POEMS syndrome, osteosclerotic myeloma and Castleman's disease: a case report

Abstract Peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes are often the presenting features of the POEMS syndrome. Approximately 50% of these are associated with osteosclerotic myeloma, a rare variant of multiple myeloma and some with Castlemanapos;s disease, an unusual lympho-proliferative disorder. The underlying pathogenetic mechanisms have not been elucidated but amelioration of symptoms with the disappearance of the M-component suggests possible etiopathogenic mechanisms. We present a 40-year-old woman with manifestations of all three disorders. (Aust NZ J Med 1991; 21:454–456.).     

Strontium-89: a novel treatment for a case of osteosclerotic myeloma associated with life-threatening neuropathy

Osteosclerotic myeloma is a rare disorder characterized by paraproteinaemia and osteosclerosis, and may be associated with a progressive peripheral neuropathy. Patients with widespread osteosclerotic lesions can succumb from neurological complications despite systemic chemotherapy. We present a case of disseminated osteosclerotic myeloma associated with POEMS (peripheral neuropathy, organomegaly, endocrinopathy, M band, skin changes) syndrome, which was complicated by a rapidly progressive, life-threatening neuropathy. The patient's symptoms remained unchanged in the face of combination chemotherapy. However, a substantial improvement was seen following outpatient treatment with the commonly available radioisotope strontium 89 in combination with steroids.     

Peripheral blood stem cell transplant for POEMS syndrome is associated with high rates of engraftment syndrome

Polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes (POEMS) syndrome is a devastating syndrome, characterized by peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasma cells, skin changes, papilledema, volume overload, sclerotic bone lesions, thrombocytosis and high vascular endothelial growth factor (VEGF). High-dose chemotherapy with autologous peripheral blood stem cell transplantation (ASCT) ultimately yields excellent clinical responses, but there can be considerable peritransplant morbidity. We have treated 30 POEMS patients with ASCT at Mayo Clinic, Rochester. During transplant period, patients had high rates of fever, diarrhea, weight gain and rash (93%, 77%, 53% and 43%, respectively). Only 13% remained outpatient, and median time to discharge from hospital was transplant day 17 (range 0-175). Splenomegaly was the baseline factor that best predicted for a complicated peritransplant course. Depending on the definition used, approximately 50% of patients satisfied criteria for engraftment syndrome. Earlier and more aggressive use of corticosteroids may be associated with less complicated post-transplant courses. Median overall survival has not been reached; the treatment-related mortality was 3%. In addition, important clinical improvements and reductions in plasma VEGF levels can occur in the absence of significant decrease in the monoclonal protein. Unraveling the mechanisms of the syndrome both in the context of ASCT and in general are challenges for the future.     

A case of POEMS syndrome with thrombocytopenia and biliverdinaemia

Summary This report describes a case of POEMS syndrome with extremely rare complications of thrombocytopenia and biliverdinaemia. The thrombocytopenia appeared to be due to immunopathogenesis. Steroid treatment resulted in complete disappearance of the biliverdinaemia in this patient as well as symptoms and signs of POEMS syndrome, suggesting that the biliverdinaemia was related to POEMS syndrome.     

POEMS综合征13例临床分析

目的 分析研究POEMS综合征的临床特征、以提高对本症的诊治水平。方法 采用回顾性分析对13例POEMS综合征进行分析。结果 13例患者中,4例伴骨髓瘤,其他最主要临床表现为多发性神经病变（100%),皮肤色素沉着（84.6%）,水肿（84.6%),肝、肿大（69.0%及61.5%）及内分泌病变（84.6％）。院外13例均误诊,入院后误诊9例;11例经治疗后症状缓解,2例自动出院。结论 POEMS综合征仍是一个被人们认识不足的疾病,因此应予经重视。     

POEMS综合征32例临床分析

目的加深临床医师对POEMS综合征的认识,比较国内外POEMS综合征患者的临床特征。方法回顾性分析北京协和医院32例POEMS综合征患者的临床特征,并与国内外大宗病例报告进行比较。结果32例患者,平均年龄45、8岁,男女比例2．2：1。临床特征：（1）多发性周围神经病（P）：麻木（84．4％）、感觉异常（87．5％）、肌无力（90．6％）等。肌电图92．9％为神经源性改变,神经活检75．0％为轴索变性。（2）脏器肿大（O）：肝肿大71．9％、脾肿大87．5％、淋巴结肿大62．5％。9例淋巴结活检中5例为Castleman病。（3）内分泌病变（E）：男性勃起功能障碍72．7％、女性月经改变60．0％。（4）单克隆浆细胞增生（M）：血清M蛋白阳性率72．0％（18／25）。（5）皮肤色素沉着（S）：93．8％的患者有皮肤色素沉着。与其他报道相比,临床特征基本相似,但视乳头水肿、脾肿大、多汗等方面有一定差异;M蛋白的发生率与国外报道无差异,显著高于国内既往报道。结论本组POEMS综合征临床特征与国外基本相似,血清M蛋白的发生率并不低于国外。免疫电泳以及骨髓活检轻链组化染色可能有助于提高“M”的检测敏感性。     

POEMS syndrome

POEMS syndrome is a rare paraneoplastic syndrome secondary to a plasma cell dyscrasia. Recognition of the complex of a combination of peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasmaproliferative disorder, skin changes, papilledema, extravascular volume overload (peripheral edema, pleural effusions, ascites), sclerotic bone lesions, thrombocytosis, Castleman disease is the first step in effectively managing the disease. A rise in the blood levels of vascular endothelial growth factor is usually confirmatory. More than 95% of patients will have monoclonal lambda sclerotic plasmacytoma(s) or bone marrow infiltration. 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 those who have not demonstrated stabilization of their disease 3 to 6 months after completing radiation therapy. For those patients with diffuse disease, systemic therapy is indicated. Useful approaches include therapy with 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. The role of anti-VEGF therapies, immune modulatory drugs, and proteasome inhibitors has not yet been defined, but drugs with known high rates of treatment related neuropathy should not be considered as first line therapy.     

以腹腔积液为主要症状的POEMS综合征1例报道并文献复习

<正>POEMS综合征是一种浆细胞疾病。主要症状包括多发性周围神经病变(polyneuropathy,P)、器官肿大(organomegaly,O)、内分泌异常(endocrinopathy,E)、M蛋白血症(M protein,M)及皮肤改变(skin changes,S)。其他症状包括:视神经乳头水肿、多浆膜腔积液、骨质损伤和血小板/红细胞增多等。血管内皮生长因子(vascular endothelial growth factor,VEGF)     

POEMS syndrome, arterial thrombosis and thrombocythaemia

The case of a 22-year-old man with polyneuropathy, endocrinopathy, skin change and monoclonal gammopathy of IgG-lambda type is described. There was no solitary plasmocytoma, osteosclerotic myeloma or Castleman's disease. However, significant thrombocytosis occurred and the patient developed arterial thrombosis, these were attributed to essential thrombocythaemia in the absence of other aetiological factors.     

Dramatic improvement of POEMS syndrome following autologous haematopoietic cell transplantation

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, serum monoclonal protein and skin changes) is a rare plasma cell disorder of unknown pathogenesis and is diagnosed by the demonstration of a plasma cell proliferation at the biopsy of an osteoesclerotic lesion. When the lesions are in a limited area, radiation therapy is usually highly effective. Patients with disseminated disease require systemic chemotherapy, which is not effective in most cases. A patient with severe widespread POEMS syndrome resistant to melphalan who experienced a dramatic improvement after high-dose melphalan followed by autologous haematopoietic cell transplantation (AHCT) is reported. We believe that this is the first reported case of POEMS syndrome treated with AHCT, a procedure that could be considered in similarly affected patients.     

POEMS综合征误诊为肝硬化伴顽固性腹水1例

1．临床资料：患者,男性,60岁。因肢体无力11个月,腹胀1个月就诊。患者于2005年10月起,逐渐出现手足麻木、乏力,外院诊断为周围神经炎,未予治疗。2005年12月在我院神经内科亦诊断为周围神经病变,予强的松15 mg/d治疗1个月,10mg/d治疗2个月,5mg/d治疗至2006年6月。2006年7月开始出现腹胀,B超示大量腹水,停用强的松,予白蛋白、速尿治疗,效果不佳。[第一段]     

POEMS syndrome complicated by extensive arterial thromboses

POEMS syndrome—characterised by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes—is a rare multi-systemic disease. Its skin change is often like that of connective tissue diseases such as scleroderma. Although clinical manifestations of POEMS syndrome are known to be diverse, arterial or venous thrombosis is a less-recognised feature. We report a 41-year-old man with scleroderma-like skin change who was initially negative for monoclonal protein, but finally confirmed as having POEMS syndrome. During the disease course, he was complicated by extensive arterial thromboses including stroke. This case evokes the need to include POEMS syndrome in the differential diagnoses in patients with scleroderma-like skin change and to repeat measurements of monoclonal protein to confirm its diagnosis. A POEMS patient with extensive arterial thromboses may have a catastrophic disease course, requiring earlier diagnosis and more aggressive treatment.     

Bortezomib: a new therapeutic option for POEMS syndrome

Objective: POEMS syndrome with its classical five findings (Polyneuropathy, Organomegaly, Endocrinopathy, M protein, and Skin changes) is a rare multisystem disease. Proinflammatory and proangiogenic cytokines play important roles in its pathogenesis. Treatment options are still debated. Methods: We present a 65‐year‐old man with POEMS syndrome who was successfully treated with bortezomib. Results: After seven cycles of this protocol, serum M protein level declined to normal range, and near‐to‐complete remission was achieved. His symptoms of polyneuropathy improved dramatically. Conclusion: Bortezomib may be an effective and safe therapeutic option for patients with POEMS syndrome.     

POEMS综合征相关肾损害——肾小球弥漫系膜溶解

49岁中年男性,因神经系统和内分泌系统异常,脾大,少量蛋白尿和镜下血尿,肾活检形态学为弥漫系膜溶解,结合临床和实验室检查,考虑POEMS综合征相关肾损害。     

POEMS syndrome: A multisystem clonal disorder

Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome is a rare condition defined by monoclonal plasma cell disorder, peripheral neuropathy, and other systemic symptoms. The pathophysiology of POEMS syndrome is unknown, but the overproduction of vascular endothelial growth factor (VEGF) appears to be an important contributory element. The diagnosis of POEMS syndrome requires the presence of both mandatory criteria (ie, polyneuropathy and a monoclonal plasma cell disorder), at least one major criterion (ie, osteosclerotic bone lesions, Castleman disease, or elevated serum or plasma levels of vascular endothelial growth factor), and at least one of the six minor criteria. POEMS syndrome lacks a standard treatment, but patients with limited sclerotic bone lesions are typically treated with radiation therapy. In contrast, those with widespread lesions receive chemotherapy and hematopoietic stem cell transplantation.     

A case of POEMS syndrome with high concentrations of interleukin-6 in pericardial fluid

The POEMS syndrome (plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy of various forms, monoclonal gammopathy, skin changes) is a rare multisystem disorder of unknown pathogenesis. Overexpression of proinflammatory cytokines has been implicated in the pathogenesis of POEMS syndrome, however, it is not known whether there is an association between abnormalities in cytokines and pericardial fluid. We present a case of POEMS syndrome with high concentrations of interleukin-6 (IL-6) in pericardial fluid. In our patient, pericarditis developed into cardiac tamponade, and the concentration of IL-6 in pericardial fluid was remarkably elevated compared with that in serum (1760 vs. 6.57 pg mL(-1)). We suggest that IL-6 is associated with the progression or maintenance of pericarditis as a result of POEMS syndrome.