Descripción general

El síndrome de POEMS es un trastorno poco frecuente de la sangre que daña los nervios y afecta a otras partes del cuerpo. POEMS es una sigla en inglés que comprende los siguientes signos y síntomas:

  • Polineuropatía. Entumecimiento, hormigueo y debilidad en las piernas (y, con el tiempo, tal vez en las manos) y dificultad para respirar. Esta es una característica esencial en el diagnóstico del síndrome de POEMS.
  • Organomegalia. Agrandamiento del bazo, del hígado o de los ganglios linfáticos.
  • Endocrinopatía o edema. Niveles hormonales anormales que pueden provocar baja actividad de la tiroides (hipotiroidismo), diabetes, problemas sexuales, fatiga, hinchazón de las extremidades y problemas con el metabolismo y otras funciones esenciales.
  • Proteína monoclonal. Células anormales de la médula ósea (células plasmáticas) que producen una proteína (proteína monoclonal) que puede encontrarse en el torrente sanguíneo. Esta es una característica esencial en el diagnóstico del síndrome de POEMS. La proteína monoclonal se suele asociar con un endurecimiento o engrosamiento inusual de los huesos.
  • Cambios en la piel. Tener más color de lo normal en la piel, manchas rojas, piel posiblemente más gruesa y un aumento del vello en el rostro o en las piernas.

Se desconocen las causas del síndrome de POEMS. Pero las personas con este síndrome poseen una mayor cantidad de células plasmáticas. Estas células producen una cantidad anormal de un tipo de proteína que puede dañar otras partes del cuerpo.

Es posible que se cometan errores en el diagnóstico del síndrome de POEMS porque los signos y síntomas son similares a los de otros trastornos. El síndrome de POEMS avanza rápidamente sin tratamiento y puede poner en riesgo la vida, por lo que el diagnóstico temprano es muy importante.

El tratamiento para el síndrome de POEMS puede mejorar los síntomas, pero no cura la enfermedad. Las opciones de tratamiento comprenden radioterapia, quimioterapia y el trasplante de células madre. El pronóstico para las personas con el síndrome de POEMS ha mejorado mucho en la última década.

Síntomas

The symptoms of POEMS syndrome make up its name. Symptoms most often appear over weeks to months. They include the following:

  • Polyneuropathy. This may include numbness, tingling and weakness in the legs. In time, it might affect the hands. It also involves trouble breathing. Polyneuropathy must be present for a POEMS diagnosis.
  • Organomegaly. This is an enlarged spleen, liver or lymph nodes.
  • Endocrinopathy and edema. Atypical hormone levels can cause an underactive thyroid, also called hypothyroidism. The hormone levels also can cause diabetes, sexual issues, tiredness, and swelling in arms and legs.
  • Monoclonal protein. This is a protein that atypical plasma cells in bone marrow make. The monoclonal protein goes into the bloodstream. This must be present for the diagnosis of POEMS syndrome. Monoclonal protein often is linked with bones getting harder or thicker.
  • Skin changes. This may involve having more color than usual on the skin. There may be red spots, which may be harder to see on Black or brown skin. The skin might be thicker. There might be more hair on the face or the legs.

Not all the symptoms of POEMS syndrome are in the name. And not everyone with POEMS syndrome has all the symptoms in the name. But everyone with POEMS syndrome has polyneuropathy and monoclonal protein.

Other symptoms some people with POEMS syndrome have spell out PEST. PEST stands for:

  • Papilledema. This is swelling in the back of the eyes.
  • Extravascular volume overload. This might include ankle swelling, fluid between the lung and the ribs, or fluid in the belly.
  • Sclerotic bone lesions. X-rays can show these bone spots. They're most often not painful.
  • Thrombocytosis and erythrocytosis. These are higher levels of red cells and platelets in the bloodstream.

When to see a doctor

Make an appointment with your healthcare professional if you have any of the symptoms of POEMS syndrome.

Causas

Experts don't know the cause of POEMS syndrome. But people with POEMS syndrome have a higher number of plasma cells. These cells make too much of a type of protein, called monoclonal protein. This protein can damage other parts of the body.

Factores de riesgo

Factors that raise the risk of POEMS syndrome include:

  • Older age. POEMS syndrome most often happens after age 50.
  • Male sex. People assigned male at birth are a bit more likely to get POEMS syndrome than are people assigned female at birth.
April 19, 2022

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  1. Rajkumar SV. POEMS syndrome. https://www.uptodate.com/contents/search. Accessed Feb. 8, 2020.
  2. AskMayoExpert. POEMS syndrome. Mayo Clinic; 2019.
  3. Cerri F, et al. An update on the diagnosis and management of the polyneuropathy of POEMS syndrome. Journal of Neurology. 2019; doi10.1007/s00415-018-9068-4.