We report the case of a 13-year-old male patient with beta-thalassemic trait who presented for a colic pain. An ultrasound of the abdomen revealed absence of the right kidney with a complex hypoechogenic pelvic mass causing mild pressure on the posterior bladder wall. Urography showed hypertrophy of the left kidney with moderate ureteral enlargement of the distal third due to an insertion defec...

This case demonstrates the benefits of luspatercept for thalassemic patients who are unable to receive adequate blood transfusions. It acts as a selective ligand trap and inhibits TGF- signaling via Smad2/3 promote differentiation maturation late-stage erythroid precursors. Phase III clinical data showed that 33% reduction in transfusion burden from baseline was achieved. The administration cha...

It is well known that thalassemic patients exhibit an increased frequency of thrombotic events. Most individuals with resistance to activated protein C (APCR) are the result of a point mutation replacing Arg 506 with Gln in the factor V aminoacidic sequence (factor V Leiden). Recently APCR has been shown to account for up to 50% of cases of thrombophilia. In this report, we describe a 10 year o...

Phospholipid asymmetry in the red blood cell (RBC) lipid bilayer is well maintained during the life of the cell, with phosphatidylserine (PS) virtually exclusively located in the inner monolayer. Loss of phospholipid asymmetry, and consequently exposure of PS, is thought to play an important role in red cell pathology. The anemia in the human thalassemias is caused by a combination of ineffecti...

The life threatening anemia in beta-thalassemia major (Cooley's anemia) is characterized by profound intramedullary lysis, the cause of which is incompletely understood. Using marrow obtained from beta thalassemia major patients undergoing allogeneic bone marrow transplantation in Pesaro Italy, it became possible to directly study the mechanism of the intramedullary hemolysis. Based on our prev...

The frequency of HAV, HBV, HCV and HDV infections were evaluated in children with thalassemia haematologic by ELİSA. A total 136 serum specimens tested for this purpose. Hepatitis B surface antigen (HBsAg) positivity was found 46 % patients leukemia, 50 lymphoma 16.6 thalassemic children. Anti present 8 leukeınia group group. our opinion, vira! hepatitis that have a great prevalence multitransf...

BACKGROUND Thalassemia is a blood disorder passed down through families (inherited) in which the body makes an abnormal form of hemoglobin. This disorder results in excessive destruction of red blood cells, and there is no effective treatment. Patients require lifelong blood transfusion, usually started within 6 to 12 months of birth of patient, which on other hand has its own complications. It...

Abstract Background The purpose of this study was to assess the prevalence of major depressive and anxiety disorders in hemophilic and major beta thalassemic patients related to education of their mothers as a family’s agent. Materials and Methods A case-control study was performed on 34 major beta thalassemic patients. For each patient the control group was selected and matched (with age a...