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Case Reports in Nephrology 2022A 60-year-old woman with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome and intractable ascites presented with...
A 60-year-old woman with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome and intractable ascites presented with acute renal failure and received hemodialysis (HD) therapy. Due to frequent intradialytic hypotension, ultrafiltration with cell-free and concentrated ascites reinfusion therapy (CART) was performed to adequately manage the body fluid status and massive ascites. During HD with CART, her blood pressure was maintained compared with that during HD without CART, and an ultrafiltration volume of 3.7 L was achieved after HD with CART. In HD patients with intradialytic hypotension and massive ascites, the combination of CART and ultrafiltration during HD may be an effective therapeutic option for body-fluid management.
PubMed: 36284565
DOI: 10.1155/2022/7099227 -
Journal of the Peripheral Nervous... Dec 2022Patient-reported outcome measures engage patients in disease severity measurement and the metrics reported can be meaningful to their lives. The Polyneuropathy,...
Patient-reported outcome measures engage patients in disease severity measurement and the metrics reported can be meaningful to their lives. The Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes syndrome (POEMS) is a complex multisystem disorder with disabling neuropathy which is distinct from other acquired inflammatory neuropathies. No current POEMS-specific validated disability scales exist. To address this, we have produced a Rasch-built overall disability scale (RODS) specific to POEMS. A 146-item preliminary questionnaire containing relevant activity and participation items for neuropathic disability was applied to 49 clinically stable patients with POEMS from the UK national POEMS cohort. A total of 123 items not fulfilling Rasch model expectations were sequentially removed. The final 23-item POEMS-RODS fulfilled Rasch model expectations and showed acceptable test-retest reliability. The 23-item POEMS-RODS is a disease-specific patient-reported outcome measure able to detect activity limitations within the range of ability demonstrated by the UK POEMS cohort. Larger international studies are needed to confirm the broader applicability and responsiveness of this scale in other countries.
Topics: Humans; POEMS Syndrome; Reproducibility of Results; Disabled Persons; Surveys and Questionnaires; Severity of Illness Index
PubMed: 36190435
DOI: 10.1111/jns.12516 -
Radiology Case Reports Nov 2022POEMS syndrome is a rare paraneoplastic syndrome associated with a plasma cell proliferative disorder. Gallbladder adenocarcinoma is a rare malignancy, with no...
POEMS syndrome is a rare paraneoplastic syndrome associated with a plasma cell proliferative disorder. Gallbladder adenocarcinoma is a rare malignancy, with no association with POEMS syndrome. The plasma cell dyscrasia is routinely evaluated with advanced hybrid imaging to assess both anatomic and functional components. We present a case of a 59-year-old female with a known diagnosis of POEMS syndrome who underwent a whole-body restaging evaluation with hybrid positron emission tomography (PET) and magnetic resonance imaging (MR) to restage her plasma cell dyscrasia. She also had a prior diagnosis of gallbladder adenocarcinoma. Our case focuses on the value of PET/MR in this scenario as well as a rare case of osseous metastasis from gallbladder carcinoma.
PubMed: 36132063
DOI: 10.1016/j.radcr.2022.08.059 -
JCI Insight Oct 2022POEMS syndrome is a rare monoclonal plasma cell disorder, with unique symptoms distinct from those of other plasma cell neoplasms, including high serum VEGF levels....
POEMS syndrome is a rare monoclonal plasma cell disorder, with unique symptoms distinct from those of other plasma cell neoplasms, including high serum VEGF levels. Because the prospective isolation of POEMS clones has not yet been successful, their real nature remains unclear. Herein, we performed single-cell RNA-Seq of BM plasma cells from patients with POEMS syndrome and identified POEMS clones that had Ig λ light chain (IGL) sequences (IGLV1-36, -40, -44, and -47) with amino acid changes specific to POEMS syndrome. The proportions of POEMS clones in plasma cells were markedly smaller than in patients with multiple myeloma (MM) and patients with monoclonal gammopathy of undetermined significance (MGUS). Single-cell transcriptomes revealed that POEMS clones were CD19+, CD138+, and MHC class IIlo, which allowed for their prospective isolation. POEMS clones expressed significantly lower levels of c-MYC and CCND1 than MM clones, accounting for their small size. VEGF mRNA was not upregulated in POEMS clones, directly indicating that VEGF is not produced by POEMS clones. These results reveal unique features of POEMS clones and enhance our understanding of the pathogenesis of POEMS syndrome.
Topics: Humans; POEMS Syndrome; Plasma Cells; Vascular Endothelial Growth Factor A; Single-Cell Analysis; Immunoglobulin lambda-Chains; Immunoglobulin Light Chains; Clone Cells; Multiple Myeloma; Amino Acids
PubMed: 36129760
DOI: 10.1172/jci.insight.151482 -
EJHaem Aug 2022POEMS syndrome is a rareparaneoplastic disorder driven by an underlying low level plasma cell dyscrasiaand associated with elevated serum vascular endothelial growth...
POEMS syndrome is a rareparaneoplastic disorder driven by an underlying low level plasma cell dyscrasiaand associated with elevated serum vascular endothelial growth factor (VEGF). Dueto its rarity, there are no internationally agreed standards of care, with verylimited data to guide management in the relapse setting. Agents used in myelomaare rational choices and have been employed. Daratumumab has been reported intwo case studies with lenalidomide-dexamethasone, one in the upfront and one inthe relapsed setting. We are the first to report here three cases ofdaratumumab-bortezomib-dexamethasone (DVd) use in relapsed POEMS postautologous stem cell transplant with good VEGF and clinical responses. Our casesadd to the literature on efficacy of daratumumab and are the first to report onits safe use with bortezomib in relapsed POEMS. It should be considered as aclinical option, in patients not responding to conventional first linetherapies.
PubMed: 36051015
DOI: 10.1002/jha2.492 -
Internal Medicine (Tokyo, Japan) 2022Objective Immunomodulatory drugs and proteasome inhibitors are therapeutic options for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin...
Objective Immunomodulatory drugs and proteasome inhibitors are therapeutic options for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. This study aimed to evaluate the efficacy and safety of the combination of ixazomib, lenalidomide, and dexamethasone (IRd) for POEMS syndrome. Methods Six consecutive patients with POEMS syndrome who were treated with the IRd regimen at Chiba University Hospital between April 2018 and August 2021 were included. Serum M-protein and serum vascular endothelial growth factor (sVEGF) levels, overall neuropathy limitation scales (ONLS), clinical symptoms, and adverse events were assessed. Results Of the six patients, five had received prior treatments. Patients received a median of 5 cycles (range, 3-28 cycles) of IRd. Following treatment, serum M-protein disappeared in two patients, sVEGF levels returned to normal in two patients, two patients showed a reduction in the ONLS of 1, and clinical symptoms improved in four patients. The median level of sVEGF decreased from 2,395 pg/mL (range, 802-6,120 pg/mL) to 1,428 pg/mL (range, 183-3,680 pg/mL) in three months. Adverse events, including rash, neutropenia, sensory peripheral neuropathy, and nausea, were observed in three patients, which necessitated dose reduction or discontinuation of treatment. Conclusion IRd can be a therapeutic option for POEMS syndrome, albeit with careful monitoring of adverse events.
Topics: Boron Compounds; Dexamethasone; Endocrine System Diseases; Glycine; Humans; Lenalidomide; Monoclonal Gammopathy of Undetermined Significance; POEMS Syndrome; Vascular Endothelial Growth Factor A
PubMed: 36047093
DOI: 10.2169/internalmedicine.8786-21 -
Cureus Jul 2022Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein elevation, and skin changes (POEMS) syndrome is a rare multisystem disorder that occurs due to an...
Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein elevation, and skin changes (POEMS) syndrome is a rare multisystem disorder that occurs due to an underlying plasma cell dyscrasia. A diagnosis is made with the presence of two mandatory criteria and at least one major and one minor criterion. We present a case of a 28-year-old patient who presented with weakness of bilateral arms and legs, thinning of hands, and swelling of bilateral lower limbs and abdomen. The patient also reported weight loss and loss of appetite. Examination revealed areflexic quadriparesis with sensory loss, diffuse lymphadenopathy, pleural effusion, ascites, and pulmonary hypertension. Investigations showed elevated erythrocyte sedimentation rate (ESR). Nerve conduction studies revealed severe axonal polyneuropathy of all nerves. Lymph node biopsy showed Castleman disease. A diagnosis of POEMS syndrome was made and he was sent for a stem cell transplant, which is the definitive treatment in patients eligible for stem cell transplant.
PubMed: 35989837
DOI: 10.7759/cureus.27001 -
Journal of Medical Case Reports Aug 2022Transplant-eligible patients with polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes syndrome are treated with induction therapy and autologous stem...
Successful treatment with daratumumab, lenalidomide, and dexamethasone therapy followed by autologous stem cell transplantation for newly diagnosed polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes syndrome: a case report.
BACKGROUND
Transplant-eligible patients with polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes syndrome are treated with induction therapy and autologous stem cell transplantation. Conventional induction therapies may exacerbate neuropathy and a high rate of disease progression within 5 years. Furthermore, only 50% of patients are able to walk independently after the therapies. Daratumumab, lenalidomide, and dexamethasone therapy has been reported as a less neurotoxic, highly effective therapy for patients with polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes syndrome who are ineligible for transplant or whose syndrome is relapsed/refractory, but no reports have provided data from untreated transplant-eligible patients.
CASE PRESENTATION
A 34-year-old Japanese woman displayed weakness, pain and edema in the lower limbs, decreased grip strength, amenorrhea, and abdominal distention. She was unable to walk independently. The patient was diagnosed with polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes syndrome and performed four courses of daratumumab, lenalidomide, and dexamethasone therapy, which enabled her to walk independently and did not exacerbate the neuropathy. Hematopoietic stem cells were collected using plerixafor and filgrastim in combination. Autologous stem cell transplantation was performed with high-dose melphalan. At 3-month post-transplantation follow-up, most of her clinical symptoms had disappeared.
CONCLUSIONS
Daratumumab, lenalidomide, and dexamethasone therapy followed by autologous stem cell transplantation may be more effective than conventional therapy for newly diagnosed polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes syndrome. Although there was concerns that daratumumab, lenalidomide, and dexamethasone therapy might lead to poor mobilization of hematopoietic stem cells, this was overcome with the combination of plerixafor and filgrastim. The benefit of daratumumab, lenalidomide, and dexamethasone as induction therapy prior to autologous stem cell transplantation should be confirmed in future clinical trials.
Topics: Adult; Antibodies, Monoclonal; Dexamethasone; Endocrine System Diseases; Female; Filgrastim; Hematopoietic Stem Cell Mobilization; Hematopoietic Stem Cell Transplantation; Heterocyclic Compounds; Humans; Lenalidomide; Polyneuropathies; Transplantation, Autologous
PubMed: 35978379
DOI: 10.1186/s13256-022-03552-y -
Indian Journal of Clinical Biochemistry... Jul 2022Polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes (POEMS) syndrome is a rare multisystem paraneoplastic disorder. Here we describe a case of a...
Polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes (POEMS) syndrome is a rare multisystem paraneoplastic disorder. Here we describe a case of a 50 year old post-menopausal female who presented with chief complaints of difficulty in walking, getting up from squatting position and tingling sensation of bilateral lower limbs since 1 month. Additional unusual features in the patient included hepatosplenomegaly, endocrinal involvement in the form of hypothyroidism and elevated estradiol levels for her age. There were skin changes in the form of hyperpigmentation. M protein was not noted on serum electrophoresis but was visible on serum protein immunofixation (IgA lambda). She was investigated as a case of polyneuropathy and later a provisional clinical diagnosis of POEMS was made based on the presence of major and minor criteria. The patient was managed with methyl prednisolone, calcium carbonate and vit D3 and topical antibiotics for local infections.
PubMed: 35873606
DOI: 10.1007/s12291-020-00940-3 -
American Journal of Translational... 2022POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is a kind of plasma cell disease with complex clinical manifestations involving...
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is a kind of plasma cell disease with complex clinical manifestations involving multiple systems. Metal poisonings through a mucocutaneous are rare in clinic and reported less in the literature. People may exposure to toxic metals through air, food, water, or inappropriate use of drugs. Acute or chronic poisonings can lead to various toxic effects on body tissues and organs. Both POEMS syndrome and heavy metal intoxication are uncommon with multifarious and nonspecific clinical manifestations. Here we describe a case of a 54-year-old man with polyarticular pain and IgA lambda type monoclonal protein in his serum. The diagnosis was confirmed by heavy metals testing in his urine and the herbal mixtures he took. This is the first available report of arsenic and mercury intoxication mimicking POEMS syndrome.
PubMed: 35836872
DOI: No ID Found