Radiation-free reduced-intensity hematopoietic stem cell transplantation with in vivo T-cell depletion from matched related and unrelated donors for Fanconi anemia: prognostic factor analysis

Fanconi anemia (FA) is a genetically and phenotypically heterogeneous autosomal or X ‐linked recessive disorder, clinically characterized by congenital anomalies and progressive bone marrow failure (BMF), which is the main life-threatening problem in these patients [1] Hypersensitivity to pro-inflammatory cytokines and intolerance to oxidative stress lead to apoptotic contraction of the stem cell pool and subsequently BMF in FA patients [2]. Predisposition to developing clonal cytogenetic abnormalities, myelodysplasia, acute myeloid leukemia (AML), and solid tumors (especially squamous cell carcinoma) because of inhibition of apoptotic and activating cell survival pathways a lso affects the survival of these patients [3].
Source: Experimental Hematology - Category: Hematology Authors: Tags: Article Source Type: research