Epidemiological Study among Thalassemia Intermedia Pediatric Patients
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- Category: Vol. 78, December 2010
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Epidemiological Study among Thalassemia Intermedia Pediatric Patients,NERMEEN KADDAH, KHALED SALAMA, AHMED MAHER KADDAH and RANIA ATTIA
Abstract
Background: Thalassemia intermedia is a term used to define a group of patients with beta thalassemia in whom the clinical severity of the disease is somewhere between the mild symptoms of beta thalassemia trait and the severe manifesta-tions of beta thalassemia major. The diagnosis is a clinical one based on the patient maintaining a satisfactory hemoglobin (Hb) level of at least 6-7g/dl at the time of diagnosis without the need for regular blood transfusions.
Objectives: To study the demographic; clinical and labo-ratory data of patients with thalassemia intermedia.
Material and Methods: The present work was a retrospec-tive study on records of patients with thalassemia intermedia attending the hematology clinic, New Children Hospital, Cairo University from 2003-2006. It comprised follow-up sheets of 58 thalassemia intermedia patients, from which data were collected including full history and clinical examination, and laboratory investigations including CBC; including Hb, HCT, RBCs, MCV, MCH, also retics and Hb electrophoresis.
Results: The age of patients ranged between 1-16 years with a mean age of 4.45±2.7. Male to female ratio was 1: 1.148. There was no history of blood transfusion in 51.7% of patients while 48.3% had history of blood transfusion. Of these patients, 46.4% received blood transfusion only once, 14.3% received twice, 14.3% received 3 times, 25% received blood transfusion 4 times or more. Pallor was the most fre-quently encountered sign in 86.2% of the patients, while splenomegaly was present in 70.2%, jaundice in 67%, mon-goloid features in 65.5%. Hb ranged between 4.4-10gm/dl, while MCV ranged between 20-79.8 with a mean of 60.25. Hb electrophoresis showed Hb A ranging between 0-97.3 with a mean 70.25, Hb A2 ranging between 0.1-11 with a mean 3.09, and Hb F ranging between 2-98.04 with a mean 26.47.
Conclusion: Thalassemia intermedia patients present at older age unlike thalassemia major which usually becomes evident during the 1st year of life. Pallor is the commonest symptom. Also it may present with jaundice or enlarged abdomen. They usually are able to maintain a satisfactory Hb level of at least 6-7g/dl at the time of diagnosis withoutn the need for regular blood transfusion. Some of these patients need no transfusion therapy while others need frequent blood transfusion.