[Lymphocytic variant of the hypereosinophilic syndrome]. / Variante linfoide del síndrome hipereosinófilo.

OBJECTIVES: Hypereosinophilic syndromes (HSS) are a rare group of heterogeneous disorders characterized by prominent and persistent eosinophilia and organ dysfunction. Secondary causes of eosinophilia must be excluded. Recent advances in molecular biology and cytogenetics have permitted the characterization of different subsets of hypereosinophilic syndrome. We describe a patient with the lymphocytic variant. PATIENT: A 46-year old male Philippine patient presented skin lesions, fever, red eyes, enlarged lymph nodes and marked eosinophilia. RESULTS: Lymphocytic phenotyping by flow cytometry analysis was performed on peripheral blood and an aberrant population of T lymphocytes CD3-CD4+ producing interleukin 5 was found. TCR gene rearrangement using PCR amplification confirmed T cell clonality. CONCLUSIONS: The lymphocytic variant of the hypereosinophilic syndrome is a primitive lymphocytic disorder characterized by a non-malignant T cell population expansion producing eosinophilopoietic cytokines, with an indolent clinical course but that can transform into a peripheral T lymphoma. We report the first case of such a variant published in our country and review the characteristics of this variety.

Variante linfoide del síndrome hipereosinófilo / Lymphocytic variant of the hypereosinophilic syndrome

Fundamento: El síndrome hipereosinófilo (SHE) es un grupo heterogéneo de procesos raros caracterizados por un aumento prominente y sostenido de eosinófilos en sangre y daño orgánico. Se deben excluir otras causas de hipereosinofilia. Avances recientes en biología molecular y citogenética han permitido caracterizar diferentes subtipos. Se describe un caso de la variedad linfoide. Paciente: Varón filipino de 46 años que consulta por lesiones cutáneas, fiebre, conjuntivitis, adenopatías y eosinofilia prominente. Resultados: El estudio de poblaciones linfocitarias en sangre periférica revela una población aberrante de linfocitos T (LT) CD3-CD4+ productora de interleucina 5 (IL-5). Se confirmó la clonalidad del TCR mediante PCR. Conclusiones: La variedad linfoide del SHE está caracterizada por una expansión clonal de LT no maligna que produce IL-5, de comportamiento indolente, pero que puede evolucionar a linfoma T periférico. Se describe el primer caso de variedad linfoide publicada en nuestro país y se revisan las características de esta variedad (AU)

Objectives: Hypereosinophilic syndromes (HSS) are a rare group of heterogeneous disorders characterized by prominent and persistent eosinophilia and organ dysfunction. Secondary causes of eosinophilia must be excluded. Recent advances in molecular biology and cytogenetics have permitted the characterization of different subsets of hypereosinophilic syndrome. We describe a patient with the lymphocytic variant. Patient: A 46-year old male Philippine patient presented skin lesions, fever, red eyes, enlarged lymph nodes and marked eosinophilia. Results: Lymphocytic phenotyping by flow cytometry analysis was performed on peripheral blood and an aberrant population of T lymphocytes CD3-CD4+ producing interleukin 5 was found. TCR gene rearrangement using PCR amplification confirmed T cell clonality. Conclusions: The lymphocytic variant of the hypereosinophilic syndrome is a primitive lymphocytic disorder characterized by a non-malignant T cell population expansion producing eosinophilopoietic cytokines, with an indolent clinical course but that can transform into a peripheral T lymphoma. We report the first case of such a variant published in our country and review the characteristics of this variety (AU)

Tumor intraabdominal desmoplásico de células pequeñas y redondas. Revisión de la literatura a propósito de un caso con estudio citológico, histopatológico, inmunohistoquímico y molecular / No disponible

Propósito: Revisar los hallazgos citohistopatológicos,inmunohistoquímicos y moleculares del tumorde células pequeñas redondas (DSRCT).Material y métodos: Varón de 18 años conmasa intraabdominal epigástrica.Resultados: Tumoración constituida por unaproliferación en nidos y difusa de células pequeñasindiferenciadas sobre estroma desmoplásico, conformación de rosetas, inmunorreactivas para citoqueratinas,vimentina, desmina y enolasa y la fracciónWT1 de la proteína EWS/WT1. El estudio moleculardemostró la traslocación t(11;22)(p13;q12).Conclusiones: La formación de rosetas y lasáreas sólidas han sido descritas en otros casos deDSRCT. El producto quimérico puede detectarse medianteRT-PCR, Southern-blot, Western-blot e inmunohistoquímica.El estudio citológico está especialmenteindicado en el diagnóstico de recidivas. Eldiagnóstico diferencial debe incluir el grupo de tumoresindiferenciados de células pequeñas y redondas

Purpose: To review the cyto-histopathological, immunohistochemical and molecular characteristicsof small round cell desmoplastic tumor (DSRCT).Material and methods: A 18 year old malewith an intraabdominal epigastric mass.Results: The tumor consisted of nests andmasses of undifferentiated small round cellsembedded in a desmoplastic stroma, with areas ofrosette formation immuno-reactive to cytokeratins,vimentin, desmin, enolase, and WT1 fraction of theEWS/WT1 fusion protein transcript. The molecularstudy demonstrated the existence of the translocationt(11;22)(p13;q12).Conclusions: The solid areas and rosetteformation have been described in other cases ofDSRCT. The chimeric transcription product can bedetected by RT-PCR, Southern-blot, Western-blot,and immunohistochemistry. Cytology is especiallyuseful in recurrences. The differential diagnosisshould be made with the small round undifferented cell tumors group (AU)

Leukemic infiltration of the prostate causing acute urinary retention.

A case of early involvement of the prostate by chronic lymphocytic leukemia first manifested by urinary retention is reported. The patient was prostatectomized without specific findings. Only about 60 similar previous cases have been reported in the literature.