علاج فقر الدم فانكوني مع فشل نخاع العظم الشديد أو فقر الدم اللاتنسجي (Hgb <8 غ/ديسيليتر)

References

• Aplastic anemia (hemoglobin (Hgb) <8 g/dL or absolute neutrophil count (ANC) <500/uL or platelet count <20,000/uL)
• MDS or AML
• Progressive complex cytogenetic abnormalities known to be associated with malignancy
• Patients with FA who develop persistent and severe cytopenias or evidence of MDS or AML should be considered for HCT provided the patient is not too old and has adequate organ function.
• Patients with an exceptional risk of HCT-related mortality (e.g., patients with severe organ dysfunction, those who are 35 years or older, and those with pre-existing malignancies or life-threatening systemic infections) may consider alternative treatment options first, such as the use of androgens.
• The only curative option for bone marrow failure (BMF) in patients with FA is a hematopoietic cell transplant (HCT).
• HLA 10/10 (followed by 9/10) allele-matched sibling
• HLA 10/10 (followed by 9/10) allele-matched relative other than sibling
• HLA 10/10 (followed by 9/10) allele-matched unrelated adult volunteer
• HLA 10-8/10 antigen matched UCB