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Unrelated hematopoietic stem cell transplantation in a patient with congenital dyserythropoietic anemia and iron overload
Author(s) -
Buchbinder David,
Nugent Diane,
Vu Dan,
Soni Amit,
Stites Jill,
Hsieh Loan,
Puthenveetil Geetha
Publication year - 2012
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/j.1399-3046.2010.01414.x
Subject(s) - medicine , ineffective erythropoiesis , hematopoietic stem cell transplantation , anemia , transplantation , erythropoiesis , haematopoiesis , stem cell , chelation therapy , gastroenterology , thalassemia , genetics , biology
Buchbinder D, Nugent D, Vu D, Soni A, Stites J, Hsieh L, Puthenveetil G. Unrelated hematopoietic stem cell transplantation in a patient with congenital dyserythropoietic anemia and iron overload. Abstract: CDA is a heterogenous group of disorders that result in morphologically abnormal erythroid maturation and ineffective erythropoiesis. Curative therapy for CDA focuses on the use of HSCT using fully matched sibling donors. This is the first report of a Type II CDA patient with severe iron overload who was successfully treated with HSCT using a HLA‐matched unrelated donor after aggressive chelation therapy. Given the challenges of HSCT in any patient with CDA and severe iron overload, the role of novel approaches to iron chelation and HSCT is discussed.