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2.
Journal of Experimental Hematology ; (6): 1645-1648, 2021.
Article in Chinese | WPRIM | ID: wpr-922309

ABSTRACT

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.


Subject(s)
Aged , Aged, 80 and over , Hematopoietic Stem Cell Transplantation , Humans , Lenalidomide , Middle Aged , POEMS Syndrome/therapy , Retrospective Studies , Transplantation, Autologous
3.
J. Health Biol. Sci. (Online) ; 8(1): 1-4, 20200101. ilus
Article in Portuguese | LILACS | ID: biblio-1123718

ABSTRACT

Introdução: Síndrome de POEMS consiste na desordem paraneoplásica rara, por displasia celular plasmática, caracterizada, principalmente, por neuropatia. Relato de Caso: Paciente com síndrome de POEMS, sexo masculino, 56 anos, deambulando com o auxílio de andador, com incapacidade de caminhar por longas distâncias. Foi submetido à avaliação de espirometria, baropodometria e Teste do Índice do Andar Dinâmico (DGI) e SF-36, e, posteriormente, ao protocolo de intervenção para alongamento, força e equilíbrio. A avaliação pós-intervenção demonstrou aumento de força muscular e velocidade de marcha. Conclusão: As intervenções com exercícios físicos têm-se mostrado eficazes na melhora do equilíbrio e da qualidade de vida.


Introduction: POEMS syndrome consists of a rare paraneoplastic disorder, due to plasma cell dysplasia, characterized mainly by neuropathy. Case Report: Male patient with POEMS syndrome, 56 years old, walking with the help of a walker, unable to walk long distances. He was submitted to the evaluation of spirometry, baropodometry and Dynamic Floor Index Test (DGI) and SF-36, and, subsequently to the intervention protocol for stretching, strength and balance. The post-intervention evaluation showed an increase in muscle strength and gait speed. Conclusion: Physical exercise measures have proven effective in improving balance and quality of life.


Subject(s)
Exercise , POEMS Syndrome , Quality of Life
4.
Article in Chinese | WPRIM | ID: wpr-827175

ABSTRACT

OBJECTIVE@#To investigated the clinical and pathological characteristics of related-renal damage in patients with POEMS syndrome.@*METHODS@#Five patients diagnosed as POEMS syndrome in our hospital were selected. Their clinical manifestation, pathological characteristics of kidney and laboratory examination were analyzed retrospectively. Among the 5 patients, three males and two females with a median age of 50 years old. The mean interval before diagnosis was 13.0±7.2 months.@*RESULTS@#All the patients showed neuropathy, endocrinopathy, monoclonal plasma cell-proliferative disorder, skin changes and extravascular volume overload, in which 4 patients showed organomegaly. Proteinuria was found in 5 patients, and microhematuria was found in 4 patients. Moreover, 4 patients showed an elevated blood urea, while 2 patients showed creatinine elevation. 1 patient at chronic kidney disease (CKD)-G1 stage, 2 patients at CKD-G2 stage, and 1 patient at CKD-G3b stage, moreover, 1 patient at CKD-G5 stage. Endothelial injury and mesangial lesion were the main characteristics of renal pathology. 3 patients were pathologically diagnosed as thrombotic microangiopathy kidney damage, while 2 patients as light chain amyloidosis.@*CONCLUSION@#POEMS syndrome is a multi-systemic disease with complex clinical manifestations. 5 patients had different degrees of renal insufficiency. Endothelial injury and mesangial lesion are the main features of renal pathology.


Subject(s)
Female , Humans , Kidney , Male , Middle Aged , POEMS Syndrome , Paraproteinemias , Renal Insufficiency , Retrospective Studies
5.
Rev. fac. cienc. méd. (Impr.) ; 16(2): 31-34, jul.- dic. 2019. ilus
Article in Spanish | LILACS | ID: biblio-1140249

ABSTRACT

El síndrome de POEMS se caracteriza por polineuropatía, organomegalia, endocrinopatia, proteína M, y alteraciones cutáneas, también incluye papiledema, sobrecarga de volumen extravascular, trombocitosis/eritrocitosis, niveles elevados de factor de crecimiento endotelial vascular, una predisposición hacia la trombosis y está incorporado en el grupo de las gammapatias monoclonales. Se diagnostica en base a una combinación de clínica y de hallazgos laboratoriales. Objetivo: describir un síndrome clínico poco frecuente, las dificultades diagnósticas, así como diferenciarlo de otras polineuropatías crónicas. Presentación del caso clínico: femenina de 52 años, con hiperpigmentacion de piel, disminución de agudeza visual bilateral y debilidad distal progresiva con arreflexia y síntomas sensitivos asociados, organomegalia, hipotiroidismo; se comprobó polineuropatía crónica en estudios de neuroconducción, trastornos hematológicos, pérdida de peso y niveles bajos de vitamina B12, cumpliendo la mayoría de los criterios del síndrome, aunque no se encontró trastorno monoclonal, la literatura resalta normal en un 25% de los casos la electroforesis de proteínas. Conclusión: Es una enfermedad poco frecuente, para su diagnóstico se requiere una alta sospecha clínica, como hallazgos insulares edema de papila y niveles bajos de vitamina B12, descritos en la literatura. Debido a la diversidad en la forma de presentación del síndrome es necesario estar alerta sobre las múltiples manifestaciones y la búsqueda clínica, analítica y radiológica debe ser dirigida...(AU)


Subject(s)
Humans , Female , Middle Aged , Polyneuropathies/complications , POEMS Syndrome/diagnosis , Papilledema
6.
Article in English | WPRIM | ID: wpr-813246

ABSTRACT

POEMS syndrome is a rare paraneoplastic disorder. A 60-year-old female patient was admitted to the Department of Gastroenterology, Xiangya Hospital of Central South University (Changsha, China), complaining of abdominal distension, severe edema of both lower limbs and shortness of breath for more than 1 year. After intensive and careful medical investigations, the patient manifested with polyneuropathy, M-proteinemia, splenomegaly, lymphadenopathy, hypothyroidism, extravascular volume overload, sclerotic bone lesions, elevated VEGF and pulmonary hypertension. According to the latest diagnostic criteria of POEMS syndrome, this patient met two mandatory major criteria, two other major criteria and three minor criteria, the diagnosis was clear after ruling out differential diagnosis. The patient was treated with dexamethasone and lenalidomide, which relieved her clinical symptoms. The pathogenesis of POEMS syndrome is not fully understood; however, increased levels of vascular endothelial growth factor may contribute to most of the clinical manifestations. This patient had been in physical discomfort for more than 14 months, which seriously affected her quality of life. Clinically, the awareness of early diagnosis and treatment of POEMS syndrome should be improved.


Subject(s)
China , Dyspnea , Edema , Female , Humans , Lower Extremity , Middle Aged , POEMS Syndrome , Quality of Life , Vascular Endothelial Growth Factor A
7.
Article in English | WPRIM | ID: wpr-762438

ABSTRACT

POEMS syndrome is a rare paraneoplastic syndrome, which includes polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes due to plasma cell (PC) neoplasm. Diagnosis of this disease is challenging because of its rarity and complex clinical manifestations. We attempted to identify the key clinical features and characteristic bone marrow (BM) findings of POEMS syndrome, by reviewing the medical records and BM analyses of 24 Korean patients. Frequent clinical manifestations included polyneuropathy (100%), monoclonal gammopathy (100%), organomegaly (92%), extravascular volume overload (79%), and endocrinopathy (63%). The BM analyses revealed mild PC hyperplasia (median PCs: 5.5%) and frequent megakaryocytic hyperplasia (88%), megakaryocyte clusters (88%), and hyperlobation (100%). Flow cytometry of BM aspirates using CD138/CD38/CD45/CD19/CD56 showed normal (67%, 4/6) or neoplastic PC immunophenotypes (33%, 2/6). A diagnosis of POEMS syndrome must be considered when a patient suspected of having PC dyscrasia shows the above clinical presentation and BM findings.


Subject(s)
Bone Marrow , Diagnosis , Flow Cytometry , Humans , Hyperplasia , Medical Records , Megakaryocytes , Paraneoplastic Syndromes , Paraproteinemias , Plasma Cells , POEMS Syndrome , Polyneuropathies , Skin
8.
Acta bioquím. clín. latinoam ; 52(1): 43-48, mar. 2018. ilus, tab
Article in Spanish | LILACS | ID: biblio-886159

ABSTRACT

Se reporta el caso de un hombre de 45 años con síntomas y signos consistentes con el Sindrome de POEMS (del inglés: polineuropatía, organomegalia, endocrinopatía, gammapatía monoclonal y cambios dérmicos), un raro desorden paraneoplásico. El mismo contaba con antecedentes de tabaquismo, hipotiroidismo y últimamente había perdido 20 kg de peso. Se destaca que una historia clínica y revisión detallada seguida de estudios de laboratorio, radiología y biopsia de médula ósea, entre otros, son herramientas necesarias para reconocer los componentes de este síndrome y no demorar el diagnóstico. El paciente presentó 2 criterios obligatorios (gammapatía monoclonal y neuropatia periférica sensitivo-motora), un criterio mayor (lesión ósea) y varios criterios menores (desórdenes endocrinos, manifestaciones cutáneas, organomegalia). Actualmente se encuentra bajo supervisión hematológica y continúa su seguimiento neurológico, lo que muestra una buena respuesta a la terapia específica. Las enfermedades raras como este síndrome resultan un desafío diagnóstico para los profesionales de la salud.


The case of a 45 - year- old- man whose symptoms and signs were consistent with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes), a rare paraneoplastic disorder, has been reported. He had a previous history of smoking, hypothyroidism and a 20 kg weight loss. It was emphasized that a detailed clinical history and examination followed by laboratory and radiological studies and bone marrow biopsy, among others tests, were necessary in order to recognise the components of this syndrome and not to delay the time of diagnosis. This patient had two mandatory criteria (monoclonal gammopathy and sensorimotor polyneuropathy), one major criterion (bone lesion) and several minor criteria (endocrine disorders, cutaneous manifestations, organomegaly). He is currently under the supervision of the doctors of the hematology department and continues neurological follow-up, having a good response to the specific therapy. Rare diseases like this syndrome are a diagnostic challenge for health professionals.


Informa-se o caso de um homem de 45 anos com sintomas e sinais compatíveis com a Síndrome de POEMS (do inglês: polineuropatia, organomegalia, endocrinopatia, gamopatia monoclonal e alterações cutâneas), um distúrbio paraneoplásico raro. O homem tinha antecedentes de tabagismo, hipotiroidismo e ultimamente tinha perdido 20 kg de peso. Enfatizamos que um prontuario médico e exame detalhado, seguido de estudos de laboratório e radiológicos, e uma biópsia de medula óssea, dentre outros, são ferramentas necessárias para reconhecer os componentes desta síndrome e não demorar o tempo de diagnóstico. Nosso paciente apresentou dois critérios obrigatórios (gamopatia monoclonal e neuropatia periférica sensório-motora), um critério maior (lesão óssea) e vários critérios menores (anormalidades endócrinas, alterações cutâneas, organomegalia). Encontra-se atualmente sob supervisão hematológica e continua seu seguimento neurológico, mostrando uma resposta boa à tera,pia concreta. Doenças raras como essa síndrome são um desafio diagnóstico para os profissionais da saúde.


Subject(s)
Humans , Male , Middle Aged , Endocrine System Diseases , Paraproteinemias , POEMS Syndrome/diagnosis , Polyneuropathies , Case Reports , Paraproteinemias , POEMS Syndrome , Skin Abnormalities , Syndrome
9.
Article in Korean | WPRIM | ID: wpr-766714

ABSTRACT

Ischemic stroke caused by the cerebral vasculopathy is a rare complication of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. We present a case of recurrent ischemic strokes caused by cerebral vasculopathy in a patient with POEMS syndrome. A 34-year-old man presented with gait disturbance and dizziness. Brain magnetic resonance imaging demonstrated acute ischemic stroke in the middle cerebral artery-anterior cerebral artery (MCA-ACA) border zones of bilateral hemispheres. Repeated angiographic studies showed progressive worsening of the left distal internal carotid artery, ACA, and MCA stenoses, along with sustained steno-occlusion of right MCA.


Subject(s)
Adult , Brain , Carotid Artery, Internal , Cerebral Arteries , Constriction, Pathologic , Dizziness , Gait , Humans , Magnetic Resonance Imaging , Paraproteinemias , POEMS Syndrome , Polyneuropathies , Skin , Stroke , Vascular Diseases
10.
Journal of Experimental Hematology ; (6): 1225-1229, 2018.
Article in Chinese | WPRIM | ID: wpr-689501

ABSTRACT

POEMS syndrome is a rare multiple organ involvement of the parasympathetic syndrome associated with abnormal plasma cells, mostly with high-dose chemotherapy and stem cell transplantation for the treatment. Recently, more treatment attempts to treat POEMS syndrome have been utilized so as to improve the efficacy and safety for the patients with POEMS syndrome, such as immunomodulator, alkylating agent, cytokine-induced killer cells and so on. Lenalidomide has a significant effect on relapse/refractory POEMS syndrome and patients with endocrinopathy. Cytokine-induced killer cells are also a safe and effective regimen for the treatment of POEMS syndrome. This review described the efficacy and safety of immunomodulatos, alkylators, cytokine-induced killer cells, ASCT, proteasome inhibitors and monoclonal antibodies for POEMS syndrome, and the newest clinical research and progress of POEMS syndrome ware summarized briefly.


Subject(s)
Cytokine-Induced Killer Cells , Humans , Immunologic Factors , POEMS Syndrome , Stem Cell Transplantation , Transplantation, Autologous
11.
Article in English | WPRIM | ID: wpr-119353

ABSTRACT

No abstract available.


Subject(s)
POEMS Syndrome , Stroke
13.
Rev. méd. Chile ; 144(4): 516-520, abr. 2016. ilus
Article in Spanish | LILACS | ID: lil-787124

ABSTRACT

POEMS syndrome is characterized by Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes. We report a woman with the syndrome, who had peripheral polyneuropathy, osteosclerotic myeloma, monoclonal IgA elevation, hypothyroidism, hypogonadotrophic hypogonadism, hyperprolactinemia, adrenal insufficiency, hepatosplenomegaly, lymphadenopathy, thyroid and parotid enlargement, Castleman’s disease, papilledema, stiff and hyperpigmented skin, white nails, clubbing, ascites and chronic diarrhea. She had also a nephropathy characterized by microscopic hematuria, proteinuria, renal insufficiency and a unilateral kidney retraction. She was treated with melphalan and prednisone, achieving remission of the disease and nephropathy. She survived twelve years and died due to a myocardial infarction 20 years after POEMS diagnosis.


Subject(s)
Humans , Female , Adult , POEMS Syndrome/complications , Renal Insufficiency, Chronic/ethnology , POEMS Syndrome/physiopathology , POEMS Syndrome/pathology , Fatal Outcome , Disease Progression , Renal Insufficiency, Chronic/physiopathology , Renal Insufficiency, Chronic/pathology
14.
Article in English | WPRIM | ID: wpr-145686

ABSTRACT

POEMS syndrome is a rare paraneoplastic syndrome and there are few reports of polyneuropathy and monoclonal gammopathy associated with kidney dysfunction. Here, we report a case of POEMS syndrome with recurrent acute kidney injury (AKI). A 52-year-old man presented with bilateral aggravating paresthesia and latermotor weakness of the lower extremities accompanied by repeated elevation of serum creatinine. The patient was finally diagnosed with POEMS syndrome on the basis of fulfilling the two mandatory major criteria (polyneuropathy and monoclonal gammopathy), one other major criterion (sclerotic bone lesion), and several minor criteria. A renal biopsy was performed to clarify the cause of AKI and showed membranoproliferative glomerulonephritis-like lesions with mesangiolysis and endothelial cell injury. This case illustrates that renal manifestations, not included in the diagnostic criteria for POEMS, can be apparent before various other systemic symptoms.


Subject(s)
Acute Kidney Injury , Biopsy , Creatinine , Endothelial Cells , Humans , Kidney , Lower Extremity , Middle Aged , Paraneoplastic Syndromes , Paraproteinemias , Paresthesia , POEMS Syndrome , Polyneuropathies
15.
Medicina (B.Aires) ; 75(5): 324-327, Oct. 2015. tab
Article in Spanish | LILACS | ID: biblio-841522

ABSTRACT

Se presentan cuatro casos de un raro síndrome paraneoplásico de una discrasia de células plamáticas compuesto por polineuropatía, organomegalia, endocrinopatía, banda monoclonal y lesiones cutáneas (POEMS). El objetivo de la comunicación de esta serie es alertar sobre diferentes formas de presentación del síndrome de POEMS para disminuir el tiempo de diagnóstico, ya que el tratamiento temprano reduce las secuelas y mejora la calidad de vida a largo plazo; también, señalar la importancia de la clasificación de la enfermedad hematológica para realizar el tratamiento específico.


Four cases of a rare paraneoplastic syndrome associated to a plasmatic cell disorder with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin lesions (POEMS) are here reported. The purpose of the communication is to warn of different forms of presentation of POEMS syndrome to decrease the time of diagnosis, because early treatment reduces sequels and improves quality of life in the long term, also to remark the importance of classifying the hematological disease for specific treatment.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , POEMS Syndrome/diagnosis , POEMS Syndrome/drug therapy , Glucocorticoids/therapeutic use , Fatal Outcome , Edema
18.
Journal of Experimental Hematology ; (6): 1165-1167, 2015.
Article in Chinese | WPRIM | ID: wpr-274073

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the morphological manifestation of bone marrow cells in newly-diagnosed patients with POEMS syndrome.</p><p><b>METHODS</b>The bone marrow cells in 155 patients with POEMS syndrome were classified and counted by OLYMPUS BX51 microscope, and the abnormal morphology of bone marrow cells was observed.</p><p><b>RESULTS</b>The count of plasma cells with normal morphology was 83.9% (130/155), the count of plasma cells with abnormal morphology (< 5 percent) was 12.3% (19/155), the count of plasma cells with obvious abnormal morphology (> 10 percent) was 3.8% (6/155) in patients with POEMS syndrome.</p><p><b>CONCLUSION</b>The morphology of plasma cells in the most patients with the POEMS syndrome are normal, the minor patients of the POEMS syndrome have little abnormal plasma cell morphology, the extremely few patients showed obvious morphological abnormality in the bone marrow plasma cells. The higher proportion of plasma cells, the more easily and more abnormal plasma cells will be found.</p>


Subject(s)
Bone Marrow Cells , Humans , POEMS Syndrome , Plasma Cells
19.
Article in Korean | WPRIM | ID: wpr-213783

ABSTRACT

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a monoclonal plasma cell disorder. Patients with POEMS syndrome also have various clinical manifestations including generalized edema, pleural effusion, ascites, papilledema, and sclerotic bone lesions. These manifestations can lead to a misdiagnosis or delayed diagnosis. We recently experienced a 51-year-old male patient with POEMS syndrome whose sclerotic bone lesion was misdiagnosed as malignant bone metastasis of papillary thyroid carcinoma. We reassessed the patient and found polyneuropathy, hepatosplenomegaly, hypothyroidism, partial hypopituitarism, immunoglobulin G lambda-type monoclonal gammopathy, hypertrichosis, ascites, and multiple sclerotic bone lesions, all of which led us to a diagnosis of POEMS syndrome. Treatment with thalidomide and dexamethasone resulted in clinical and radiological improvement. The patient has remained in remission after peripheral blood stem cell transplantation.


Subject(s)
Ascites , Delayed Diagnosis , Dexamethasone , Diagnosis , Diagnostic Errors , Edema , Humans , Hypertrichosis , Hypopituitarism , Hypothyroidism , Immunoglobulin G , Male , Middle Aged , Neoplasm Metastasis , Papilledema , Paraproteinemias , Peripheral Blood Stem Cell Transplantation , Plasma Cells , Pleural Effusion , POEMS Syndrome , Polyneuropathies , Skin , Thalidomide , Thyroid Gland , Thyroid Neoplasms
20.
Chinese Journal of Hematology ; (12): 1065-1068, 2014.
Article in Chinese | WPRIM | ID: wpr-278945

ABSTRACT

<p><b>OBJECTIVE</b>To evaluate the diagnostic performance and clinical correlation of serum vascular endothelial growth factor (VEGF) in patients with newly diagnosed POEMS syndrome.</p><p><b>METHODS</b>We recruited 104 patients with POEMS syndrome who diagnosed at Peking Union Medical College Hospital during October 2010 to April 2014 in the current study. Clinical data and serum samples were collected. Serum VEGF levels were measured, comparing to both disease and healthy controls, and its correlation with clinical features were analyzed.</p><p><b>RESULTS</b>Patients with newly diagnosed POEMS syndrome showed markedly elevated levels of serum VEGF (median 3503 ng/L, P<0.01). A cut-off value of 1 200 ng/L had a specificity of 90.2%, with a sensitivity of 83.7%, in support of a POEMS diagnosis. Further analyses indicated that patients with higher serum VEGF level (>2 000 ng/L) had more prevalent osteosclerosis (61.0% vs 37.0%, P=0.031) and papilledema (70.1% vs 44.4%, P=0.017).</p><p><b>CONCLUSION</b>Serum VEGF level was useful in differentiating POEMS syndrome from other disorders with overlapping clinical presentations, and its levels correlated with several disease features.</p>


Subject(s)
Humans , POEMS Syndrome , Vascular Endothelial Growth Factor A
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