{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T17:30:35Z","timestamp":1790875835907,"version":"4.1.0"},"reference-count":50,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2003,1,28]],"date-time":"2003-01-28T00:00:00Z","timestamp":1043712000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2003,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Severe chronic neutropenia (SCN) is defined as an absolute neutrophil (ANC) of less than 0.5 \u00d7 10<jats:sup>9<\/jats:sup>\/L, lasting for months or years. Congenital, cyclic, and idiopathic neutropenia are principal categories of SCN. Since 1994, the Severe Chronic Neutropenia International Registry (SCNIR) has collected data to monitor the clinical course, treatments, and disease outcomes for SCN patients. This report summarizes data for 853 patients, almost all treated with daily or alternate\u2010day recombinant human granulocyte colony\u2010stimulating factor (G\u2010CSF or Filgrastim). G\u2010CSF treatment increased the ANC overall from 0.34 \u00d7 10<jats:sup>9<\/jats:sup>\/L \u00b1 0.018 pre\u2010treatment to 3.70 \u00d7 10<jats:sup>9<\/jats:sup>\/L \u00b1 0.18 during the first year of treatment. For most patients, the responses were durable with patients remaining on the same dose of G\u2010CSF for many years. Long\u2010term hematological observations showed stable mean leukocyte and neutrophil counts and gradually increasing hemoglobin levels. Thrombocytopenia developed in 4% of patients. As of January 1, 2000, myelodysplasia (MDS) or acute myelogenous leukemia (AML) has occurred in 35 of 387 patients with congenital neutropenia with a cumulative risk of 13% after 8 years of G\u2010CSF treatment. This event occurred without a predictable relationship to the duration or dose of G\u2010CSF treatment. No patients with cyclic or idiopathic neutropenia developed MDS or AML. Other important adverse events included hepatomegaly, osteoporosis, vasculitis, glomerulonephritis, and deaths in 4 of 14 cases requiring splenectomy. Growth and development and the outcome of pregnancy appeared to be unaffected by G\u2010CSF treatment. These data indicate that congenital, cyclic, and idiopathic neutropenia can be effectively treated with long\u2010term G\u2010CSF. The risk of leukemia, osteoporosis, other potentially adverse events, and pregnancy outcome need to be further evaluated with continuing long\u2010term observations. Am. J. Hematol. 72:82\u201393, 2003. \u00a9 2003 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajh.10255","type":"journal-article","created":{"date-parts":[[2003,1,29]],"date-time":"2003-01-29T15:36:05Z","timestamp":1043854565000},"page":"82-93","source":"Crossref","is-referenced-by-count":301,"title":["Severe chronic neutropenia: Treatment and follow\u2010up of patients in the Severe Chronic Neutropenia International Registry"],"prefix":"10.1002","volume":"72","author":[{"given":"David C.","family":"Dale","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tammy E.","family":"Cottle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carol J.","family":"Fier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Audrey 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