Sickle Cell Anemia in India
- Bone marroww transplant (BMT), specifically hematopoietic stem cell transplant (HSCT), is a potential curative treatment for pediatric patients with sickle cell anemia (SCA).
- Sickle Cell Anemia is a genetic blood disorder characterized by the production of abnormal hemoglobin (hemoglobin S), leading to distorted, sickle-shaped red blood cells. This results in various complications, including pain crises, anemia, increased risk of infections, and organ damage.
Types of Sickle Cell Anemia in India
- Matched Sibling Donor Transplant: The best outcomes are generally seen with a fully matched sibling donor.
- Haploidentical Transplant: A half-matched relative (e.g., a parent) can serve as a donor.
- Unrelated Donor Transplant: Suitable when no familial match exists, using registries to find a compatible donor.
Indications for Sickle Cell Anemia
- Severe Disease Manifestations
- Severe Anemia
- Stroke or Transient Ischemic Attacks (TIAs)
- Organ Damage
Donor Selection
- Matched Sibling Donor:The ideal donor is often a sibling who is a perfect (HLA-identical) match. This option typically provides the best outcomes in terms of engraftment and lower risks of complications like graft-versus-host disease (GVHD).
- Haploidentical Donor:A half-matched relative, such as a parent, can also be considered. Advances in haploidentical transplant techniques, including post-transplantation cyclophosphamide (PTCy), have improved outcomes for these patients.
Diagnosis
- Screening methods (newborn screening, hemoglobin electrophoresis)
- Challenges in early analysis in provincial regions
- Importance of genetic counseling
Clinical Features
- Common symptoms (pain crises, anemia, fatigue) .
- Acute and chronic complications (e.g., infections, organ damage)
- Influence on personal satisfaction and everyday exercises
Complications and Management
- Standard treatment options (pain management, blood transfusions)
- Disease-modifying therapies (hydroxyurea, bone marroww transplant)
- Challenges in accessing treatment in India
Recent Advances and Research
- Innovations in Sickle Cell Anemia transplant techniques .
- Role of immunotherapy and new treatment modalities .
- Progressing research and clinical preliminaries in India.
Conclusion
- Hematopoietic stem cell transplant offers a potential cure for children with sickle cell anemia, especially for those with severe disease.
- Careful patient selection, thorough evaluation, and a multidisciplinary approach are crucial for optimizing outcomes and managing complications.
- Families should work closely with their healthcare team to understand the risks and benefits of the transplant process.
Departments
Blood Cancer
Sickle Cell Anaemia
Allogeneic Transplant
Haploidentical Transplant
Syngeneic Transplant
Related Hospitals
Indraprastha Apollo Hospitals (Jasola, Delhi)
Max Super Specialty Hospital Gurgaon
Artemis Hospital (Gurgaon)
Jaypee Hospital Noida Delhi NCR India
