Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a low prevalence multisystemic paraneoplastic disease. The name is an acronym composed by its most relevant clinical manifestations, which are polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. More than 95% of the POEMS syndrome cases are monoclonal for lambda light chains; however, few cases have been reported in the literature with a biclonal component. In this paper, we report a rare case of a patient who has POEMS syndrome with biclonal gammopathy. To the best of our knowledge, this is the first reported case in the literature of POEMS syndrome with expression of IgG kappa/IgG lambda biclonal gammopathy.
The 8p11 myeloproliferative syndrome (EMS) is an aggressive neoplasm associated with chromosomal translocations involving the fibroblast growth factor receptor 1 tyrosine kinase gene on chromosome 8p11. We report the case of a 31-year-old man with no prior medical history who presents with two weeks of sore throat and cervical lymphadenopathy up to 5 cm. Initial peripheral blood examination showed leukocytosis with predominantly neutrophils and eosinophilia. A CT scan demonstrated mediastinal lymphadenopathies, liver enlargement and splenomegaly. An excisional biopsy of a cervical lymph node demonstrated findings consistent with a diagnosis of T-cell lymphoblastic lymphoma. Bone marrow aspirate and biopsy revealed hypercellular marrow with granulocytic predominance, left-shifted granulopoiesis, eosinophilia and the cytogenetic analysis showed the following karyotype: 46, XY, t(8;13). The final diagnosis was a myeloproliferative syndrome with eosinophilia related to t(8;13) and T-cell acute lymphoblastic lymphoma (8p11 myeloproliferative syndrome). We review the relevant literature about this unusual entity.
Los abscesos asépticos constituyen una entidad en descripción que se ha asociado en la mayoría de los casos a enfermedad inflamatoria intestinal (EII), principalmente enfermedad de Crohn. Su diagnóstico constituye un reto pues exige descartar exhaustivamente infecciones por diversos microorganismos; además porque su aparición puede darse antes, concomitantemente o después del diagnóstico de la EII. A continuación, presentamos el caso de un paciente de 20 años con pérdida de peso, fiebre persistente, diarrea y dolor abdominal en quien se documentó abscesos esplénicos asépticos y lesiones en piel compatibles con enfermedad de Crohn metastásica y hallazgos inflamatorios inespecíficos en biopsia de tracto gastrointestinal, logrando resolución de todos sus síntomas tras inicio de esteroide
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