{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,15]],"date-time":"2026-03-15T10:41:03Z","timestamp":1773571263798,"version":"3.50.1"},"reference-count":14,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2006,7,11]],"date-time":"2006-07-11T00:00:00Z","timestamp":1152576000000},"content-version":"vor","delay-in-days":7861,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1985,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Congenital dysgranulopietic neutropenia (CDN) is a recently proposed entity that describes a small subgroup of children with clinically severe neutropenia. We followed and studied a 3\u2010year\u2010old girl with neutropenia (&lt;500\/mm<jats:sup>3<\/jats:sup>) and recurrent severe infections in whom repeated marrow evaluations revealed large (30\u201050 \u03bcm) multinucleated promyelocytes to polymorphonuclear cells with as many as 4 to 16 nuclei or nuclear lobes, respectively. In addition to the nuclear endoreduplication, ultrastructural and cytochemical evaluation of these cells demonstrated abnormalities in granule genesis and centriole structure. Concomitantly. immunoperoxidase staining indicated that many of the granules were devoid of lactoferrin but not lysozyme. In vitro proliferation studies revealed normal to increased thymidine labeling, normal numbers of colony\u2010forming cells, and normal colony\u2010stimulating activity from blood and marrow mononuclear cells, findings consistent with ineffective myelopoiesis. However, serum folate, B<jats:sub>12<\/jats:sub>, and lysozyme levels were normal. The nuclear and cytoplasmic abnormalities in this patient result in an extreme example of CDN, distinct from previously described cases.<\/jats:p>","DOI":"10.1002\/ajh.2830180109","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T10:04:11Z","timestamp":1172052251000},"page":"59-71","source":"Crossref","is-referenced-by-count":14,"title":["Severe congenital neutropenia with unique features of dysgranulopoiesis"],"prefix":"10.1002","volume":"18","author":[{"given":"Alton L.","family":"Lightsey","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richard T.","family":"Parmley","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"suffix":"Jr.","given":"William L.","family":"Marsh","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aruna K.","family":"Garg","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"William J.","family":"Thomas","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Baruch","family":"Wolach","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laurence A.","family":"Boxer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,7,11]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V56.3.465.465"},{"key":"e_1_2_1_3_2","first-page":"257","article-title":"HEMPAS: Congenital dyserythropoietic anemia (Type II)","volume":"42","author":"Verwilghen RL","year":"1973","journal-title":"Quart J Med"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V6.12.1274.1274"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1177\/14.4.291"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1177\/12.2.115"},{"key":"e_1_2_1_7_2","first-page":"221","volume-title":"Principles and Techniques of Electron Microscopy","author":"Salpeter MM","year":"1972"},{"key":"e_1_2_1_8_2","first-page":"249","volume-title":"Hemopoietic Cellular Proliferation","author":"Robinson WA","year":"1970"},{"key":"e_1_2_1_9_2","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1182\/blood.V37.1.1.1","article-title":"Colony formation by normal and leukemic marrow cells in culture: Effect of conditioned medium from human leukocytes","volume":"37","author":"Iscove NN","year":"1971","journal-title":"Blood"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.169.3946.691"},{"key":"e_1_2_1_11_2","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1182\/blood.V47.4.531.531","article-title":"Granulopoiesis in severe congenital neutropenia","volume":"47","author":"Amato D","year":"1976","journal-title":"Blood"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830090110"},{"key":"e_1_2_1_13_2","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1182\/blood.V44.4.469.469","article-title":"Azurophil and specific granules of blood neutrophils in chronic myelogenous leukemia: An ultrastructural and cytochemical analysis","volume":"44","author":"Ullyot JL","year":"1974","journal-title":"Blood"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.91.3.131s"},{"key":"e_1_2_1_15_2","first-page":"395","article-title":"Impaired microtubule function correctable by cyclic GMP and cholinergic agonists in the Chediak\u2010Higashi syndrome","volume":"85","author":"Oliver JM","year":"1976","journal-title":"Am J Pathol"}],"container-title":["American Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajh.2830180109","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.2830180109","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,20]],"date-time":"2023-10-20T21:28:01Z","timestamp":1697837281000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajh.2830180109"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1985,1]]},"references-count":14,"journal-issue":{"issue":"1","published-print":{"date-parts":[[1985,1]]}},"alternative-id":["10.1002\/ajh.2830180109"],"URL":"https:\/\/doi.org\/10.1002\/ajh.2830180109","archive":["Portico"],"relation":{},"ISSN":["0361-8609","1096-8652"],"issn-type":[{"value":"0361-8609","type":"print"},{"value":"1096-8652","type":"electronic"}],"subject":[],"published":{"date-parts":[[1985,1]]}}}