Renal disease in POEMS syndrome: report on a case and review of the literature

POEMS syndrome is a multisystem disorder associated with plasmacell dyscrasias. This report describes a patient with POEMS-associatedrenal disease and reviews the literature on biopsy-proven renalinvolvement in POEMS syndrome. Our patient had glomerulonephritiswith membranoproliferative features on light-microscopy withoutcharacteristic findings on immunofluorescence, and with ultra-structural evidence of glomerular microangiopathy. Ultrastructuralevidence of microangiopathy was also found in vasa nervorum.In 20 other cases of POEMS- associated renal disease, 16 hadglomerular disease. Light-microscopy showed membranoproliferative-likeglomerulopathy in 14 patients and glomerular microan giopathyin two. Ultrastructural evidence of microangi opathy was presentin all 15 patients in whom electron- microscopy was done. Thus,in most patients with POEMS-associated glomerular disease acharacteristic lesion is present with evidence of endothelialinjury. As endothelial damage is also found in endoneural vessels,generalized endothelial injury may play a role in non-renalmanifestations of POEMS syndrome. In previous reviews manifestationsof the POEMS syndrome were similar for patients with or withoutmyeloma. Among patients with biopsy-proven glomerular disease,however, myeloma patients are underrepres ented. Whether thisrepresents a sampling error or has true pathophysiological significanceremains to be established.     

Imaging spectrum in sclerotic myelomas: an experience of three cases

The classic radiographic presentation of multiple myeloma is lytic skeletal lesions. Primary sclerotic manifestations are rare and occur only in 3 % of cases. The imaging spectrum in three cases of multiple myeloma with primary osteosclerosis is described. The first patient had spiculated sclerosis of the orbit, which is an uncommon site for myeloma. The second patient with POEMS syndrome had multiple, scattered, skeletal lesions with sclerotic margins. The third patient presented with a chest wall mass and had an expansile thick spiculated sclerosis in the rib. The wide imaging spectrum possible in sclerotic myelomas and their relevant differential diagnosis is emphasized. Received: 14 March 2000; Accepted: 25 April 2000     

Association of osteonecrosis of the jaws and POEMS syndrome in a patient assuming rituximab

POEMS syndrome, is a rare condition characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal proteinaemia, and skin lesions.We report a rare case of a patient affected by Waldenström macroglobulinemia, who developed POEMS syndrome and who presented at the time of diagnosis with oral manifestations of the lymphoma and an osteonecrosis of the jaw (ONJ) after rituximab treatment.Although the etiology of ONJ is not known, it is likely that several factors are at play, including endothelial cell damage, decreased angiogenesis, and microvascular compromise. Our patient was treated with rituximab for a long period, and recent studies have demonstrated the possibility that rituximab, a monoclonal antibody directed against the CD20 can exert part of its anti-tumor action, through its action on angiogenesis.Although our report does not allow identification of rituximab as a new risk factor for the onset of the ONJ, further studies seem necessary to exclude a role of the antibody in the alterations of angiogenesis that could lead to the development of the syndrome after rituximab treatment.     

Efficacité à moyen terme de l’autogreffe de cellules souches hématopoïétiques périphériques sur la polyneuropathie du syndrome POEMS : expérience chez 5 cas

Introduction

POEMS syndrome (polyneuropathy, organomegaly, endocrynopathy, M-protein, and skin changes) is a rare multisystem disease associated with plasma cell dyscrasia. The efficacy of autologous peripheral blood stem cell transplantation (auto-PBSCT) reported in case series has been mainly based on hematologic criteria and clinical recovery of peripheral neuropathy dysfunctions but has not been specifically evaluated. This retrospective study aimed to analyze the efficacy of auto-PBSCT on disability and electrophysiological patterns in patients with POEMS syndrome.

Methods

Five patients presenting with POEMS syndrome received auto-PBSCT. Disability was evaluated before treatment and at 6 and 12 months using the Overall Neuropathy Limitation Scale (ONLS) and MRC sumscore of 28 muscles. Nerve conduction studies were performed before and one year after treatment, on median, ulnar, fibular and tibial nerves.

Results

Mean age was 60.6 years (49–70). Disease duration between first symptoms and auto-PBSCT was 15.4 months (2–33). Before auto-PBSCT, mean ONLS score was 4.2 (1–10) and mean MRC sumscore 115.8/140 (74–140). At M6, mean ONLS score decreased and mean MRC sumscore increased; both were improved in all patients at M12: mean ONLS score 3 (range 0–8) at M6 and 2.2 (range 0–7) at M12; mean MRC sumscore 118/140 (77–140) at M6 and 122.4/140 (80–140) at M12. Significant recovery in electrophysiological patterns was observed in all patients on ulnar and median nerves: before-after treatment differences were observed for motor conduction velocities (34.41 vs. 45.47 m/s; P < 0.001), distal CMAP amplitudes (5.04 vs. 5.96 mV; P = 0.004), and sensory conduction velocities (43.20 vs. 49.20 m/s; P = 0.001). Distal CMAP amplitude remained low in fibular and tibial nerves (0.41 vs. 0.17 mV).

Conclusions

Clinical and electrophysiological improvement is obvious in POEMS syndrome peripheral neuropathy within one year after treatment with auto-PBSCT, undoubtedly resulting from extensive remyelinisation and axonal regeneration. Further studies are required to examine long-term outcome in patients with POEMS syndrome given auto-PBSCT.     

硼替佐米皮下注射方案治疗3例POEMS综合征并文献复习

目的:探讨含硼替佐米方案治疗POEMS综合征的有效性和安全性。方法:报道3例POEMS综合征经含硼替佐米方案治疗的过程及结果,并对相关文献进行复习。结果:3例病人经化疗后1例神经症状得到明显改善,1例腹胀症状消失,1例神经症状及胸闷、气短症状改善。结论:含硼替佐米方案治疗POEMS综合征患者取得了较好疗效,未出现神经毒副作用。     

CT联合超声诊断POEMS综合征

目的探讨POEMS综合征CT和超声影像学表现特点,提高认识能力。方法选取4例经CT和超声诊断的POEMS综合征患者临床资料,分析影像学表现。结果 4例均为男性,CT表现4例腋窝、腹股沟淋巴结多发肿大,3例纵隔淋巴结肿大,增强扫描呈明显均匀强化,超声显示淋巴结内部及周围血流丰富;4例多浆膜腔积液;3例脊柱、骨盆、胸骨多发局限性骨质硬化;3例乳腺发育;3例肺动脉高压。结论骨质硬化性改变、多中心富血供淋巴结肿大、肺动脉高压在POEMS综合征有一定影像学特点,密切结合临床可做出正确诊断。     

POEMS综合征8例临床分析

目的探讨POEMS综合征发病机制及临床特征，提高对POEMS综合征的诊治水平。方法分析8例POEMS综舍征的一般资料、临床表现、相关的辅助检查、治疗及预后，并结舍文献进行讨论。结果本病是累及多系统的疾病，以周围神经病最具有特征性，肝、脾和（或）淋巴结肿大，内分泌的病变，皮肤改变，水肿及骨髓浆细胞增多比较常见。治疗以激素、免疫抑制剂为主，可结合抗雌激素、放疗的方法，预后差。结论POEMS综合征与浆细胞病关系密切，累及多系统的病变，临床表现复杂多样，应注意多系统的检查，提高临床的诊断水平。     

Revue de la littérature récente sur les neuropathies périphériques : actualités thérapeutiques

Improvement of therapeutic strategies for peripheral neuropathies requires multicentric clinical trials. For chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), a randomized controlled multicentric study compared IgIV to pulses of methylprednisolone (MP) given for 6 months. The primary endpoint was treatment discontinuation due to inefficacy or intolerance; 45 patients were enrolled: more patients had interrupted MP than IVIg, usually because of inefficacy. A multicentric randomized clinical trial (PREDICT) evaluated long-term remission of CIDP after short-term corticosteroid therapy (pulses of dexamethasone or prednisolone); 39 patients were enrolled: 26% achieved cure or remission, a relapse occurred in 50% after a delay of 11 to 17 months. Differential diagnosis was identified in 58% of patients who had not responded to any therapy. For refractory CIDP, a retrospective study showed the possibility of functional improvement in 24% of cases after adjunction of animmunomodulatory agent; cyclosporine was associated with the highest rate of adverse events or side effects. In familial amyloidotic polyneuropathy, a multicentric controlled study against placebo with tafamidis, an akinetic stabilizer of transthyretin (TTR) 20 mg/d, in early stage of Val30MetTTR showed efficiency in the evaluable group and led to marketing authorization by the EMA in stage 1 to slow the progression of the neuropathy. A Cochrane database system review showed that there are no randomized or quasi-randomized controlled clinical trials of treatment for POEMS syndrome, for neuropathies with anti-MAG antibodies, or multifocal motor neuropathy on which to base practice. This review underlines the usefulness of multicentric randomized trials to assess treatments in peripheral neuropathies.