Citopenias autoinmunes: tratamiento según el diagnóstico. Respuesta de los autores / Autoimmune cytopenias: Diagnosis Before Treatment. Reply by the authors

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Rituximab en el tratamiento de citopenias autoinmunitarias refractarias a tratamientos convencionales / Role of rituximab in the management of refractory autoimmune cytopenia

Objetivo: Evaluar el papel del rituximab en el tratamiento de citopenias autoinmunitarias refractarias a tratamientos convencionales. Material y métodos: Estudio descriptivo longitudinal constituido por una serie de casos clínicos (n = 7) durante el período comprendido entre 2003 y 2010. Resultados: Se recogen 7 pacientes: 4, trombocitopenia inmune primaria; 2, anemia hemolítica autoinmunitaria, y 1, neutropenia autoinmunitaria. Un paciente había recibido trasplante de progenitores hematopoyéticos. La dosis fue de 375mg/m2/semana. Cuatro pacientes recibieron 4 dosis y 3 pacientes recibieron 2, 6 y 8 dosis cada uno. Cinco de los pacientes (71%) cumplieron criterios de respuesta global (completa en 4 pacientes y parcial en 1). A las 8,5 semanas, respondieron la mitad de los pacientes (rango: 3,5-19,5). La mediana de la duración de la respuesta fue de 35,5 semanas (rango: 12,5-53,5). El 100% de los respondedores pudo disminuir su tratamiento previo. Se registraron 2 recaídas. No se registran efectos adversos graves. Conclusiones: El 71% de los pacientes de esta serie responden al tratamiento, disminuyendo el 100% de los respondedores su tratamiento previo. Rituximab es un tratamiento bien tolerado, sin efectos secundarios graves en el período de seguimiento estudiado (AU)

Objectives: This study examined the efficacy of rituximab in children with refractory autoimmune cytopenia. Material and methods: Longitudinal descriptive study comprising a series of clinical cases (n=7) during the period 2003 to 2010. Results: A series 7 patients were included (4 had primary immune thrombocytopenia, 2 autoimmune hemolytic anemia, and 1 autoimmune neutropenia). One patient had received stem cell transplantation. Rituximab was administered intravenously to all patients at a dose of 375mg/mg2 weekly. Four patients received 4 doses. Three patients received 2, 6, and 8 doses, respectively. Overall, 5 patients responded (4 complete responses plus 1 partial response). The median time to achieve complete response was 8.5 weeks (range: 3.5-19.5 weeks). Two patients achieved complete response in the first 3.5 weeks, and the remaining 3 patients between 8.5 and 19.5 weeks. The median time of response was 35.5 weeks (range: 12.5-53.5 weeks). Two patients relapsed. No serious adverse events were recorded. Conclusions: Overall, seventy one percent of patients in this study respond to treatment, 100% of responders decrease their previous treatment. Rituximab was a well tolerated and no related serious side effects were recorded during the study period (AU)

Nefroma mesoblástico congénito con hiperecogenicidad medular que simula nefrocalcinosis / Congenital mesoblastic nephroma with medullary hyperechogenicity simulating nephrocalcinosis

El nefroma mesoblástico congénito (NMC) es un tumor raro, siendo el más frecuente a nivel renal en los pacientes menores de 2 meses. Su origen histológico es la estroma renal inmadura, y se distinguen los subtipos clásico, mixto y celular. El tratamiento de elección es quirúrgico y su pronóstico es excelente. Se han descrito casos de NMC asociado a nefrocalcinosis en relación con la hipercalcemia paraneoplásica. Exponemos el caso de un recién nacido que presenta en la ecografía imágenes de hiperecogenicidad medular renal bilateral, similar a una nefrocalcinosis, en el contexto clínico de un NMC(AU)

Congenital mesoblastic nephroma (CMN) is a rare tumour which is the most frequent in the first 2 months of life. Its histological origin is the immature renal stromal cells. There are three histological subtypes: clasic, mixte and cellular. The treatment of choice is surgical and the prognosis is excellent. CMN has been reported associated with nephrocalcinosis in relation to paraneoplasic hypercalcaemia. We report a case of a new born with ultrasound imagen of renal medullary hyperechogenicity simulating nephrocalcinosis in the clinical setting of CNM(AU)

[Role of rituximab in the management of refractory autoimmune cytopenia]. / Rituximab en el tratamiento de citopenias autoinmunitarias refractarias a tratamientos convencionales.

OBJECTIVES: This study examined the efficacy of rituximab in children with refractory autoimmune cytopenia. MATERIAL AND METHODS: Longitudinal descriptive study comprising a series of clinical cases (n=7) during the period 2003 to 2010. RESULTS: A series 7 patients were included (4 had primary immune thrombocytopenia, 2 autoimmune hemolytic anemia, and 1 autoimmune neutropenia). One patient had received stem cell transplantation. Rituximab was administered intravenously to all patients at a dose of 375 mg/mg(2) weekly. Four patients received 4 doses. Three patients received 2, 6, and 8 doses, respectively. Overall, 5 patients responded (4 complete responses plus 1 partial response). The median time to achieve complete response was 8.5 weeks (range: 3.5-19.5 weeks). Two patients achieved complete response in the first 3.5 weeks, and the remaining 3 patients between 8.5 and 19.5 weeks. The median time of response was 35.5 weeks (range: 12.5-53.5 weeks). Two patients relapsed. No serious adverse events were recorded. CONCLUSIONS: Overall, seventy one percent of patients in this study respond to treatment, 100% of responders decrease their previous treatment. Rituximab was a well tolerated and no related serious side effects were recorded during the study period.

Exantema petequial y disnea en un adolescente: síndrome de embolismo graso / Petechial rash and dyspnea in an adolescent: fat embolism syndrome

El síndrome de embolismo graso es un proceso asociado con frecuencia a las fracturas de los huesos largos y pélvicos, manifestad otras un periodo asintomático de 24-72 horas. La tríada clásica de presentación consiste en hipoxemia, afectación neurológica y exantema petequial. Su diagnóstico se basa encriterios clínicos, y el tratamiento fundamental es de soporte. Se han utilizado diversas estrategias terapéuticas con resultados no concluyentes. Comunicamos el caso de una adolescente con un cuadro de dificultad respiratoria aguda y exantema petequial asociado a una fractura de fémur (AU)

The fat embolism syndrome is a process usually associated with long bones and pelvis fractures that typically appear after an asymptomatic period of 24 to 72 hours. The presentation classic triad consists of hypoxemia, neurologic involvement and a petechial rash. The diagnosis is based in clinical criteria and the treatment should be mainly supportive. Many drugs have been used, but the results are inconclusive. We present the case of an adolescent with an acute respiratory distress syndrome and petechial rash, associated with femur fracture (AU)

Hiperlipidemia transitoria secundaria al tratamiento con asparraginasa y prednisona / Transient hyperlipidemia secondary to treatment with asparaginase and prednisone

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Masa supraclavicular / Supraclavicular mass

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