{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T15:26:35Z","timestamp":1781105195830,"version":"3.54.1"},"reference-count":0,"publisher":"National Academy of Sciences","issue":"13","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[1989,7]]},"abstract":"<jats:p>Severe combined immunodeficiency disease (SCID) is a congenital disorder of severe B- and T-lymphocyte dysfunction in which several pathogenic mechanisms have been identified. The present study describes a female child with SCID who had a primary defect in the ability of T cells to secrete interleukin 2 (IL-2). B- and T-cell numbers were normal, but their functions were severely deficient. Mitogen and antigen-driven lymphoproliferative responses were diminished but were correctable in vitro with recombinant IL-2 (rIL-2). The patient's phytohemagglutinin-stimulated lymphocytes expressed IL-2 receptors normally. Despite the presence of the gene for IL-2, the patient's cells were grossly deficient in messenger RNA for IL-2 and endogenous IL-2 production. Pokeweed mitogen-driven B-cell differentiation was decreased and was not corrected by the addition of normal T cells to the B cells. Two attempts at immune reconstitution by haploidentical bone marrow transplantation failed. Therapy with rIL-2 (30,000 units\/kg, given daily i.v.) resulted in marked clinical improvement as well in improved T-cell functions. The child, now 3 yr old, has been on rIL-2 therapy for 2 yr and receives rIL-2 (30,000 units\/kg) three times weekly at home. This case study points to a new direction in the treatment of such disorders with rIL-2.<\/jats:p>","DOI":"10.1073\/pnas.86.13.5069","type":"journal-article","created":{"date-parts":[[2006,5,31]],"date-time":"2006-05-31T06:58:01Z","timestamp":1149058681000},"page":"5069-5073","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":80,"title":["Recombinant interleukin 2 therapy in severe combined immunodeficiency disease."],"prefix":"10.1073","volume":"86","author":[{"given":"R","family":"Pahwa","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Schneider Children's Hospital of Long Island Jewish Medical Center, New Hyde Park, NY 11042."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T","family":"Chatila","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Schneider Children's Hospital of Long Island Jewish Medical Center, New Hyde Park, NY 11042."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S","family":"Pahwa","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Schneider Children's Hospital of Long Island Jewish Medical Center, New Hyde Park, NY 11042."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C","family":"Paradise","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Schneider Children's Hospital of Long Island Jewish Medical Center, New Hyde Park, NY 11042."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N K","family":"Day","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Schneider Children's Hospital of Long Island Jewish Medical Center, New Hyde Park, NY 11042."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R","family":"Geha","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Schneider Children's Hospital of Long Island Jewish Medical Center, New Hyde Park, NY 11042."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S A","family":"Schwartz","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Schneider Children's Hospital of Long Island Jewish Medical Center, New Hyde Park, NY 11042."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H","family":"Slade","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Schneider Children's Hospital of Long Island Jewish Medical Center, New Hyde Park, NY 11042."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N","family":"Oyaizu","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Schneider Children's Hospital of Long Island Jewish Medical Center, New Hyde Park, NY 11042."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R A","family":"Good","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Schneider Children's Hospital of Long Island Jewish Medical Center, New Hyde Park, NY 11042."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[1989,7]]},"container-title":["Proceedings of the National Academy of Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/pnas.org\/doi\/pdf\/10.1073\/pnas.86.13.5069","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,4,13]],"date-time":"2022-04-13T12:55:13Z","timestamp":1649854513000},"score":1,"resource":{"primary":{"URL":"https:\/\/pnas.org\/doi\/full\/10.1073\/pnas.86.13.5069"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1989,7]]},"references-count":0,"journal-issue":{"issue":"13","published-print":{"date-parts":[[1989,7]]}},"alternative-id":["10.1073\/pnas.86.13.5069"],"URL":"https:\/\/doi.org\/10.1073\/pnas.86.13.5069","relation":{},"ISSN":["0027-8424","1091-6490"],"issn-type":[{"value":"0027-8424","type":"print"},{"value":"1091-6490","type":"electronic"}],"subject":[],"published":{"date-parts":[[1989,7]]},"assertion":[{"value":"1989-07-01","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}