What are the indications for blood transfusions in adults with thalassemia?

Indications for Blood Transfusion in Adults with Thalassemia

Regular blood transfusions are indicated for adults with thalassemia major to maintain hemoglobin levels above 9-10 g/dL to prevent anemia-related symptoms, improve quality of life, and prevent complications from ineffective erythropoiesis. 1

Transfusion Indications Based on Thalassemia Type

Thalassemia Major

• Primary indication: Regular transfusion therapy starting in childhood and continuing through adulthood
• Target hemoglobin: Maintain pre-transfusion hemoglobin >9-10 g/dL 2
• Frequency: Typically every 2-4 weeks based on individual needs
• Goal: Suppress ineffective erythropoiesis, prevent bone marrow expansion, and avoid anemia-related symptoms

Thalassemia Intermedia

• Symptomatic anemia despite other interventions
• Extramedullary hematopoiesis causing organ compression
• Growth failure or poor development in young adults
• Pregnancy with significant anemia
• Before surgical procedures when hemoglobin is significantly low

Specific Indications for Transfusion

1. Severe anemia with hemoglobin <8 g/dL causing symptoms 2
2. Symptomatic anemia even with higher hemoglobin levels (fatigue, exercise intolerance, tachycardia)
3. Complications of ineffective erythropoiesis:
   • Bone deformities or pathological fractures
   • Extramedullary hematopoiesis
   • Growth retardation
4. Cardiac complications or risk of heart failure
5. Pregnancy - higher hemoglobin targets may be needed
6. Perioperative period - to optimize oxygen delivery

Transfusion Considerations and Best Practices

Blood Product Selection

• Leukoreduced packed red blood cells - universal practice to reduce transfusion reactions 3
• Extended matching beyond ABO/Rh - phenotype matching for Rh and Kell antigens to reduce alloimmunization risk 3
• Consider irradiated blood products for selected patients (immunocompromised)

Monitoring Requirements

• Pre-transfusion assessment:
  • Complete blood count
  • Vital signs
  • Symptoms of anemia
• Post-transfusion assessment:
  • Hemoglobin levels
  • Signs of transfusion reactions
  • Volume status

Transfusion Complications to Monitor

• Alloimmunization - occurs in approximately 17% of transfused thalassemia patients 3
• Iron overload - requires regular monitoring of serum ferritin levels
• Transfusion-transmitted infections - despite screening, remains a risk (HCV, HIV, HBV) 4

Iron Chelation Requirements

Iron chelation therapy must accompany regular transfusions to prevent iron overload complications:

• Initiate iron chelation when serum ferritin exceeds 1000 ng/mL 5
• Available chelation options:
  • Deferasirox (oral)
  • Deferiprone (oral, 75 mg/kg/day in 3 divided doses)
  • Deferoxamine (subcutaneous or intravenous, 40-50 mg/kg/day) 5

Monitoring for Transfusion-Related Complications

• Serum ferritin - every 3-6 months
• Cardiac T2 MRI* - for early detection of cardiac iron overload (severe: T2* <10 ms) 5
• Liver iron concentration - assessment every 6-12 months in patients with cirrhosis 5
• Endocrine function - regular screening for diabetes, hypothyroidism, and hypogonadism 6

Special Considerations

• Splenectomized patients may have different transfusion requirements and increased risk of thrombotic complications 4
• Pregnancy may require more intensive transfusion support and careful monitoring
• Surgery requires optimization of hemoglobin levels before procedures

Pitfalls to Avoid

• Inadequate transfusion leading to chronic anemia symptoms and complications of ineffective erythropoiesis
• Overtransfusion increasing iron overload burden unnecessarily
• Failure to provide appropriate iron chelation alongside transfusion therapy
• Inconsistent transfusion schedules leading to fluctuating hemoglobin levels
• Inadequate monitoring for transfusion-related complications

Regular blood transfusions remain the cornerstone of management for thalassemia major and severe forms of thalassemia intermedia, with the primary goal of maintaining adequate hemoglobin levels to prevent complications while minimizing transfusion-related adverse effects through appropriate monitoring and chelation therapy.