Stem cell defect in aplastic anemia

1999 ◽  
pp. 3-20 ◽  
Author(s):  
Judith C.W. Marsh ◽  
Nydia G. Testa
2002 ◽  
Vol 3 (5) ◽  
pp. 230-236 ◽  
Author(s):  
Sian Rizzo ◽  
John Scopes ◽  
Modupe O Elebute ◽  
Helen A Papadaki ◽  
Edward C Gordon-Smith ◽  
...  

Hematology ◽  
2014 ◽  
Vol 2014 (1) ◽  
pp. 77-81 ◽  
Author(s):  
Corey Cutler

Abstract Allogeneic hematopoietic stem cell transplantation (HSCT) for myelodysplastic syndrome (MDS) is a potentially curative procedure, but is associated with a significant risk of morbidity and mortality. With the recent approval of disease-modifying agents, the appropriate timing of allogeneic HSCT needs to be addressed. Similarly, the optimal use of these disease-modifying agents before HSCT needs to be determined. In severe aplastic anemia, HSCT is a proven cure, but HLA-matched sibling donors are found in fewer than 25% of newly diagnosed patients. The use of early unrelated donor HSCT is an evolving concept that will become more accepted as improvements in HSCT outcomes continue.


2011 ◽  
Vol 9 (2) ◽  
pp. 229-235
Author(s):  
Phillip Scheinberg

Overall survival in severe aplastic anemia has markedly improved in the past four decades due to advances in stem cell transplantation, immunosuppressive therapies and supportive care. Horse anti-thymocyte globulin plus cyclosporine is the standard immunosuppressive regimen in severe aplastic anemia, and often employed as initial therapy as most are not candidates for a matched related stem cell transplantation. With this regimen, hematologic response can be achieved in 60 to 70% of cases, but relapse is observed in 30 to 40% of responders and clonal evolution in 10 to 15% of patients. Efforts to improve outcomes beyond horse anti-thymocyte globulin plus cyclosporine have been disappointing, with no significant improvement in the critical parameter of hematologic response, which strongly correlates with long-term survival in severe aplastic anemia. Furthermore, rates of relapse and clonal evolution have also not improved with the development of three drug regimens or with more lymphocytotoxic therapies. Therefore, horse anti-thymocyte globulin plus cyclosporine remains the standard immunosuppression of choice as first therapy in severe aplastic anemia. Interestingly, survival has markedly improved over the years in large part due to better anti-infective therapy and more successful salvage therapies with immunosuppression and stem cell transplantation. In this review general aspects of diagnosis and management are discussed.


Author(s):  
Wang Jun ◽  
Feng Jianfei ◽  
Wang Wei ◽  
Hu Yu ◽  
Zhao Xuelian ◽  
...  

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