{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T11:21:36Z","timestamp":1790767296455,"version":"4.1.0"},"reference-count":54,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2021,9,1]],"date-time":"2021-09-01T00:00:00Z","timestamp":1630454400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2021,9,1]],"date-time":"2021-09-01T00:00:00Z","timestamp":1630454400000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Pulm. circ."],"published-print":{"date-parts":[[2021,10]]},"abstract":"<jats:p>Busulfan is widely used to treat malignant diseases, particularly for therapeutic intensification prior to an autologous stem cell graft. Numerous side effects consecutive to busulfan are described, but few descriptions of pulmonary hypertension exist, while bronchiolitis obliterans remains a rare complication. We report the clinical observations of four patients from the French Pulmonary Hypertension Registry who experienced subacute pulmonary hypertension after receiving busulfan as preparation regimen before an autologous stem cell graft for malignancies (Hodgkin\u2019s disease, Ewing\u2019s sarcoma and primary large B cell lymphoma of the brain). Patients experienced severe pulmonary arterial hypertension 2 to 4.5\u00a0months after busulfan administration. Pulmonary hypertension improved after treatment with approved drugs for pulmonary arterial hypertension and\/or corticosteroids. During the follow\u2010up period, two patients developed chronic respiratory insufficiency due to interstitial lung disease, leading to double lung transplantation. The pathological assessment of explanted lungs revealed interstitial lung fibrosis with advanced bronchiolar lesions and severe pulmonary vascular damage. Three of the four patients were still alive after 36 to 80\u00a0months and the fourth died unexpectedly and suddenly after 5\u00a0months. In conclusion, PAH is a rare but severe complication associated with busulfan chemotherapy in adults. Histological examinations provide evidence for diffuse pulmonary vascular damage combined with interstitial lung injury in most cases.<\/jats:p>","DOI":"10.1177\/20458940211030170","type":"journal-article","created":{"date-parts":[[2021,6,25]],"date-time":"2021-06-25T04:20:48Z","timestamp":1624594848000},"page":"1-12","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":11,"title":["Pulmonary hypertension associated with busulfan"],"prefix":"10.1002","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2252-4150","authenticated-orcid":false,"given":"Jean","family":"Hagenburg","sequence":"first","affiliation":[{"name":"Assistance Publique \u2013 H\u00f4pitaux de Paris (AP\u2010HP) Department of Respiratory and Intensive Care Medicine Pulmonary Hypertension National Referral Center H\u00f4pital Bic\u00eatre Le Kremlin\u2010Bic\u00eatre France"},{"name":"Universit\u00e9 Paris\u2010Saclay School of Medicine Le Kremlin\u2010Bic\u00eatre France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurent","family":"Savale","sequence":"additional","affiliation":[{"name":"Assistance Publique \u2013 H\u00f4pitaux de Paris (AP\u2010HP) Department of Respiratory and Intensive Care Medicine Pulmonary Hypertension National Referral Center H\u00f4pital Bic\u00eatre Le Kremlin\u2010Bic\u00eatre France"},{"name":"Universit\u00e9 Paris\u2010Saclay School of Medicine Le Kremlin\u2010Bic\u00eatre France"},{"name":"INSERM UMR_S 999\u00a0Pulmonary Hypertension: Pathophysiology and Novel Therapies H\u00f4pital Marie Lannelongue Le Plessis\u2010Robinson France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benoit","family":"Lechartier","sequence":"additional","affiliation":[{"name":"Assistance Publique \u2013 H\u00f4pitaux de Paris (AP\u2010HP) Department of Respiratory and Intensive Care Medicine Pulmonary Hypertension National Referral Center H\u00f4pital Bic\u00eatre Le Kremlin\u2010Bic\u00eatre France"},{"name":"Universit\u00e9 Paris\u2010Saclay School of Medicine Le Kremlin\u2010Bic\u00eatre France"},{"name":"INSERM UMR_S 999\u00a0Pulmonary Hypertension: Pathophysiology and Novel Therapies H\u00f4pital Marie Lannelongue Le Plessis\u2010Robinson France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria\u2010Rosa","family":"Ghigna","sequence":"additional","affiliation":[{"name":"INSERM UMR_S 999\u00a0Pulmonary Hypertension: Pathophysiology and Novel Therapies H\u00f4pital Marie Lannelongue Le Plessis\u2010Robinson France"},{"name":"Service d\u2019anatomopathologie H\u00f4pital Marie Lannelongue Le Plessis Robinson France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie\u2010Camille","family":"Chaumais","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris\u2010Saclay School of Medicine Le Kremlin\u2010Bic\u00eatre France"},{"name":"INSERM UMR_S 999\u00a0Pulmonary Hypertension: Pathophysiology and Novel Therapies H\u00f4pital Marie Lannelongue Le Plessis\u2010Robinson France"},{"name":"Assistance Publique H\u00f4pitaux de Paris Service de Pharmacie H\u00f4pital Bic\u00eatre Le Kremlin Bic\u00eatre France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xavier","family":"Ja\u00efs","sequence":"additional","affiliation":[{"name":"Assistance Publique \u2013 H\u00f4pitaux de Paris (AP\u2010HP) Department of Respiratory and Intensive Care Medicine Pulmonary Hypertension National Referral Center H\u00f4pital Bic\u00eatre Le Kremlin\u2010Bic\u00eatre France"},{"name":"Universit\u00e9 Paris\u2010Saclay School of Medicine Le Kremlin\u2010Bic\u00eatre France"},{"name":"INSERM UMR_S 999\u00a0Pulmonary Hypertension: Pathophysiology and Novel Therapies H\u00f4pital Marie Lannelongue Le Plessis\u2010Robinson France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Sitbon","sequence":"additional","affiliation":[{"name":"Assistance Publique \u2013 H\u00f4pitaux de Paris (AP\u2010HP) Department of Respiratory and Intensive Care Medicine Pulmonary Hypertension National Referral Center H\u00f4pital Bic\u00eatre Le Kremlin\u2010Bic\u00eatre France"},{"name":"Universit\u00e9 Paris\u2010Saclay School of Medicine Le Kremlin\u2010Bic\u00eatre France"},{"name":"INSERM UMR_S 999\u00a0Pulmonary Hypertension: Pathophysiology and Novel Therapies H\u00f4pital Marie Lannelongue Le Plessis\u2010Robinson France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc","family":"Humbert","sequence":"additional","affiliation":[{"name":"Assistance Publique \u2013 H\u00f4pitaux de Paris (AP\u2010HP) Department of Respiratory and Intensive Care Medicine Pulmonary Hypertension National Referral Center H\u00f4pital Bic\u00eatre Le Kremlin\u2010Bic\u00eatre France"},{"name":"Universit\u00e9 Paris\u2010Saclay School of Medicine Le Kremlin\u2010Bic\u00eatre France"},{"name":"INSERM UMR_S 999\u00a0Pulmonary Hypertension: Pathophysiology and Novel Therapies H\u00f4pital Marie Lannelongue Le Plessis\u2010Robinson France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9358-6922","authenticated-orcid":false,"given":"David","family":"Montani","sequence":"additional","affiliation":[{"name":"Assistance Publique \u2013 H\u00f4pitaux de Paris (AP\u2010HP) Department of Respiratory and Intensive Care Medicine Pulmonary Hypertension National Referral Center H\u00f4pital Bic\u00eatre Le Kremlin\u2010Bic\u00eatre France"},{"name":"Universit\u00e9 Paris\u2010Saclay School of Medicine Le Kremlin\u2010Bic\u00eatre France"},{"name":"INSERM UMR_S 999\u00a0Pulmonary Hypertension: Pathophysiology and Novel Therapies H\u00f4pital Marie Lannelongue Le Plessis\u2010Robinson France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,9]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbmt.2015.07.032"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbmt.2014.05.003"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bulcan.2017.10.018"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bulcan.2017.06.020"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1345\/aph.10071"},{"key":"e_1_2_10_7_1","first-page":"3","article-title":". [Busulfan\u2010induced pneumopathy].","volume":"4","author":"Massin F","year":"1987","journal-title":"Rev Mal Respir"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.2174\/157488608783333899"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01913-2018"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01032-2015"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajpath.2014.10.021"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1038\/bmt.2016.84"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.115.014207"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00612-2019"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1634\/theoncologist.2012-0384"},{"key":"e_1_2_10_16_1","first-page":"3","article-title":"Hepatic veno\u2010occlusive disease development in the hematopoietic stem cell transplantation patients: incidence and associated risk factors, a meta\u2010analysis","author":"Xia Y","journal-title":"Eur J Gastroenterol Hepatol. Epub ahead of print"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01887-2018"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00228-011-1209-y"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.transproceed.2010.04.024"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1093\/cvr\/cvp215"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0030897"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.183.1.139"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1165\/rcmb.2009-0389TR"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02032091"},{"key":"e_1_2_10_25_1","first-page":"1007","article-title":"Reversible pulmonary hypertension in POEMS syndrome \u2013 another etiology of triggered pulmonary vasculopathy?","volume":"16","author":"Paciocco G","year":"2000","journal-title":"Can J Cardiol"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1136\/pgmj.58.679.282"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1080\/15376516.2016.1243601"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.120.1.151"},{"key":"e_1_2_10_29_1","first-page":"49","article-title":"Allogeneic bone marrow transplantation in high\u2010risk myeloid disorders using busulfan, cytosine arabinoside and cyclophosphamide (BAC).","volume":"9","author":"Ratanatharathorn V","year":"1992","journal-title":"Bone Marrow Transplant"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.5045\/br.2018.53.2.145"},{"key":"e_1_2_10_31_1","first-page":"719","article-title":". [Transplant\u2010related mortality following allogeneic hematopoietic stem cell transplantation using fludarabine\/busulfan\u2010based reduced\u2010intensity conditioning \u2013 a retrospective analysis in a single institution].","volume":"42","author":"Yoshida T","year":"2015","journal-title":"Gan To Kagaku Ryoho"},{"key":"e_1_2_10_32_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12185-015-1830-0"},{"key":"e_1_2_10_33_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.23656"},{"key":"e_1_2_10_34_1","doi-asserted-by":"publisher","DOI":"10.1111\/tri.12093"},{"key":"e_1_2_10_35_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.128.1.153"},{"key":"e_1_2_10_36_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.04.00084804"},{"key":"e_1_2_10_37_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1704377"},{"key":"e_1_2_10_38_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.1998.00617.x"},{"key":"e_1_2_10_39_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.95.08081269"},{"key":"e_1_2_10_40_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V81.8.2194.2194"},{"key":"e_1_2_10_41_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-111-5-368"},{"key":"e_1_2_10_42_1","doi-asserted-by":"publisher","DOI":"10.1159\/000196183"},{"key":"e_1_2_10_43_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.10-2838"},{"key":"e_1_2_10_44_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2013.08.007"},{"key":"e_1_2_10_45_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(61)90229-7"},{"key":"e_1_2_10_46_1","doi-asserted-by":"publisher","DOI":"10.1136\/thx.23.2.187"},{"key":"e_1_2_10_47_1","doi-asserted-by":"publisher","DOI":"10.1136\/thx.25.2.257-a"},{"key":"e_1_2_10_48_1","doi-asserted-by":"publisher","DOI":"10.1136\/thx.24.6.639"},{"key":"e_1_2_10_49_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.4.5734.530"},{"key":"e_1_2_10_50_1","doi-asserted-by":"publisher","DOI":"10.3109\/10428199609054777"},{"key":"e_1_2_10_51_1","doi-asserted-by":"publisher","DOI":"10.1097\/00001813-200503000-00014"},{"key":"e_1_2_10_52_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V84.7.2144.2144"},{"key":"e_1_2_10_53_1","doi-asserted-by":"publisher","DOI":"10.1080\/17425255.2017.1360277"},{"key":"e_1_2_10_54_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1702377"},{"key":"e_1_2_10_55_1","doi-asserted-by":"publisher","DOI":"10.1183\/09059180.00003313"}],"container-title":["Pulmonary Circulation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1177\/20458940211030170","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1177\/20458940211030170","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1177\/20458940211030170","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,7,5]],"date-time":"2024-07-05T05:34:47Z","timestamp":1720157687000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1177\/20458940211030170"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,9]]},"references-count":54,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2021,10]]}},"alternative-id":["10.1177\/20458940211030170"],"URL":"https:\/\/doi.org\/10.1177\/20458940211030170","archive":["Portico"],"relation":{},"ISSN":["2045-8940","2045-8940"],"issn-type":[{"value":"2045-8940","type":"print"},{"value":"2045-8940","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,9]]}}}