Treatment Thalassemia Major
Blood Transfusion: Hypertrasnfusion protocol. Washed packed cells or leucodepleted packed calls.
Chelation Therapy: Desferoxamine, Deferiprone, Deferasirox.
Monitoring and treatment of complications: Endocrine complications, cardiac complications, infections, Blood transfusion reaction all needs a life time heamo-vigilance to decrease the incidence of mortality and morbidity and ultimately improves the survival and quality of life.
Treatment modalities related to Thalassemia Intermedia
- Non-Transfused Thalassemia intermedia (NTTI)
- Transfusion dependent Thalassemia Intermedia (NTTI)
Non-Transfused Thalassemia intermedia (NTTI)
A baseline red blood cell phenotype should be obtained from patients, who should then be seen at a thalassemia center every three to six months, with attention to overall clinical well-being. Following parameters should be monitored: Growth and development, cardiopulmonary assessment, pulmonary hypertension, extramedullary hematopoiesis.
When to start regular transfusion in (NTTI):
- On the basis of following parameters
- Clinical and Lab Assessment
- Symptomatic anemia
- Poor school performance
- Falling Growth velocity
- Delayed puberty
- Extramedullary hematopoiesis
- Leg ulcers
- Pulmonary hypertension
- Thrombosis
- Hypersplensim
When to plan splenectomy in (NTTI):
- Massive splenomegaly
- Hypersplenism or unable to receive transfusions
Transfusion dependent Thalassemia intermedia (TDTI)
- Regular folic acid
- Chelation if required
- Regular echo
- Growth and pubertal assessment
- Transfusion if indicated
- Extramedullary Haemopoiesis chest or spine .Radiation therapy if symptomatic.
- Hydroxyurea