, neurological disorders, haematological disorders, amyloidosis, POEMS syndrome). These raise the reason because solitary plasmacytoma has better prognosis compared to multiple myeloma.     

Plasma cell dyscrasia with polyneuropathy--POEMS syndrome presenting with vasculitic skin lesions and responding to combination chemotherapy.

We report a 61-year-old male patient who presented with severe sensorimotor neuropathy, leg edema and skin lesions with M-paraprotein and 50% plasma cells in the bone marrow. The POEMS (Crow-Fukase) syndrome was diagnosed and the skin lesions were compatible with vasculitis according to the histopathology. The patient was treated with aggressive combined chemotherapy, which induced improvement in both the clinical and laboratory parameters of his disease. To the best of our knowledge this is the first report of a vasculitic process underlying the skin changes in the POEMS syndrome. Our findings may shed light on the unknown pathogenesis of this syndrome and the successful results of treatment support the adoption of an aggressive therapeutic approach in symptomatic patients.     

POEMS syndrome: experience with fourteen cases

The POEMS syndrome, also known as Crow-Fukase disease, is a rare multisystem disorder, which may take several years to evolve fully. The combination of symptoms and signs is highly complex and some of the features are detected at sub-clinical level requiring high level of suspicion. The clinical data on POEMS is still evolving with only a few case reports from India. Herein, we report a series of 14 cases with POEMS syndrome at our centre over the past 8 years, which were analysed retrospectively for their clinical features, response to therapy and treatment outcome. Presence of plasma cell dyscrasia (PCD) was essential for inclusion in this study. Confirmation of PCD was done by positive "M" spike in serum and/or urine, bone marrow plasmacytosis or presence of plasmacytoma on biopsy. In addition, the diagnosis of POEMS syndrome needed the presence of at least two of the following features: polyneuropathy, organomegaly, endocrinopathy and/or skin changes. Patients were excluded from study if there was a secondary cause of polyneuropathy like amyloidosis, drugs like vincristine, nerve root or spinal cord compression. Two patients had complete form (all five features) of the syndrome, whereas 12 had incomplete form. Median age was 48 years (range 32-65). Peripheral neuropathy was seen in 13 (92.85%) cases, organomegaly 10 (71.42%), endocrinal involvement 7 (50%) and skin changes 9 (64.28%). An association with Castleman's disease and vasculitis was also noted. With different chemotherapy protocols, all treated patients (n=12), had significant symptomatic improvement with or without objective improvement at median follow up of 48 months (range 6-120). In conclusion, high level of suspicion is required to detect this rare entity.     

IgM multiple myeloma: report of four cases and review of the literature

The differential diagnosis between multiple myeloma (MM) and Waldenstr?m's macroglobulinemia (WM) is generally well defined. Consistent with a diagnosis of MM is the presence of a non-IgM monoclonal gammopathy associated to multiple osteolytic lesions and plasma cell infiltration of the bone marrow. Characteristic of WM is the presence of an IgM monoclonal gammopathy associated to lymphoadenopathy, hepatosplenomegaly, anemia and hyperviscosity syndrome in the presence of a monoclonal lymphoplasmacytoid proliferation in the bone marrow. Nonetheless, few cases of IgM myeloma have been reported that display clinico-pathologic features intermediate between MM and WM. Here, this study describes four of 317 (1.2%) patients with an IgM monoclonal gammopathy in whom the morphologic and clinical features were consistent with a diagnosis of IgM myeloma.     

Angiogenetic factors and biochemical markers of bone metabolism in POEMS syndrome treated with high-dose therapy and autologous stem cell support

The cause of the polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder and skin changes (POEMS) syndrome is currently unknown, but increased levels of vascular endothelial growth factor (VEGF) appear to play a pathogenetic role. Osteosclerotic bone lesions are a characteristic finding in POEMS, but there are no data about the specific role of various molecules that control bone remodeling in patients with POEMS. Serum levels of angiogenic cytokines (VEGF, angiogenin, angiopoietin-2, and basic fibroblast growth factor) along with a series of bone remodeling indices (C-telopeptide of type I collagen, bone-alkaline phosphatase [bALP], osteocalcin [OC], soluble receptor activator of nuclear factor-kappaB ligand [RANKL], osteoprotegerin [OPG], and macrophage inflammatory protein-1alpha) were measured in 2 patients with POEMS before and after high-dose therapy (HDT) with autologous stem cell transplantation and in age- and sex-matched controls. Increased VEGF levels before HDT were reduced significantly after treatment, although levels of the other angiogenetic factors did not differ from that of controls and were less influenced by HDT. Serum RANKL levels were increased before HDT, whereas OPG levels were within the levels of healthy controls, resulting in an abnormal soluble RANKL to OPG ratio. Levels of bone resorption markers (C-telopeptide of type I collagen) were very low or undetectable before HDT, although bALP and OC levels were similar to that of controls. After HDT, soluble RANKL levels decreased, OPG remained rather stable, bone resorption markers increased to levels of normal individuals, bALP levels were rather unchanged, and OC levels increased. Decreasing VEGF levels parallel clinical improvement, and the restoration of normal bone metabolism follows HDT.     

POEMS综合征的诊断和治疗

POEMS综合征是一种罕见的浆细胞疾病,近年来在POEMS的诊断和治疗方面都取得了很大进展。本文将重点阐述POEMS综合征的诊断标准以及早期诊断的要点。在治疗方面,自体造血干细胞移植由于其较好的疗效已经成为年轻POEMS综合征患者的一线治疗。马法兰联合地塞米松也有着较高的缓解率和很好的安全性,可以作为老年或其他不适合移植患者的治疗选择。新药例如来那度胺等给POEMS综合征患者提供了更多的治疗选择。      

Treatment of poems syndrome

Opinion statement (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome is a rare multisystemic paraneoplastic syndrome driven by an underlying plasma cell dyscrasia. More than 95% of patients will have monoclonal lambda sclerotic plasmacytoma( s) or bone marrow infiltration. Recognition of the complex of a combination of peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasmaproliferative disorder, skin changes, sclerotic bone lesions, Castleman disease, thrombocytosis, papilledema, peripheral edema, pleural effusions, ascites, fingernail clubbing, and white nails is the first step in effectively managing the disease. Once a patient has been completely evaluated, each component of the disease should be addressed, while finalizing a treatment plan for the underlying plasma cell proliferative disorder. In patients with a dominant sclerotic plasmacytoma, first-line therapy should include radiation to the lesion. Retrospective analysis and personal experience would dictate that systemic therapy be considered for patients with diffuse sclerotic lesions or absence of any bone lesion and for patients who have not demonstrated stabilization of their disease 3 to 6 months after completing radiation therapy. Treatments with demonstrated benefit include corticosteroids, low-dose alkylator therapy, and high-dose chemotherapy with peripheral blood stem cell transplant. Until the pathogenesis is fully understood, these are the mainstays of treatment for patients with POEMS syndrome.