{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,10,25]],"date-time":"2023-10-25T17:10:09Z","timestamp":1698253809953},"reference-count":31,"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":4574,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1994,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>This report describes a patient with combined immune deficiency associated with congenital neutropenla (CID\/CN) and reports a partial characterization of his hematopoletic abnormalities. The CID\/CN syndrome described is characterized by neutropenla and by deficiencies in B\u2010lymphoid and T\u2010lymphoid cell number and function. Red cell and platelet counts were normal. In vitro assays indicate that the myeioid lineage was developmentally arrested at the level of the committed monocyte\/granulocyte progenitor (CFU\u2010GM), while precursors to the CFU\u2010GM progenitor were normal. In vitro studies showed that the defect in myeiold development was not corrected with G\u2010CSF or GM\u2010CSF. However, combinations of cytokines present in conditioned media from the T\u2010cell lines MO or C5MJ, or defined multiple cytokine combinations containing IL\u20101, IL\u20103, GM\u2010CSF, kit ligand, IL\u20106, and IL\u20109, restored myelopoiesis in\u2010vitro. In contrast, CBMJ\u2010conditioned media did not correct deficiencies in immune function in the patient's lymphocytes and accessory cells. No abnormalities in the production of G\u2010CSF, GM\u2010CSF, M\u2010CSF, or IL\u20101 from the patient could be identified to account for the defects in myelopoiesis orimmune function. \u00a9 1994 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajh.2830450110","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T13:51:22Z","timestamp":1172065882000},"page":"63-72","source":"Crossref","is-referenced-by-count":2,"title":["Dyshematopoiesis in combined immune deficiency with congenital neutropenia"],"prefix":"10.1002","volume":"45","author":[{"given":"Cristina","family":"Gasparetto","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Clay","family":"Smith","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Meri","family":"Firpo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dieter","family":"Dennig","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Trudy","family":"Small","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alfred P.","family":"Gillio","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert","family":"Lichtenberg","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richard J.","family":"O'Reilly","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Malcolm A. 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