Journal of Blood Disorders & Transfusion

Abstract

Postsplenectomy Thromboembolic Risk in Children with Hematologic Disorders: Case Report

Mara Ferrara, Fabrizia Bertocco, Dolores Ferrara and Laura Capozzi

Objective and Importance: We have evaluated retrospectively in splenectomized hematologic patients by 10 year follow-up the incidence of thromboembolic events (VTE) in relationship not only with thrombocytosis but also with other possible risk factors, particularly anemia and some genetic mutations. Clinical presentation: Fifty children of both sexes with Hereditary Spherocytosis (HS), Piruvate Kinase Deficit (PK), Congenital Dyserythropoiesis II (CDII), Chronic Idiopathic Thrombocytopenic Purpura (cITP), β thalassemia intermedia (TI), splenectomized at 12.8 ± 6.2 years and untransfused or not transfused in the last 5 years were followed. Four patients with iron deficiency anemia had, on follow-up, deep venous thrombosis (DVT) during pregnancy (1 HS and 1 cITP) or after blood losses (2 PK deficits). Two patients with mild chronic anemia affected by CDII and TI respectively showed cerebrovascular thrombosis (CVT). None patient with VTE had genetic mutations. Intervention (& technique): Platelet count (PLT), Hb levels, clotting tests were performed once a year and compared with healthy controls. Genetic mutations of Methylentetrahydrofolate reductase (MTHFR) C677T, Factor V Leiden G1691A and Prothrombin G20210A were studied. VTE was diagnosed by Doppler Ultrasonography and Magnetic Resonance. Conclusion: CDII, TI and PK deficit subjects had altered Anti-thrombin III, Protein S, Protein C, activated partial thromboplastin time, thrombocytosis and lower Hb levels than other pat ients (p<0.05). Longer persistence of altered red cells and/or restricted erythropoiesis may increase the risk of VTE.