Aplastic Anemia ka treatment
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Aplastic Anemia ka Treatment
Sabse Pehle: Severity Classification
Treatment severity ke hisaab se decide hoti hai:
| Category | Criteria |
|---|---|
| Severe AA (SAA) | BM cellularity <25% + ≥2 of: ANC <500, Platelets <20,000, Reticulocytes <20,000 |
| Very Severe AA (VSAA) | SAA jaise + ANC <200 |
| Non-Severe AA | Criteria poore nahi hote |
1. Definitive Treatment
A) Hematopoietic Stem Cell Transplantation (HSCT) — First Choice
- Kiske liye: Young patients (<40 years) jinka HLA-matched sibling donor ho
- Kab: Diagnosis ke saath hi HLA typing karwa leni chahiye
- Results: Children mein ~90% long-term survival
- Fayde vs IST:
- Relapse ka risk kaafi kam
- MDS/AML aur PNH hone ka khatra bhi kam
⚠️ Important: Transplant candidates ko family members ka blood bilkul mat chadhaein — HLA sensitization ka risk hota hai.
Alternative Donors (agar matched sibling nahi):
- Matched Unrelated Donor (MUD) — large registries mein available, results sibling jaisi
- Cord blood — khaas taur pe bacchon ke liye
- HLA-Haploidentical donor (family member) — ab increasingly popular; post-transplant cyclophosphamide se GVHD rokta hai
B) Immunosuppressive Therapy (IST) — First Choice jab HSCT nahi
Kiske liye:
- Jo patients HSCT ke eligible nahi (age >40, ya matched sibling nahi)
- Kuch cases mein young adults mein bhi front-line
Standard Triple Therapy (Current Gold Standard):
| Drug | Dose | Role |
|---|---|---|
| Horse ATG (hATG) | 40 mg/kg/day × 4 days | T-cell destruction |
| Cyclosporine (CsA) | 12–15 mg/kg/day divided BD | Immune suppression, relapse prevention |
| Eltrombopag | 150 mg/day | TPO-receptor agonist, ~20% response badhata hai |
| Corticosteroids | Pehle 2 weeks | Serum sickness rokne ke liye |
Horse ATG > Rabbit ATG — Front-line treatment mein horse ATG superior hai.
Response: 70–80% patients mein hematologic recovery hoti hai (transfusion independence + adequate WBC count)
Timeline:
- Granulocytes mein improvement ~2 months mein dikhti hai
- Full response aane mein months lag sakte hain
- Cyclosporine/eltrombopag tapering pe relapse ho sakta hai — careful taper karein
2. Supportive Care (Sabke liye zaruri)
Blood Products:
- Irradiated blood products dein — Transfusion-associated GVHD rokne ke liye
- Leukocyte-reduced products dein — Alloimmunization aur CMV infection kam hoti hai
- Central venous catheter strongly consider karein
Infection Management:
- Fungal aur bacterial infections — SAA mein major cause of death
- Active fungal infection definitive therapy (IST/HSCT) mein delay nahi karni chahiye
- Prophylactic G-CSF add karne se IST mein extra benefit nahi milta
3. Refractory / Relapse Cases
- Re-treatment with IST ya alternative donor HSCT
- Eltrombopag monotherapy ne refractory cases mein bhi kaam kiya hai
- Androgens (e.g., danazol) — purana treatment, ab mainly refractory/older patients mein; Fanconi anemia mein bhi use hota hai
- Romiplostim / Danazol — investigational salvage options
4. Special Situations
Pregnancy mein Aplastic Anemia:
- 25–30% mein spontaneous remission — delivery ya termination ke baad
- Stringent supportive care ya Cyclosporine consider karein
- Low platelets + PNH ke saath complications zyada hoti hain
Constitutional AA (Fanconi Anemia, Dyskeratosis Congenita):
- HSCT is treatment of choice — leukemia/MDS ka bhi risk hota hai
- Sex hormones (androgens) — palliative role
- Standard IST utni effective nahi hoti
5. Prognosis
| Treatment | Outcome |
|---|---|
| IST (hATG + CsA + Eltrombopag) | 70–80% hematologic response; 5-yr survival 60–85% |
| IST long-term | 15-yr overall survival ~60%; failure-free survival <50% |
| Matched sibling HSCT | ~90% survival in children |
| Alternative donor HSCT | Improving outcomes; haploidentical + post-transplant Cy promising |
Sources:
- Harrison's Principles of Internal Medicine, 22nd Ed. — Chapter 107
- Goldman-Cecil Medicine, International Ed. — Chapter 151
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