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English Wikipedia
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The incidence of leukemia and mortality from sepsis in patients with severe congenital neutropenia receiving long-term G-CSF therapy Rosenberg PS; Alter BP; Bolyard AA; Bonilla MA; Boxer LA; Cham B; Fier C; Freedman M; Kannourakis G; Kinsey S; Schwinzer B; Zeidler C; Welte K; Dale DCBlood 2006[Jun]; 107 (12): 4628-35In patients with severe congenital neutropenia (SCN), sepsis mortality is reduced by treatment with granulocyte colony-stimulating factor (G-CSF), but myelodsyplastic syndrome and acute myeloid leukemia (MDS/AML) have been reported. We studied 374 patients with SCN and 29 patients with Shwachman-Diamond syndrome (SDS) on long-term G-CSF enrolled in the Severe Chronic Neutropenia International Registry. In SCN, sepsis mortality was stable at 0.9% per year. The hazard of MDS/AML increased significantly over time, from 2.9% per year after 6 years to 8.0% per year after 12 years on G-CSF. After 10 years, the cumulative incidence was 8% for sepsis mortality and 21% for MDS/AML. A subgroup of SCN patients (29%) received more than the median dose of G-CSF (> or = 8 microg/kg/d), but achieved less than the median absolute neutrophil count (ANC) response (ANC < 2.188 x 10(9)/L [2188/microL] at 6-18 months). In these less-responsive patients, the cumulative incidence of adverse events was highest: after 10 years, 40% developed MDS/AML and 14% died of sepsis, compared with 11% and 4%, respectively, of more responsive patients whose ANC was above the median on doses of G-CSF below the median. Risk of MDS/AML may be similar in SDS and SCN. In less-responsive SCN patients, early hematopoietic stem cell transplantation may be a rational option.|*Registries[MESH]|Female[MESH]|Follow-Up Studies[MESH]|Granulocyte Colony-Stimulating Factor/*administration & dosage/adverse effects[MESH]|Humans[MESH]|Incidence[MESH]|Leukemia, Myeloid, Acute/blood/drug therapy/etiology/*mortality[MESH]|Leukocyte Count[MESH]|Male[MESH]|Myelodysplastic Syndromes/blood/drug therapy/etiology/*mortality[MESH]|Neutropenia/blood/complications/congenital/drug therapy/*mortality[MESH]|Retrospective Studies[MESH]|Risk Factors[MESH]|Sepsis/blood/drug therapy/etiology/*mortality[MESH]|Survival Rate[MESH] |
