Improvement in Prognosis and Survival

Improvement in Prognosis and Survival

Historically, patients who had thalassemia had a poor prognosis. In a United States cohort born between 1960 and 1976, the median was 17 years (Ehler et al., 1991). Remarkable and promising improvement in survival of patients who have thalassemia has been made (Borgna-Pignatti, 2004). The prognosis of patients with homozygous B-thalassemia major has been improved by transfusion and iron-chelation therapy. Prognosis for survival without cardiac disease is excellent for patients who receive regular transfusions and whose serum ferritin concentrations remain below 2500 ng per milliliter with chelation therapy (Borgna-Pignatti, 2004).

Table (1):    Causes of death in patients with β –Thalassemia

Cause of Death	Percentage of people with thalassemia major
Heart problems: -          Heart failure -          Irregular heart rhythm  -          Heart attack	60% 7% 2%
Infection	7%
Liver cirrhosis	4%
Thrombosis (blood clot in brain, lungs or heart)	4%
Cancer	4%
Diabetes	3%
Unknown causes	3%
Other (e.g., accidents, renal failure, HIV/AIDS, anemia)	7%

Management of β-Thalassemia Major

Red cell transfusion

Regular red cell transfusion eliminates the complications of anaemia and ineffective erythropoiesis, permit normal growth and development throughout childhood and extend survival in thalassemia major (Oliverri et al., 1997). 6-8 cc/Kg of PRBC’s at 2-3 weekly intervals decreases the yearly iron load by 20% (Spanos at al., 1995). The goal of long-term hypertransfusional support is to maintain the patient's Hb at 9-10 g/dL, thus improving the patient's sense of  well-being  simultaneously.