ABSTRACT
Introducción: La purpura trombocitopénica idiopática es un trastorno adquirido causado por auto-anticuerpos contra antígenos plaquetarios.A la fecha no se han estudiado las características clínicas y respuesta al tratamiento en Bolivia. Objetivo: Describir las características clínicas y resultados del tratamiento en pacientes adultos con purpura trombocitopénica idiopática de reciente diagnóstico. Métodos: Se realizó un estudio descriptivo, retrospectivo de una serie de casos de adultos con púrpura trombocitopénica idiopática en un Servicio de Hematología de La Paz, Bolivia durante el periodo 2009-2014. Resultados: El análisis incluyó 23 pacientes con una edad media de 59,9 años. Al diagnóstico todos los pacientes presentaron manifestaciones hemorrágicas (nasal 82,6%, cutáneo 56,5%, y oral 34,78%), la media del recuento plaquetario al diagnóstico fue de 26.7 x 109/L. Se detectó anticuerpos contra Helicobacter pylori en 4 de 6 que contaban con esta prueba. El hallazgo más frecuente en médula ósea fue la presencia de un número aumentado o normal de megacariocitos. La respuesta al tratamiento de primera línea fue del 96% (RC: 61%, R: 35%). Tras una media de seguimiento de 24 meses, el 60% de los pacientes presentaron recaída en un promedio de 12,5 meses desde el diagnóstico. Conclusiones: Al momento del diagnóstico las manifestaciones hemorrágicas son las más frecuentes, los recuentos plaquetarios por lo general son inferiores a 30 x 109/L. Los corticoides son el tratamiento inicial de elección, con respuestas favorables en la mayoría de los pacientes, sin embargo, más de la mitad de los pacientes presentan recaída en tiempo promedio de 1 año.
Introduction: Idiopathic thrombocytopenic purpura is an acquired disorder caused by autoantibodies against platelet antigens. To date have not been studied the clinical characteristics and treatment response in Bolivia. Objective: To describe the clinical characteristics and treatment outcomes in adult patients with newly diagnosed immune thrombocytopenic purpura. Methods: A descriptive, retrospective study of an adult case series with immune thrombocytopenic purpura was performed in a Hematology Service of La Paz, Bolivia during the period 2009-2014. Results: We included 23 patients with a median age of 59.9 years. All patients had hemorrhagic manifestations at diagnosis (nasal 82.6%, oral 56.5% and cutaneous 34.78%). The average platelet count was 26.7 x 109/L. The presence of antibodies against Helicobacter pylori was detected in 4 of 6 patients. The presence of normal or increased megakaryocyte numbers was the predominant finding in bone marrow. The response to first-line treatment was 96% (CR: 61%, R: 35%).After a mean follow-up of 24 months, 60% of the patients had a relapse in an average of 12.5 months from diagnosis. Conclusions: At the time of diagnosis, hemorrhagic manifestations are the most frequent, platelet counts are usually less than 30x109/L. Corticosteroids are the initial treatment of choice, with favorable responses in the majority of patients, however, more than half of the patients present relapse in an average time of 1 year.
ABSTRACT
Human parvovirus B19 (B19V) is a small (5.5kb) single-stranded DNA virus with known tropism and cytotoxicity for erythroid progenitors. Although B19V was known to be incapable of propagating in the cells other than erythroid progenitors in vitro, leukopenia and thrombocytopenia sometimes occur in addition to erythrocytopenia in patients with B19V infection. Recently, idiopathic thrombocytopenic purpura (ITP) was reported as a rare complication in children following B19V infection. We experienced a case of B19V infection with erythroid aplasia and ITP in 10 years-old girl who presented with severe nasal bleeding and whole body petechiae. Marked thrombocytopenia (4x109/L) and reticulocytopenia (0.1%) were observed. The bone marrow aspiration smears revealed increased number of megakaryocytes and severe red cell hypoplasia with occasional giant pronormoblasts and nearly absence of mature normoblasts. Serum anti- B19V IgM and IgG antibodies were positive.