EFFECT OF RECOMBINANT HUMAN GRANULOCYTE AND GRANULOCYTE-MACROPHAGE COLONY-STIMULATING FACTOR IN SHWACHMANS SYNDROME

Shwachman's syndrome is a rare congenital disorder associating exocrin e pancreatic insufficiency and bone marrow failure. Infants with this syndrome have a high morbidity related to infection because of neutrop enia. We report the case of an infant treated by recombinant human (rh ) hematopoietic growth factors: rh granulocyte colony stimulating fact or (G-CSF) and rh granulocyte-macrophage colony-stimulating factor (GM -CSF). G-CSF was infused at the daily dose of 5 mg/kg increasing absol ute neutrophil count (ANC), which reached a level >1(sic)109/1 on the 6th day of treatment, but decreased as soon as the treatment was disru pted. GM-CSF was infused at an increasing daily dose, from 5 to 15 mg/ kg. It increased ANC and its effect lasted longer after the treatment was disrupted: ANC remained above 1(sic) 109/1 for 3 weeks. No adverse effect was observed. In conclusion, these two hematopoietic growth fa ctors are able to increase the ANC and may help to treat bacterial inf ections, which can be life-threatening and even fatal.