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Marrow Failure and Myelodysplasia (Pre-leukemia) Program
Marrow Failure and Myeledysplasia (Pre-leukemia) Program

Selected Publications

  1. Dror Y, Freedman MH. Shwachman-Diamond syndrome is an inherited pre-leukemic bone marrow failure disorder with aberrant haematologic progenitors and faulty marrow microenvironment. Blood 1999.
  2. Dror Y, Ward A, Tauw I, Freedman MH. Combined corticosteroid/granulocyte colony-stimulating factor therapy in the treatment of severe congenital neutropenia unresponsive to G-CSF. Exp Hematol 2000.
  3. Dror Y, Leaker M, Bernstein A, Freedman MH: Mastocytosis cells bearing a c-kit activation mutation are characterized by hypersensitivity to stem cell factor and increased programmed cell death. Brit J Haematol 2000.
  4. Dror Y, Freedman MH. Marrow cells from Shwachman-Diamond syndrome patients are characterized by abnormally increased apoptosis mediated through the Fas pathway. Blood 2001.
  5. Thornley I, Dror Y, Sung L, Wynn RF, Freedman MH: Abnormal telomere shortening in leukocytes of children with Shwachman-Diamond syndrome. Brit J Haematol 2002.
  6. Dror Y, Durie P, Ginzberg H, Herman R, Banerjee A, Champagne M, Shannon K, Malkin D, Freedman MH. Clonal evolution in marrows of patients with Shwachman-Diamond syndrome. Exp Hematol 2002.
  7. Dror Y. Mitochondrial-mediated apoptosis in a myelodysplastic syndrome secondary to congenital deletion of chromosome 4p. Exp Hematol 2003.
  8. Dror Y, Freedman MH, Leaker M, Saunders FE, Doyle JJ. Nonmyeloablative hematopoietic stem-cell transplantation across human leukocyte antigen barriers in dyskeratosis congenita Bone Marrow Transplant 2003.
  9. Majeed F, Jadko S, Freedman M, Dror Y: Mutation analysis of SBDS in pediatric acute myeloblastic leukemia with and without chromosome 7 abnormalities. Paediatric Blood and Cancer 2005.
  10. Steele JM, Klassen R, Fernandez C, Yanofsky R, Wu J, Odame I, Silva M, Kaiser A, Brossard J, Samson Y, Champagne J, Abish S, Le D, Jardine L, Hand JP, Lipton J, Freedman M, Charpentier K, Stephens D, Dror Y: Disease progression in recently diagnosed patients with inherited marrow failure syndromes. Pediatr Blood Cancer 2006.
  11. Leung E, Rujkijyanont P, Beyene J, Freedman J, Dror Y. Shwachman-Diamond syndrome: an inherited model of aplastic anemia with increased angiogenesis. Brit J Haematol 2006
  12. Cheretakis C, Leung R, Sun CX, Dror Y, Glogauer M: Timing of neutrophil tissue repopulation predicts restoration of innate immune protection in a murine bone marrow transplant model. Blood 2006
  13. Rujkijyanont P, Beyene J, Kahn F, Dror Y: Leukemia-related gene expression in bone marrow cells from patients with the pre-leukemic disorder Shwachman-Diamond syndrome. Brit J Haematol 2007
  14. Calado RT, Graf SA, Wilkerson KL, Kajigaya S, Ancliff PJ, Dror Y, Chanock SJ, Lansdorp PM, Young NS: Mutations in the SBDS gene in acquired aplastic anemia. Blood 2007
  15. Pongtanakul B, Das PK, Charpentier K, Dror Y. Outcome of children with aplastic anemia treated with immunosuppressive therapy. Pediatr Blood Cancer 2007
  16. Rosenberg PS, Alter BP, Link DC, Stein S, Rodger E, Bolyard AA, Aprikyan AA, Bonilla MA, Dror Y, Kannourakis G, Newburger PE, Boxer LA, Dale DC: Neutrophil Elastase Mutations and Risk of Leukemia in Severe Congenital Neutropenia. Brit J Haematol 2008.
  17. Rujkijyanont P, Watanabe KI, Ambekar C, Wang H, Schimmer AD, Beyene J, Dror Y: Sbds-deficient cells undergo accelerated apoptosis through the Fas-pathway and not through the Bax/Bcl2/BclXL pathway. Haematologica 2008
  18. Teo JT, Klaassen R, Fernandez CV, Yanofsky R, Wu J, Champagne J, Silva M, Lipton JH, Brossard J, Samson Y, Abish S, Steele M, Ali K, Dower N, Uma Athale, Jardine L, Hand JP, Tsangaris E, Beyene B, Dror Y. Clinical and genetic analysis of unclassifiable inherited bone marrow failure syndromes. Pediatrics 2008.
  19. Ward AC, Gits J, Majeed F, Aprikyan AA, Lewis RS, Freedman M, Shigdar S, Touw IP, Dale DC, Dror Y. Functional interaction between mutations in the granulocyte colony-simulating factor receptor in severe congenital neutropenia. Brit J Haematol 2008.
  20. Tsangaris E, Adams SL, Yoon G, Chitayat D, Lansdorp P, Dokal I, Dror Y. Ataxia and pancytopenia caused by a mutation in TINF2. Human Genetics 2008.
  21. Watanabe K, Ambekar C, Wang W, Ciccolini A, Schimmer A, Dror Y. SBDS-deficiency results in specific hypersensitivity to Fas stimulation and accumulation of Fas at the plasma membrane. Apoptosis 2009.