[Transient erythroblastopenia in the context of Epstein-Barr virus infection]. / Eritroblastopenia transitoria en el contexto de infección por el virus de Epstein-Barr.

The authors report the case of a previously healthy 5-year-old boy who, 1 month after tonsillitis, presented severe aregenerative anemia, macrocytosis, and increased hemoglobin F associated with mild thrombocytopenia. Peripheral blood smear showed no significant alterations and bone marrow aspirate revealed markedly decreased red blood cell precursors. Etiological investigation showed elevated titers of Epstein-Barr virus antibodies (IgM anti-viral capsid antigen). Bone marrow biopsy showed no criteria for red cell aplasia and in situ hybridization revealed intracellular Epstein-Barr virus. Reticulocytosis emerged from days 10-17 of follow-up, together with rising hemoglobin. Mean corpuscular volume and hemoglobin F returned to normal 2 months later. Immunological study revealed partial IgG3 deficit that persisted after 1 year of follow-up.

Eritroblastopenia transitoria en el contexto de infección por el virus de Epstein-Barr / Transient erythroblastopenia in the context of Epstein-Barr virus infection

Los autores describen el caso clínico de un niño varón de 5 años de edad, previamente sano que, 1 mes después de una amigdalitis, presentó una anemia arregenerativa grave con macrocitosis y hemoglobina F aumentada, asociada a una trombocitopenia ligera. El frotis de la sangre periférica no presentaba alteraciones significativas y el aspirado de medula ósea revelaba una hipoplasia eritroide marcada. La investigación etiológica reveló la presencia de un título elevado de anticuerpos anti-viral capsid antigen IgM para el virus Epstein-Barr. En la biopsia ósea, sin criterios de aplasia medular, se comprobó la presencia intracelular del virus por hibridación in situ. El día 17 de seguimiento fue posible documentar la aparición de reticulocitosis y el incremento del valor de hemoglobina. El volumen globular medio y el porcentaje de hemoglobina F se normalizaron a los 2 meses. El estudio inmunológico reveló un déficit parcial de IgG3, que mantiene después de 1 año de seguimiento (AU)

[Brain stem ischemia in a boy with resistance to C activated protein and elevated lipoprotein A]. / Isquemia del tronco cerebral en niño con resistencia a la proteína C activada y a la lipoproteína (a) elevada.

INTRODUCTION: Activated protein C resistance is the most common hereditary coagulation abnormality. In the majority of cases it results from a point mutation Arg506-->Gln of the factor V gene, and characterized by a poor anticoagulant response to activated protein C. CLINICAL CASE: We report the clinical case of a 6-year-old obese boy, who presented with acute hemiparesis. A cerebral MRI revealed an area of infarction in the left hemiprotuberance. Further investigation identified activated protein C resistance (heterozygosity for factor V Leiden) and elevation in lipoprotein (a). His mother also had factor V Leiden mutation. Prophylaxis with acetylsalicylic acid was instituted with favorable evolution. CONCLUSIONS: This mutation, isolated, is usually asymptomatic, unless other risk factors coexist. Although venous thromboembolism seems to be the main clinical manifestation, recent reports consider that activated protein C resistance is also a risk factor for arterial thrombosis and stroke in children. We reinforce the need for systematic and thorough evaluation of etiology and risk factors in cases of stroke in children.