{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,10,22]],"date-time":"2023-10-22T05:40:03Z","timestamp":1697953203557},"reference-count":23,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2006,7,11]],"date-time":"2006-07-11T00:00:00Z","timestamp":1152576000000},"content-version":"vor","delay-in-days":6735,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1988,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The capacity of granulopoietic precursor cells (CFU\u2010GM) to differentiate in vitro was evaluated in five children with congenital neutropenia using short\u2010term colony assays and long\u2010term marrow cultures. In all five children, methylcellulose assays revealed normal numbers of CFU\u2010GM, which displayed an appropriate response to various sources of GM\u2010CSF and differentiated up to the polymorphonuclear leukocyte state (PMN). In contrast, neutrophil PMN were not observed in long\u2010term bone marrow cultures from three patients, despite a normal production of CFU\u2010GM, myeloblasts, and promyelocytes during the 5\u20106\u2010week culture period. Thus, in these patients, the characteristic \u201cblock\u201d in granulocytic maturation observed in vivo was reproduced in vitro in long\u2010term cultures. Granulocytic differentiation proceeded normally in long\u2010term cultures from the two other patients, thus indicating heterogeneity in the expression of the defect. These results might indicate abnormal interactions between stromal and hematopoietic cells in long\u2010term marrow cultures from some patients with congenital neutropenia. Furthermore, our results showed some correlation between the granulocytic defect in vitro and the clinical outcome in vivo.<\/jats:p>","DOI":"10.1002\/ajh.2830270205","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T11:31:27Z","timestamp":1172057487000},"page":"93-98","source":"Crossref","is-referenced-by-count":10,"title":["Granulopoietic differentiation in long\u2010term bone marrow cultures from children with congenital neutropenia"],"prefix":"10.1002","volume":"27","author":[{"given":"Laure","family":"Coulombel","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nada","family":"Morardet","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Florence","family":"Veber","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christine","family":"Leroy","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francoise","family":"Mielot","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alain","family":"Fisher","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fran\u00e7oise","family":"Teillet\u2010Thiebaud","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gil","family":"Tchernia","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claude","family":"Griscelli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claude","family":"Parmentier","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":"10.1111\/j.1651-2227.1975.tb03847.x"},{"key":"e_1_2_1_3_2","doi-asserted-by":"crossref","first-page":"396","DOI":"10.1542\/peds.59.3.396","article-title":"Abnormal in vitro granulopoiesis in phenotypically normal parents of some children with congenital neutropenia","volume":"59","author":"Rich K","year":"1977","journal-title":"Pediatrics"},{"key":"e_1_2_1_4_2","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1182\/blood.V38.1.74.74","article-title":"In vitro induction of myeloid proliferation and maturation in infantile genetic agranulocytosis","volume":"38","author":"Barak Y","year":"1971","journal-title":"Blood"},{"key":"e_1_2_1_5_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_6_2","first-page":"18","article-title":"Granulopoiesis in infantile genetic agranulocytosis","volume":"16","author":"Olofsson T","year":"1976","journal-title":"In vitro cloning of marrow cells in agar culture. Scand J Haematol"},{"key":"e_1_2_1_7_2","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1542\/peds.59.5.739","article-title":"Evaluation of congenital neutropenic disorders by in vitro bone marrow cultures","volume":"59","author":"Falk P","year":"1977","journal-title":"Pediatrics"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830080310"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830200304"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.70.3.669"},{"key":"e_1_2_1_11_2","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1182\/blood.V49.3.425.425","article-title":"Congenital neutropenia: An intrinsic cell defect demonstrated by electron microscopy of soft agar colonies","volume":"49","author":"Zucker\u2010Franklin J","year":"1977","journal-title":"Blood"},{"key":"e_1_2_1_12_2","doi-asserted-by":"crossref","first-page":"723","DOI":"10.1182\/blood.V46.5.723.723","article-title":"Congenital neutropenia: Neutrophil proliferation with abnormal maturation","volume":"46","author":"Parmley RT","year":"1975","journal-title":"Blood"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.77.8.4756"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1002\/jcp.1040910303"},{"key":"e_1_2_1_15_2","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1182\/blood.V62.2.291.291","article-title":"Enzymatic treatment of longterm human marrow cultures reveals the preferential location of primitive hemopoietic progenitors in the adherent layer","volume":"62","author":"Coulombel L","year":"1983","journal-title":"Blood"},{"key":"e_1_2_1_16_2","first-page":"231","article-title":"Methylcellulose culture of human granu\u2010locytic progenitor cells (CFC): Results of bone marrow and blood cultures of normal subjects","volume":"21","author":"Parmentier C","year":"1979","journal-title":"Nouv Rev Franc Hematol"},{"key":"e_1_2_1_17_2","first-page":"327","article-title":"Survival of granulocytic progenitors in the non\u2010adherent and adherent compartments of human long\u2010term marrow cultures","volume":"12","author":"Slovick FT","year":"1984","journal-title":"Exp Hematol"},{"key":"e_1_2_1_18_2","first-page":"273","volume-title":"Long\u2010Term Bone Marrow Culture","author":"Salvado AJ","year":"1984"},{"key":"e_1_2_1_19_2","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1016\/S0308-2261(21)00153-3","article-title":"Cell interactions in vitro","volume":"8","author":"Dexter TM","year":"1979","journal-title":"Clin Haematol"},{"key":"e_1_2_1_20_2","doi-asserted-by":"crossref","first-page":"724","DOI":"10.1182\/blood.V58.4.724.724","article-title":"Human granulocytes generated in continuous bone marrow culture are physiologically normal","volume":"58","author":"Greenberg HM","year":"1981","journal-title":"Blood"},{"issue":"1","key":"e_1_2_1_21_2","first-page":"81a","article-title":"Characterization of the specific defect in a patient with congenital agranulocytosis (Kostman's syndrome)","volume":"66","author":"Weisenburger D","year":"1985","journal-title":"Blood"},{"issue":"1","key":"e_1_2_1_22_2","first-page":"334","article-title":"Severe congenital neutropenia: Spontaneous correction of the maturation arrest in long\u2010term bone marrow cultures","volume":"64","author":"Hutchinson R","year":"1984","journal-title":"Blood"},{"key":"e_1_2_1_23_2","doi-asserted-by":"crossref","first-page":"399","DOI":"10.1182\/blood.V26.4.399.399","article-title":"The cellular basis of the genetically determined hemopoietic defect in anemic mice of the genotype SI\/SId","volume":"26","author":"McCulloch EA","year":"1965","journal-title":"Blood"},{"key":"e_1_2_1_24_2","doi-asserted-by":"publisher","DOI":"10.1038\/269412a0"}],"container-title":["American Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajh.2830270205","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.2830270205","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,21]],"date-time":"2023-10-21T08:40:29Z","timestamp":1697877629000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajh.2830270205"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1988,2]]},"references-count":23,"journal-issue":{"issue":"2","published-print":{"date-parts":[[1988,2]]}},"alternative-id":["10.1002\/ajh.2830270205"],"URL":"https:\/\/doi.org\/10.1002\/ajh.2830270205","archive":["Portico"],"relation":{},"ISSN":["0361-8609","1096-8652"],"issn-type":[{"value":"0361-8609","type":"print"},{"value":"1096-8652","type":"electronic"}],"subject":[],"published":{"date-parts":[[1988,2]]}}}