Beta Thalesemia Treatment

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
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