RezumatSferocitoza ereditarã este o maladie congenitalã, caracterizatã prin anemie hemoliticã, ce afecteazã membranele eritrocitelor. Manifestãrile clinice variazã de la forme aproape asimptomatice la forme severe, ce necesitã transfuzii. Diagnosticul se bazeazã pe examenul clinic, hemograma cu numãrul reticulocitelor, prezenţa unui istoric familial şi teste de laborator specific, cum ar fi testul EMA (eosin-5-maleimide binding test) sau testul AGLT (Acidified Glycerol Lysis Time). Splenectomia este consideratã tratamentul standard în formele moderate pânã la severe de boalã. Totuşi, este cunoscut faptul cã splenectomia totalã expune pacientul la complicaţii infecţioase, uneori letale, ceea ce a determinat indicarea acestei metode cu precauţie. Astfel splenectomia subtotalã sau parţialã a devenit o alternativã de tratament fezabilã. Aceasta reduce distrugerea eritrocitelor, în timp ce conservã funcţia imunã a splinei. Majoritatea studiilor au arãtat beneficii certe, pe termen scurt, dupã splenectomie, în cazul acestor pacienţi. Totuşi, pânã în prezent, rezultate pe termen lung certe, adicã pe o perioadã de peste 5 ani de urmãrire postoperatorie, încã lipsesc.Cuvinte cheie: sferocite, hemoliză, citometrie în flux, splenectomie, rezultate pe termen lung
AbstractHereditary spherocytosis (HS) is a disease affecting the red blood cells membrane and belongs to the congenital hemolytic anemias. The clinical spectrum ranges from asymptomatic patients to severe forms requiring transfusions in early childhood. The diagnosis can be based on the physical examination, complete red blood cell count, reticulocytes count, medical history and specific tests, preferentially the EMA test (eosin-5-maleimide binding) test and AGLT (Acidified Glycerol Lysis Time). Splenectomy is considered the standard surgical treatment in moderate and severe forms of hereditary spherocytosis. Total splenectomy exposes the patient to a life -long risk of potentially lethal infections and thus, its usage was reconsidered. Because of this reason, a feasible alternative is the partial splenectomy. The use of partial splenectomy aims to retain splenic immunologic function, while at the same time to decrease the rate of hemolysis. The long -term outcomes of patients with total or subtotal splenectomy for congenital hemolytic anemia, still remain unclear, but the majority of the studies showed a qualitative resolution of anemia and reduction of transfusion rate. Despite the well known advantages of conservative surgery, the optimal choice of treatment and outcomes should be confirmed with the patient.