Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, And Skin Research Articles

Progressive Limb Weakness in A Young Man: A Case Report of POEMS Syndrome

Polyneuropathy is an initial presentation and essential feature of POEMS ( polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome. Neuropathy is typically distal, symmetric and slowly progressive with demyelinating changes. After a gradual proximal spread, it usually results in severe muscle weakness and functional disabilities. In the present report, we describe a 40-year-old diabetic male presented with gradually progressive weakness of both lower limbs for 1 year followed by the involvement of both upper limbs for the last 3 months. On examination hyperpigmentation, lymphadenopathy, gynecomastia, anasarca, hepatomegaly, bilateral optic disc swelling, sensory-motor polyneuropathy was found. Laboratory findings showed IgG lambda monoclonal gammopathy, raised VEGF, sensory-motor demyelinating and axonal polyneuropathy. All findings were consistent with POEMS syndrome. The patient was treated with lenalidomide and dexamethasone cyclical therapy with some clinical improvement.
 Bangladesh Journal of Neuroscience 2019; Vol. 35 (1): 51-56

Poems

POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome is a rare cause of demyelinating polyneuropathy with plasma cell dyscrasia, overproduction of vascular endothelial growth factor (VEGF), and multiorgan involvement. VEGF is likely to play an important role in the pathogenesis of the disorder by its strong action on neo-vasculization and increased vascular permeability. POEMS syndrome is serious disease, because of progressive neuropathy and massive pleural effusion/ascites. There is no established treatment guideline. However, in appropriate candidates, high-dose chemotherapies with autologous peripheral blood stem cell transplantation results in obvious clinical improvement with a significant decrease in serum VEGF levels. For patients who are not indicated for transplantation, a recent randomized trials has shown efficacy of thalidomide. Treatments that should be considered as future therapy include lenalidomide and bortezomib or other proteasome inhibitors. The efficacy of anti-VEGF monoclonal antibody (bevacizumab).is controversial. By induction of these novel treatments after 2000, the prognosis of POEMS syndrome has been significantly improved.

POEMS syndrome: a challenging diagnosis of a rare disease

British Journal of Hospital MedicineVol. 75, No. 7 Case ReportPOEMS syndrome: a challenging diagnosis of a rare diseaseA Creamer, B Gaastra, S Odogwu, BC WhitelawA CreamerSearch for more papers by this author, B GaastraSearch for more papers by this author, S OdogwuSearch for more papers by this author, BC WhitelawSearch for more papers by this authorA Creamer; B Gaastra; S Odogwu; BC WhitelawPublished Online:7 Jul 2014https://doi.org/10.12968/hmed.2014.75.7.408AboutSectionsView articleView Full TextPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareShare onFacebookTwitterLinked InEmail View article References Dispenzieri A, Kyle RA, Lacy MQ et al. (2003) POEMS syndrome: definitions and long-term outcome. Blood 101(7): 2496–506 Crossref, Medline, Google ScholarDispenzieri A (2007) POEMS syndrome. Blood Rev 21(6): 285–99 Crossref, Medline, Google ScholarFukatsu A, Ito Y, Yuzawa Y, Yoshida F, Kato M, Miyakawa K, Matsuo S (1992) A case of POEMS syndrome showing elevated serum interleukin 6 and abnormal expression of interleukin 6 in the kidney. Nephron 62(1): 47–51 Crossref, Medline, Google ScholarKuwabara S, Dispenzieri A, Arimura K, Misawa S, Nakaseko C (2012) Treatment for POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome. Cochrane Database Syst Rev 6: CD006828 Medline, Google ScholarLi J, Zhou DB (2013) New advances in the diagnosis and treatment of POEMS syndrome. Br J Haematol 161(3): 303–15 Crossref, Medline, Google ScholarScarlato M, Previtali SC, Carpo M et al. (2005) Polyneuropathy in POEMS syndrome: role of angiogenic factors in the pathogenesis. Brain 128(Pt 8): 1911–20 Crossref, Medline, Google ScholarSoubrier M, Dubost JJ, Serre AF et al. (1997) Growth factors in POEMS syndrome: evidence for a marked increase in circulating vascular endothelial growth factor. Arthritis Rheum 40(4): 786–7 Crossref, Medline, Google Scholar FiguresReferencesRelatedDetails 2 July 2014Volume 75Issue 7ISSN (print): 1750-8460ISSN (online): 1759-7390 Metrics History Published online 7 July 2014 Published in print 2 July 2014 Information© MA Healthcare LtdPDF download

Treatment for POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome.

POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome is a rare cause of demyelinating and axonal mixed neuropathy with monoclonal plasma cell proliferative disorder and multiorgan involvement. The pathogenesis of POEMS syndrome is not well understood, but overproduction of vascular endothelial growth factor (VEGF), probably secreted by plasmacytomas, is likely to be responsible for most of the characteristic symptoms. POEMS syndrome is a potentially fatal disease, and patients' quality of life deteriorates because of progressive neuropathy, massive pleural effusion or ascites, or thromboembolic events. There is a need for efficacious therapy to improve prognosis. This is the first update of a review first published in 2008. To assess the effects of treatment for POEMS syndrome. We searched the Cochrane Neuromuscular Disease Group Specialized Register (23 February 2012), CENTRAL (2012, Issue 2), MEDLINE (January 1966 to February 2012), EMBASE (January 1980 to February 2012) and CINAHL Plus (January 1937 to February 2012) for all papers on POEMS syndrome We sought all randomized and quasi-randomized controlled trials, and non-randomized controlled studies. Since we discovered no such clinical trials, we assessed and summarized all retrospective case series including five or more patients in the 'Discussion' section. Two review authors independently reviewed and extracted details of all potentially relevant trials with any treatment for POEMS syndrome. We then collated and summarized information on the outcome. We found no randomized or non-randomized prospective controlled trials of treatment for POEMS syndrome. We summarized the results of retrospective case series containing five or more patients in the 'Discussion' section. There are no randomized or quasi-randomized controlled clinical trials of treatment for POEMS syndrome on which to base practice.

POEMS Syndrome Diagnosed 10 Years after Disabling Peripheral Neuropathy

Peripheral neuropathy is characterized as a generalized, relatively homogeneous process affecting many peripheral nerves and predominantly affecting distal nerves. The epidemiology of peripheral neuropathy is limited since the disease presents with varying etiology, pathology, and severity. Toxic, inflammatory, hereditary, and infectious factors can cause damage to the peripheral nerves resulting in peripheral neuropathy. Peripheral neuropathy is most commonly caused by diabetes, alcohol, HIV infection, and malignancy. We report a case of a 42-year-old female with 10-year history of progressively worsening peripheral neuropathy, hypothyroidism, and skin changes who presents with dyspnea secondary to recurrent pleural and pericardial effusions. Prior to her arrival, her peripheral neuropathy was believed to be secondary to chronic demyelinating inflammatory polyneuropathy (CDIP) given elevated protein in the cerebral spinal fluid (CSF) which was treated with intravenous immunoglobulin (IVIG) and corticosteroids. Unfortunately, her peripheral neuropathy did not have any improvement. Incidentally, patient was found to have splenomegaly and papilledema on physical exam. Serum protein electrophoresis showed a monoclonal pattern of IgA lambda. Patient met the diagnostic criteria for POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome. An underlying diagnosis of POEMS syndrome should be considered in patients with chronic debilitating neuropathy and an elevated protein in the CSF.

Detection of Bone Lesions by CT in POEMS Syndrome

To study the utility of CT for detection of small bone lesions in POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome. For patients with a solitary bone lesion, irradiation is a first-line treatment, whereas systemic chemotherapy is indicated for patients with multiple bone lesions. Therefore it is important to correctly identify the number of bone lesions. We studied the sensitivity of chest/abdomen/pelvic CT to detect bone lesions in 28 patients with POEMS syndrome. (99m)Tc-HMDP bone scintigraphy was performed in 14 patients, and the results were compared with CT. CT showed multiple bone lesions in 68% of the 28 patients, and 71% of the lesions had a diameter <10 mm. In 14 patients who underwent both CT and scintigraphy, bone lesions were detected in 57% by CT and in 79% by scintigraphy, but the location and nature of the identified lesions were considerably different; CT frequently showed small lesions (diameter <10 mm) in the vertebrae and pelvis, which were not detected by scintigraphy, whereas scintigraphy could show lesions in the skull and long bones. Overall, by using both examinations, multiple bone lesions were found for 86% of patients. CT is particularly useful to detect small bone lesions. CT and bone scintigraphy are complementary, and therefore both should be performed for bone survey in POEMS syndrome.

POEMS syndrome: cryoglobulinemia an unusual association

Associations between plasma cell dyscrasia and peripheral neuropathy were well recognized as early as the 1950s. In 1980, Bardwick coined the acronym POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes). We report a 56-year-old woman who presented with excessive sweating, lethargy, intermittent clawing of fingers and painful limb spasms for 2 years. Clinical examination showed hyperpigmented skin lesions, bilateral pleural effusions and organomegaly. Relevant investigations revealed the presence of sensorimotor polyneuropathy, raised cerebrospinal fluid protein, IgM monoclonal band on serum protein electrophoresis, and reactive bone marrow. The patient was treated with combination therapy which included cyclophosphamide, dexamethasone, and intravenous immunoglobulin with moderate response. Presence of cryoglobulinemia in this case has not been described in the literature.

Coexistence of primary AL amyloidosis and POEMS syndrome: Efficacy of melphalan‐dexamethasone and role of biochemical markers in monitoring the diseases course

American Journal of HematologyVolume 85, Issue 2 p. 131-132 LetterFree Access Coexistence of primary AL amyloidosis and POEMS syndrome: Efficacy of melphalan-dexamethasone and role of biochemical markers in monitoring the diseases course† Fausto Adami, Corresponding Author Fausto Adami [email protected] Department of Clinical and Experimental Medicine, Hematology and Clinical Immunology Section, Padova University School of Medicine, Padova, ItalyDepartment of Clinical and Experimental Medicine, Hematology and Clinical Immunology Section, Padova University School of Medicine, Via Giustiniani 2, Padova 35128, ItalySearch for more papers by this authorChiara Briani, Chiara Briani Department of Neurosciences, Padova University School of Medicine, Padova, ItalySearch for more papers by this authorGianni Binotto, Gianni Binotto Department of Clinical and Experimental Medicine, Hematology and Clinical Immunology Section, Padova University School of Medicine, Padova, ItalySearch for more papers by this authorSara Altinier, Sara Altinier Department of Laboratory Medicine, Padova University School of Medicine, Padova, ItalySearch for more papers by this authorGiuseppe Tarantini, Giuseppe Tarantini Department of Cardiac, Thoracic and Vascular Sciences, Padova University School of Medicine, Padova, ItalySearch for more papers by this authorGianpietro Semenzato, Gianpietro Semenzato Department of Clinical and Experimental Medicine, Hematology and Clinical Immunology Section, Padova University School of Medicine, Padova, ItalySearch for more papers by this author Fausto Adami, Corresponding Author Fausto Adami [email protected] Department of Clinical and Experimental Medicine, Hematology and Clinical Immunology Section, Padova University School of Medicine, Padova, ItalyDepartment of Clinical and Experimental Medicine, Hematology and Clinical Immunology Section, Padova University School of Medicine, Via Giustiniani 2, Padova 35128, ItalySearch for more papers by this authorChiara Briani, Chiara Briani Department of Neurosciences, Padova University School of Medicine, Padova, ItalySearch for more papers by this authorGianni Binotto, Gianni Binotto Department of Clinical and Experimental Medicine, Hematology and Clinical Immunology Section, Padova University School of Medicine, Padova, ItalySearch for more papers by this authorSara Altinier, Sara Altinier Department of Laboratory Medicine, Padova University School of Medicine, Padova, ItalySearch for more papers by this authorGiuseppe Tarantini, Giuseppe Tarantini Department of Cardiac, Thoracic and Vascular Sciences, Padova University School of Medicine, Padova, ItalySearch for more papers by this authorGianpietro Semenzato, Gianpietro Semenzato Department of Clinical and Experimental Medicine, Hematology and Clinical Immunology Section, Padova University School of Medicine, Padova, ItalySearch for more papers by this author First published: 29 October 2009 https://doi.org/10.1002/ajh.21581Citations: 3 † Conflict of interest: Nothing to report. AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat References 1 Kuwabara S. New strategy of treatment for POEMS syndrome: Autologous peripheral blood stem cell transplantation and thalidomide therapy. Brain Nerve 2008; 60: 627– 633. 2 Dispenzieri A. POEMS syndrome. Blood Rev 2007; 21: 285– 299. 3 Kuwabara S,Dispenzieri A,Arimura K, et al. Treatment for POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome. Cochrane Database Syst Rev 2008; 4: CD006828. 4 Dispenzieri A,Kyle R,Merlini G, et al. International Myeloma Working Group guidelines for serum-free light chain analysis in multiple myeloma and related disorders. Leukemia 2009; 23: 215– 224. 5 Iwasaki T,Hamano T,Ogata A, et al. Clinical significance of vascular endothelial growth factor and hepatocyte growth factor in multiple myeloma. Br J Hematol 2002; 116: 796– 802. 6 Soubrier M,Dubost JJ,Serre AF, et al. Growth factors in POEMS syndrome: Evidence for a marked increase in circulating vascular endothelial growth factor. Arthritis Rheum 1997; 40: 786– 787. 7 Scarlato M,Previtali SC,Carpo M, et al. Polyneuropathy in POEMS syndrome: Role of angiogenic factors in the pathogenesis. Brain 2005; 128: 1911– 1920. 8 Nobile-Orazio E,Terenghi F,Giannotta C, et al. Serum VEGF levels in POEMS syndrome and in immune-mediated neuropathies. Neurology 2009; 72: 1024– 1026. 9 Patier JL,Pinilla A,Rodriguez-Posada A, et al. Primary amyloidosis associated with osteosclerotic myeloma. Rev Clin Esp 2003; 203: 162– 163. 10 Briani C,Scarlato M,Pavan A, et al. Polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes: Not always a POEMS syndrome. Hematologica 2006; 91: ECR10. 11 Watanabe O,Maruyama I,Arimura K, et al. Overproduction of vascular endothelial growth factor/vascular permeability factor is causative in Crow-Fukase (POEMS) syndrome. Muscle Nerve 1998; 21: 1390– 1397. 12 Merlini G,Stone MJ. Dangerous small B-cell clones. Blood 2006; 108: 2520– 2530. 13 Palladini G,Perfetti V,Obici L, et al. Association of melphalan and high-dose dexamethasone is effective and well tolerated in patients with AL (primary) amyloidosis who are ineligible for stem cell transplantation. Blood 2004; 103: 2936– 2938. Citing Literature Volume85, Issue2February 2010Pages 131-132 ReferencesRelatedInformation

Diagnostic value of serum peptidome analyses for protease activated pathological conditions beyond cancer diagnosis

Human serum contains thousands of proteolytically derived low-molecular-weight peptide fragments (serum peptidome). The concept of utilizing the serum peptidome for cancer diagnosis has been developed. A pathological serum peptidome appears when the homeostatic balance between proteases and protease inhibitors is disrupted. We hypothesize if analyses of the serum peptidome are of diagnostic value as information on which molecules are disrupted, and the pathological course it will take in unknown pathological conditions and disseminated intravascular coagulation (DIC). We analyzed the serum peptidome in 3 stages (early stage, pre-DIC and DIC stages) in one patient with POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) syndrome, an intractable disease with unknown pathology, using a 1-dimensional gel electrophoresis/matrix-assisted laser desorption/ionization-mass spectrometry (1-DE/MS)-based rapid quantitative approach. A very large number of peptide fragments appeared in the DIC stage, compared to pre-DIC. In addition, we identified fragments of transthyretin (ALGISPFHEHAEVVFTANDSGPR, m/z 2451.18) and alpha1-antitrypsin (EDPQGDAAQKTDTSHHDQDHPTFN, m/z 2691.02) that significantly increased in the DIC stage, compared to those in the pre-DIC stage. Rapid analyses of the serum peptidome may lead to a diagnostic method that can predict on-going protease activated pathological conditions and help to decide on multilateral strategies including nutritional support and drug therapy.

Activity of bortezomib (B) in plasma cell dyscrasia (PCD) with POEMS-like syndrome

e19564 Background: POEMS (polyneuropathy, organomegaly, endocrinopathy, m-protein, and skin changes) syndrome is a rare multi-system disorder associated with PCD. Although several reports revealed significant clinical benefit of IMIDs in POEMS syndrome cases that are not transplant candidates, no such published reports of B in this disease were found. Methods: We report the first case of PCD with POEMS like syndrome in addition to pulmonary hypertension, secondary right heart failure, and membranoproliferative glomerulonephritis that was successfully treated with single agent B. After presenting with the aforementioned signs and symptoms, a 64 year old white man, was diagnosed with PCD (bone marrow plasma cells 15%, IgG-λ, mild-moderate anemia, normal PET/CT, normal Cr and Ca) with ISS stage III. Due to being high risk for thromboembolic events, he was started on single agent B at 1.3mg/m2 days 1,4,8,11 Q21 days (cycles 1–4) which was reduced to 1.0mg/m2 days 1,4,8,11 Q21 days (cycles 5–6) due to grade 1 thrombocytopenia and neuropathy. This was followed by 3 maintenance cycles of 4 out of 5 weekly 1.0mg/m2. Results: Patient's breathing was subjectively better after cycle#1 despite experiencing side-effects from his pulmonary hypertension medications which had to be held and subsequently improved back to his baseline by cycle#4. By cycle#2, he was found to be in nCR with complete normalization of his bone marrow and serum electrophoresis by the end of therapy. He has been in nCR for almost 12 months so far. By cycle#3 his Raynaud's syndrome was significantly better with less dysesthesia which completely resolved by cycle#6. Conclusions: This case demonstrates for the first time the significant activity of B as a single agent in PCD with POEMS like syndrome. Further evaluation of the role of B as a first line agent in this disease, whether as single agent or in combination, is warranted. [Table: see text]

Multi-modal visual evoked potentials in a case of POEMS syndrome

Electrophysiological studies of visual evoked potentials (VEPs) in a case of POEMS (polyneuropathy, organomegaly, endocrinopa-thy, M-protein, and skin changes) syndrome were performed. The subject, a 58-year-old male, had bilateral optic disc swelling. Arden's score showed sensitivity losses at high spatial frequencies. In the short and middle latency VEPs elicited by flash and pattern reversal stimulation, the peak latencies of the major components of the short latency VEPs were within normal range, but those of the middle latency VEPs were delayed. Based on these results, the visual conduction process was normal in this case, but there might be some abnormalities in the cortical processing of the visual integration mechanism.