Ixazomib-Based Regimen Followed by Autologous Hematopoietic Stem Cell Transplantation in the Treatment of POEMS Syndrome / 中国实验血液学杂志

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.

The Clinical Characteristics and Outcomes of the Patients with POEMS Syndrome / 中国实验血液学杂志

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.

Sindrome de POEMS: a propósito de un caso con doble patrón monoclonal / POEMS Syndrome: a case with double monoclonal gammopathy

El Síndrome de POEMS es una patología sistémica poco frecuente de origen paraneoplásico. Se caracteriza por la presencia de una polineuropatía sensitivo-motora y otras manifestaciones como órganomegalia, endocrinopatía, gammapatía monoclonal y lesiones de piel. Se describe un paciente de 47 años que cumple con los criterios diagnósticos de la enfermedad, con la particularidad de presentar un doble patrón monoclonal sérico, IgG e IgA, Lambda, confirmado mediante electrofocalización e inmunofijación.

POEMS Syndrome is an uncommon systemic paraneoplastic disorder characerized by the presence of a sensitive-motor polineuropathy with other manifestations like organo-megalia, endocrinopathy, monoclonal gammopathy and skin changes. We report a case of a 47 years old male with diagnostic criteria for this disorder, with the singularity of presenting a double monoclonal pattern in serum, IgG and IgA class Lambda type, confirmed by isoelectric focusing and immunofixation

Síndrome POEMS: reporte de un caso clínico / POEMS syndrome: report of a clinical case

El síndrome POEMS se caracteriza por la presencia de Polineuropatía, Organomegalia, Endocrinopatía, Gammapatía Monoclonal y Cambios en la Piel. Muchas otras manifestaciones clínicas del síndrome no están incluidas en este acrónimo, como las lesiones óseas osteoescleróticas, edema de papila, sobrecarga de volumen extravascular, trombocitosis, policitemia, acropaquia, hipertensión pulmonar, baja de peso, fatiga, diarrea, compromiso renal y artralgias. Es una patología infrecuente, de mayor prevalencia en varones, con una relación 2:1, preferentemente entre la quinta y sexta década de la vida. Es una enfermedad de curso crónico y tiene sobrevida promedio de 13,8 años en algunas series. La patogenia es desconocida y no existe en la actualidad consenso respecto al tratamiento ni estudios randomizados y controlados que comparen los resultados de diferentes terapias en esta enfermedad.

POEMS syndrome is characterized by the presence of Polyneuropathy, Organomegaly, Endocrinopathies, Monoclonal Gammopathy and Skin changes. Many other clinical manifestations of the syndrome are not included in this acronym, as the osteosclerotic lesions, papillary edema, extravascular volume overload, thrombocytosis, polycythemia, acropaquia, pulmonary hypertension, weight loss, fatigue, diarrhea, arthralgia, and renal involvement. It is an infrequent disease, more prevalent in males with a 2:1 ratio, mostly between the fifth and sixth decades of life. It has a chronic course with an average survival of 13.8 years in some series. The pathogenesis is unknown and currently there is no consensus regarding the treatment or randomized controlled studies comparing the outcomes of different therapies in this disease.

Síndrome POEMS (Polineuropatia, Organomegalia, Endocrinopatia,Proteína M e Alterações da Pele): relato de caso / POEMS Syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M Protein eSkin Changes): case report

JUSTIFICATIVA E OBJETIVOS: O objetivo deste estu­do foi apresentar um relato de caso pouco comum. RELATO DO CASO: Paciente do sexo feminino com diagnóstico de polirradiculoneuropatia inflamatória crônica (PDIC) havia cinco anos; há um ano evoluindo com perda progressiva da visão e peso e, há cerca de seis meses, necessitou punções liquóricas de repetição para alívio de sintomas de hipertensão intracraniana, sem etiologia defi­nida. Ao exame físico detectou-se hiperfonese de 2a bulha, hepatoesplenomegalia e hiperpigmentação da pele, principalmente da palma das mãos e planta dos pés. Foi con­siderada a hipótese de síndrome de POEMS, confirmada após laudo de mielograma, evidenciando predomínio de plasmócitos e presença linhagem monoclonal, com gamo­patia por IgA. CONCLUSÃO: Este relato apresentou um caso de sín­drome de POEMS rico em manifestações típícas e atípi­caso Um dos diagnósticos diferenciais mais importantes é a polineuropatia inflamatória desmielizante crônica. A distinção entre as duas doenças foí fundamental para a adequada escolha do tratamento

POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, skin lesions) syndrome: a South America's report

The POEMS syndrome, also known as Crow-Fukase syndrome, is an unusual systemic disorder described mainly in Asian individuals. It is characterized by the presence of (P)polyneuropathy, (O)organomegaly, (E)endocrinopathy, (M) M-protein, and (S) skin changes. Several other associated condictions such as sclerotic bone lesions, Castleman disease, low-grade fever, edema and hematologic disorders are usually seen. We describe five Brazilian patients with this syndrome. Two patients presented Castleman disease, one patient presented osteosclerotic myeloma and in two patients no associated conditions were found.

A síndrome POEMS, também conhecida como síndrome de Crow-Fukase é uma desordem sistêmica rara descrita principalmente em asiáticos. Ela é caracterizada pela presença de (P) polineuropatia, (O) organomegalia, (E) endocrinopatia, (M) proteína M e (S) alterações de pele. Diversas outras manifestações, tais como lesões osteoescleróticas, doença de Castleman, febre baixa, edema e distúrbios hematológicos são freqüentemente observados. Apresentamos cinco pacientes brasileiros com esta síndrome. Dois pacientes apresentaram diagnóstico de doença de Castleman, um paciente com mieloma osteoesclerótico e em dois pacientes, nenhuma condição associada foi encontrada.

Síndrome de Poems: caso clínico / Poems syndrome: report of one case

Poems syndrome (polyneuropathy, organomegaly, endocrine abnormality, M-protein, plasma cell dyscrasia, and skin lesions) is a plasma cell dyscrasia whose pathogenesis is unknown. We report a 60 years old woman that presented a tetraparesis caused by a sensitive motor polyneuropathy. The subsequent work up revealed an IgA type monoclonal gammopathy, hepatomegaly, hyperestrogenism, primary adrenal failure, hypothyroidism, hyperpigmentation and erythematous lesions in the skin and ankle edema. The patient is being treated with prednisone 0.5 mg/kg and levothyroxine 100 Ág/day, with partial remission of her symptoms.

Síndrome Poems: relato de um caso brasileiro com evoluçäo de curso prolongado / Poems Syndrome: a brazilian case record with prolongued course evolution

Os autores relatam o caso de paciente do sexo masculino, de 58 anos, com polineuropatia, organomegalia, alterações endócrinas e dermatológicas e diversas lesões osteoescleróticas ao exame radiológico. Alterações no mielograma e na eletroforese de proteínas foram compatíveis com discrasia plasmocitária. Foi realizado o diagnóstico de Síndrome POEMS. A revisão do assunto mostra que o paciente apresentava vários dos critérios clínicos e laboratoriais utilizados para o diagnóstico desta síndrome. A suspeita diagnóstica de síndrome POEMS deve ser lembrada em todos os casos de polineuropatia inexplicada

Sindrome de Poems / Poems syndrome

El síndrome de P.O.E.M.S es un raro trastorno multisistémico. Se manifesta como Polinueropatía, Organomegalía, Endocrinopatía y/o Edemas, proteína Monoclonal y cambios en la piel. (Skin changes). P.O.E.M.S. Aunque alguma bibliografía lo equipara al mieloma osteoesclerótico se trata de un verdadero síndrome. La polineuropatía es habitualmente severa. Pese al hallazgo de inmunoglobulinas elevadas en el poems, no se ha aislado un anticuerpo específico que explique la polineuritis si bien es fuertemente sospechado. La organomegalia, endocrinopatía, cambios en la piel y otros sistemas y órganos involucrados podría estar en relación a productos secretados por las células plasmáticas. Se revisó la patofisiología y la bibliografía del Poems especialmente en su expresión neurológica y su ubicación nosológica diferente del mieloma osteosclerótico y una posible relación con el Herpes Vírus 8. Se cruzó en Medline los términos Poems y síndrome y se obtuvieron 271 abstracts que fueron todos examinados y finalmente selecionados los que se consideraron significativos para los objetivos. Se presenta brevemente un caso. El P.O.E.M.S. es un síndrome que está asociado a las discracias de células plasmáticas, incluindo el mieloma osteosclerótico. El pronóstico y el tratamiento varían con la enfermedad subyacente. Se insinúa como fisiopatología de este síndrome la acción de las interleukinas 1-Beta (IL-1 beta) y 6 (IL-6), el factor de crecimiento endotelial, el factor de necrosis tumoral alfa (TNF-alfa) y los anticuerpos anti-nervio.

Síndrome de Poems

Una forma poco común de discrasia de células plasmáticas asociada a polineuropatía, organomegalia, endocrinopatía, proteína monoclonal y cambios de piel ha recibido el nombre de POEMS. Informamos un caso de una paciente con mieloma múltiple asociado a amiloidosis y hallazgos clínicos de polineuropatía, hepatoesplenomegalia, intolerancia a los carbohidratos, pico monoclonal e hiperpigmentación que configuran el diagnóstico del síndrome de POEMS. Es el primer caso descrito en nuestro país y el cuarto en Latinoamérica.