Journal Information
Vol. 129. Issue 15.
Pages 578-581 (October 2007)
Vol. 129. Issue 15.
Pages 578-581 (October 2007)
DOI: 10.1157/13111711
Trasplante autólogo de progenitores hematopoyéticos en el síndrome de POEMS: resultados en 4 casos
Autologous haematopoietic stem cell transplantation in POEMS syndrome: results in 4 cases
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El síndrome de POEMS (polineuropatía, organomegalia, endocrinopatía, banda monoclonal y alteraciones cutáneas) es una entidad infrecuente, que se caracteriza por una proliferación clonal de células plasmáticas que da lugar a un componente monoclonal, por lo general poco abundante y con restricción de la cadena lambda. Clínicamente cursa con afectación multisistémica, dentro de la cual predomina la polineuropatía periférica. No existe un tratamiento específico para esta enfermedad. Se presentan 4 casos de síndrome de POEMS tratados mediante trasplante autólogo de progenitores hematopoyéticos en nuestro centro. Todos ellos mejoraron de sus manifestaciones neurológicas y sistémicas, al tiempo que presentaron una disminución o desaparición del componente monoclonal y de las lesiones óseas. En conclusión, el trasplante autólogo de progenitores hematopoyéticos constituye una opción terapéutica útil en el síndrome de POEMS.
Palabras clave:
Síndrome de POEMS
Trasplante autólogo de progenitores hematopoyéticos
POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes) syndrome is a rare disorder characterized by a clonal plasma cell proliferation with a monoclonal protein typically small and lambda restricted. Clinically, this syndrome is a multisystemic disease whose major clinical feature is a peripheral polyneuropathy. A specific treatment for this disease does not exist. We report 4 patients with POEMS syndrome treated with an autologous haematopoietic stem cell transplantation in our centre. All patients had an improvement in neurologic symptoms and other manifestations of the syndrome. The monoclonal component and bone lesions decreased or disappeared. In conclusion, autologous haematopoietic stem cell transplantation is an effective therapy in patients with POEMS syndrome.
Keywords:
POEMS syndrome
Autologous haematopoietic stem cell transplantation
Polyneuropathy
Plasma cell dyscrasia
Osteosclerotic myeloma
VEGF
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