Background: Thalassemia is one of the most common genetic disorder hemoglobin synthesis in Jammu region. Although RBC transfusion life saving for these patients, it may be associated with some complications like alloimmunization. Thus, alloimmunization against red blood cell antigens increases need and can significantly complicate therapy. Therefore, screening unexpected antibodies should a par...

βThalassemia is an inherited genetic disorder which is caused by different kinds of mutations in the HBB gene in chromosome 11. Due to several types of mutation in βgene, globin chains cannot synthesise completely and free α-globin is highly unstable and readily precipitates bound heme and iron. In βthalassemia these precipitated iron, repeated blood transfusion and increased gastrointestinal i...

BACKGROUND First and second-degree heart blocks are partly common rhythm disorders in thalassemic patients but complete heart block is a very rare complication of iron overload cardiomyopathy. CASE PRESENTATION This 15-year-old boy, a known case of major β-thalassemia was admitted to our emergency unit with dyspnea and cough because of decompensated heart failure. The electrocardiogram showed...

Major thalassemia is one of the most common hemoglobinopathies in many Asian countries including Iran. Pharmacologic agents such as hydroxyurea have been known to enhance the production of fetal hemoglobin, and also an increase in total hemoglobin level has been repeatedly reported during hydroxyurea treatment in patients with sickle cell disease and in several patients with intermediate beta-t...

Background: In thalassemia major as a chronic disease patients need to require information about the disease processes and therapeutic interventions. The aim of the present study was to evaluate the knowledge, attitude, and practice behavior of thalassemic patients. Patients and Methods: This was a cross-sectional descriptive knowledge, attitude and practice study conducted in Zafar adult tha...

Gino Schilirò, Antonio Russo, Cattedra di Ematologia Pediatrica, Istituto di Clinica Pediatrica I, Università di Catania, Viale A. Doria 6, I-95125 Catania (Italy) It is now widely accepted that the best treatment for thalassemia major is a precocious and regular transfusional regimen with the aim of maintaining pretransfusional Hb levels of 10.511 g/dl [1]. Such an approach, by minimizing the ...

Gino Schilirò, Antonio Russo, Cattedra di Ematologia Pediatrica, Istituto di Clinica Pediatrica I, Università di Catania, Viale A. Doria 6, I-95125 Catania (Italy) It is now widely accepted that the best treatment for thalassemia major is a precocious and regular transfusional regimen with the aim of maintaining pretransfusional Hb levels of 10.511 g/dl [1]. Such an approach, by minimizing the ...

The life threatening anemia in /3-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 previo...

I N PAST YEARS interesting results have been reported by a number of investigators on the l fe span of thalass mic red cells measured by the Ashby technic of differential agglutination. Kaplan and Zuelzer’ transfused blood from three children suffering from thala semia major into normal subjects; the survival curves showed a marked shortening of the half life ( 25 days ) , with a straighter dro...