Fanconi Anemia
| Autor: | Am Risitano, Eliane Gluckman, R. Peffault de Latour |
|---|---|
| Rok vydání: | 2017 |
| Předmět: |
Oncology
medicine.medical_specialty Cytopenia business.industry medicine.medical_treatment Bone marrow failure Myeloid leukemia Hematopoietic stem cell Hematopoietic stem cell transplantation medicine.disease Transplantation surgical procedures operative medicine.anatomical_structure Fanconi anemia hemic and lymphatic diseases Cord blood Internal medicine medicine business |
| DOI: | 10.1016/b978-0-12-804152-9.00016-6 |
| Popis: | Fanconi anemia (FA) is the most frequent inherited cause of bone marrow failure. Most FA patients experience hematopoietic stem cell attrition and cytopenia during childhood. Allogeneic hematopoietic stem cell transplantation (HSCT) remains the only curative treatment for FA patients in case of bone marrow failure or clonal evolution (i.e., myelodysplastic syndrome or acute myeloid leukemia). HLA identical HSCT is still the best treatment but drastic improvement has been shown using an unrelated donor. Alternative transplantation (i.e., cord blood-, mismatch-, or haplo-HSCT) is still experimental and should not be done outside experienced centers. FA diagnosis, optimal HSCT timing, best conditioning regimen, as well as, long-term follow-up of those patients are addressed in this paper. |
| Databáze: | OpenAIRE |
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