Thachil Jecko, Caswell Mark, Bolton-Maggs Paula H B, Pizer Barry, Keenan Russell
Department of Paediatric Haematology and Oncology, Royal Liverpool Children's Hospital, Eaton Road, Liverpool, United Kingdom.
Pediatr Blood Cancer. 2008 Apr;50(4):920-1. doi: 10.1002/pbc.21295.
Severe Congenital Neutropenia is a rare condition characterized by a very low neutrophil count, which pre-disposes the affected child to recurrent bacterial infections. Treatment with granulocyte colony stimulating factor (G-CSF) has dramatically improved the prognosis of these children; but in patients who have become G-CSF refractory, hematopoeitic stem cell transplant is still the only effective curative treatment. We describe a patient who was unresponsive to escalating doses of G-CSF and underwent a successful reduced intensity conditioning, matched unrelated donor allograft resulting in cure.