Humans ; POEMS Syndrome ; diagnosis ; therapy

INTRODUCTIONPatients with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal or M-protein and skin changes) syndrome exhibit a wide range of clinical manifestations and are often seen by a variety of specialists prior to diagnosis.

CLINICAL PICTUREWe describe a case of POEMS syndrome that first presented with significant neuropathy but progressed to develop further manifestations of the condition, including marked gastrointestinal symptoms.

TREATMENTThe patient was commenced on localised radiotherapy and chemotherapy in addition to immunomodulatory therapy for the neuropathy.

CONCLUSIONWe highlight several learning points that may benefit physicians from varied specialties. This case is also unique for its marked gastrointestinal manifestation. To our knowledge, this is the second reported case in the literature with this feature.

Gastrointestinal Diseases ; etiology ; physiopathology ; Humans ; Immunologic Factors ; therapeutic use ; Male ; Middle Aged ; POEMS Syndrome ; complications ; drug therapy ; radiotherapy ; Treatment Outcome

OBJECTIVE: To analyze the clinical characteristics of patients with POEMS syndrome and explore its effective treatment strategies. METHODS: The clinical data of 75 patients with POEMS syndrome treated in The First Affiliated Hospital of Zhengzhou University from June 2012 to June 2018 were collected and retrospectively analyzed. The clinical characteristics, treatment regimes and outcomes of the patients were summarized. RESULTS: The median age of 75 diagnosed patients was 50 (30-81) years old and 100% (75/75) of the patients were accompanied with peripheral neuropathy, 77.3% (58/75) with organ enlargement, 82.7% (62/75) with endocrine abnormality, 93.3% (70/75) with monoclonal plasma cell diseases and 64.0% (48/75) with skin changes. Among the 75 patients, 5 cases gave up treatment, while the others showed varying degrees of improvement after treatment. The hematological complete remission (CR CONCLUSION The clinical manifestations of POEMS syndrome are complex and diverse, the clinicians therefore should be vigilant to reduce the misdiagnosis and missed diagnosis. Bortezomib or Lenalidomide can be recommended as the first-line medicines and autologous HSCT should be considered for appropriate patients.
Aged ; Aged, 80 and over ; Hematopoietic Stem Cell Transplantation ; Humans ; Lenalidomide ; Middle Aged ; POEMS Syndrome/therapy* ; Retrospective Studies ; Transplantation, Autologous

Adult ; Boronic Acids ; therapeutic use ; Bortezomib ; Combined Modality Therapy ; Female ; Hematopoietic Stem Cell Transplantation ; Humans ; POEMS Syndrome ; therapy ; Pyrazines ; therapeutic use

This study was purposed to analyze the clinical features and evaluate the efficacy of thalidomide combined with VAD regimen for treatment of osteosclerotic myeloma (POEMS syndrome). The data of 27 patients with POEMS syndrome in the First Affiliated Hospital of Zhengzhou University were analyzed retrospectively, including clinical manifestations, laboratory tests, treatments and prognosis. The results showed that the polyneuropathy was observed in 27 patients (27/27), hepato-spleno-lymphadenectasis was found in 15 patients (15/27), endocrinopathy was found in 24 patients (24/27), skin changes was observed in 22 patients (22/27). M protein was found in 23 patients (23/27); in addition to these clinical manifestations, the papilledema serous cavity effusion and sclerotic bone lesion were also frequently observed in patients with POEMS syndrome. The remission rates of treatment of POEMS syndrome with thalidomide combined with VAD regimen for organomegaly, edema, skin changes, and endocrinopathy were 60, 58.3, 41 and 45.8 respectively. The level of serum M protein and the nervous system ODSS value decreased greatly after treatment (P < 0.01). It is concluded that the clinical characteristics of POEMS syndrome are complicated and easy to be misdiagnosed, and the evidence of monoclonal plasma cell hyperplasia should be actively searched for those patients whose serum M protein is negative. Thalidomide combined with VAD regimen for treatment of patients with POEMS syndrome has advantages such as significant curative effects, less side-effects, good tolerance, and higher safety and can be chosen as a preferred approach.
Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; Cytarabine ; Dexamethasone ; Female ; Humans ; Male ; Middle Aged ; POEMS Syndrome ; drug therapy ; Retrospective Studies ; Thalidomide ; therapeutic use ; Vincristine

BACKGROUND: POEMS syndrome is a rare paraneoplastic syndrome associated with plasma cell dyscrasia. High-dose chemotherapy followed by autologous stem cell transplantation has shown encouraging efficacy in the treatment of patients with POEMS syndrome. However, there are minimal reports on clinical outcomes after autologous stem cell transplantation for patients with advanced disease and very poor performance status. METHODS: We retrospectively evaluated 9 advanced POEMS syndrome patients, who had an Eastern Cooperative Oncology Group performance status score of 3 or 4, and were treated with high-dose melphalan therapy followed by autologous stem cell transplantation from 2004 to 2011. RESULTS: Eight patients achieved initial hematologic response, 4 of whom had complete responses. At a median follow-up of 44 months (range, 8-94 months), 7 patients were alive, with 3-year overall survival rate of 77.8%. There were no hematologic relapses in the survivors. One patient died of disease progression; the other died of pneumonia despite a hematologic response 3 months after autologous stem cell transplantation. All survivors achieved improvement in general performance status and in clinical response. CONCLUSION: High-dose melphalan followed by autologous stem cell transplantation can be considered a valid treatment option even for patients with advanced POEMS syndrome.
Disease Progression ; Drug Therapy ; Follow-Up Studies ; Humans ; Melphalan* ; Paraneoplastic Syndromes ; Paraproteinemias ; Pneumonia ; POEMS Syndrome* ; Recurrence ; Retrospective Studies ; Stem Cell Transplantation* ; Stem Cells* ; Survival Rate ; Survivors

Boronic Acids ; therapeutic use ; Bortezomib ; Humans ; Male ; Middle Aged ; POEMS Syndrome ; therapy ; Peripheral Blood Stem Cell Transplantation ; Pyrazines ; therapeutic use ; Transplantation, Autologous

OBJECTIVE: To investigate the safety and efficacy of a new proteasome inhibitor Ixazomib followed by autologous hematopoietic stem cell transplantation (AHSCT) in the treatment of POEMS syndrome. METHODS: The clinical manifestations, diagnosis and treatment process and follow-up results of 4 patients with POEMS syndrome who were treated with Ixazomib-based regimen combined with AHSCT in Wuhan No.1 Hospital from February 2018 to July 2020 were analyzed retrospectively. All patients were male, aged from 37-54 years old, with varying degrees of peripheral neuropathy, organ enlargement (liver, spleen or lymph nodes), circulatory overload (peripheral edema and/or pleural effusion), osteosclerosis, endocrine diseases (thyroid, gonads, etc.), skin changes (pigmentation, hemangioma, white nails, etc.), M protein, papilledema and other clinical manifestations and characteristics at the time of initial treatment. Two patients were pathologically diagnosed as hyaline vascular Castleman disease by lymph node biopsy. Three patients underwent lumbar puncture examinations and all showed elevated cerebrospinal fluid protein. All patients received at least 2 cycles of sequential AHSCT after induction chemotherapy based on ixazomib. The follow-up time was 10-28 months, and the median follow-up time was 16 months. RESULTS: All cases survived. The complications were controllable during the treatment. Moreover, the clinical symptoms related to the disease were improved to a certain extent after the treatment. The levels of vascular endothelial growth factor (VEGF) showed a gradual decline. CONCLUSION Ixazomib combined with AHSCT is safe and effective in the treatment of POEMS syndrome.
Adult ; Boron Compounds ; Glycine/analogs & derivatives* ; Hematopoietic Stem Cell Transplantation ; Humans ; Male ; Middle Aged ; POEMS Syndrome/therapy* ; Retrospective Studies ; Transplantation, Autologous ; Vascular Endothelial Growth Factor A

Aged ; Anti-Inflammatory Agents ; therapeutic use ; Cyclophosphamide ; therapeutic use ; Female ; Humans ; Immunosuppressive Agents ; therapeutic use ; Methylprednisolone ; therapeutic use ; POEMS Syndrome ; complications ; diagnosis ; drug therapy ; pathology ; Plasma Cells ; pathology ; gamma-Globulins ; therapeutic use

No abstract available.
Angiogenesis Inhibitors/administration & dosage ; Bevacizumab/*administration & dosage ; Follow-Up Studies ; Humans ; Intravitreal Injections ; Male ; Middle Aged ; Optic Disk/*pathology ; POEMS Syndrome/*complications/diagnosis ; Papilledema/diagnosis/*drug therapy/etiology ; Tomography, Optical Coherence ; Vascular Endothelial Growth Factor A/*antagonists & inhibitors