Hematopoyesis extramedular en paciente con esferocitosis hereditaria asintomática

En diversas patologias con alteraciones en la produccion de los elementos formes de la sangre pueden desarrollarse focos de hematopoyesis extramedular en diferentes sitios. Los mas frecuentes son: bazo, higado, ganglios linfaticos, y mas raramente otros organos como: glandulas adrenales, hillios renales, cartilagos, ligamentos, tejido adiposo, timo, pulmon, mediastino y duramadre de craneo y columna. Generalmente el proceso es difuso pero pueden formarse grandes tumores de tejido hematopoyetico. Las condiciones patologicas de la medula osea mas frecuentemente asociadas a hematopoyesis extramedular son: esferocitosis hereditaria, talasemia, sindromes mieloproliferativos con fibrosis medular, ocupacion medular por patologias neoplasicas. presentamos un paciente de 45 anos con esferocitosis hereditaria con masas de tejido hematopoyetico extramedular paravertebral mediastinal que respondieron favorablemente a la esplenectomia. Hereditary spherocytosis (HS) is a relatively common inherited hemolytic disorder in northern Europe and in the US. The reported prevalence of HS in Western countries is 1:5000. We describe a patient 45 years old, with hereditary spherocytosis with masses of mediastinal paravertebral extramedullary hematopoietic tissue, with a favorable response to splenectomy. The medical lieterature refers some cases of extramedullary hematopoiesis as a clinical expression of hereditary spherocytosis, mainly as thoracic masses with usually paravertebral localization. HS should be distinguished from other spherocytic hermolytic anemias. Diagnosis is usually made uring infancy or in young adults, but it can be at any moment of their life, until the seventh decade of life. Ocasionally, the diagnosis is first made in old age. The clinical expression of HS is highly variable, ranging from asymptomatic condition to a severe life-threatening hemolytic anemia. Laboratory features include spherocytosis, osmotic fragility, manifestations of hemolytic disease, elevated unconjugated bilirubin and reticulocytosis. The principal diagnostic test, RBC osmotic fragility, measures the surface/volume Ratio of the cells. The treatment of choice of HS in patients with inherited spherocytosis is splenectomy, which corrects hemolytic anemia. According to he literature, cases of failure following splenectomy have been reported.