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