{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,5]],"date-time":"2026-09-05T06:09:37Z","timestamp":1788588577176,"version":"build-2803163510"},"reference-count":29,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2023,2,1]],"date-time":"2023-02-01T00:00:00Z","timestamp":1675209600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Deutsche Forschungsgemeinschaft and Friedrich-Alexander-Universit\u00e4t Erlangen-N\u00fcrnberg"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>(1) Background: Systemic mastocytosis is a rare, non-curable disease with potential life-threatening complications in patients receiving cardiac surgery. (2) Methods: This systematic review of the literature was prompted by the case of a life-threatening anaphylactic reaction during cardiac surgery related to systemic mastocytosis. The search of all types of studies, using several databases (Pubmed, Scopus and Web of Science), was conducted through September 2022 to identify the relevant studies. (3) Results: Twelve studies were included describing cases of patients undergoing cardiac surgery who were diagnosed with systemic mastocytosis. An adverse effect, namely anaphylaxis, has happened in three cases. Different strategies of premedication, intraoperative and postoperative management were used. In our case, the patient was admitted for elective biological aortic valve replacement due to severe aortic stenosis. Intraoperatively, the patient developed an anaphylactic shock during the administration of protamine after separation from the cardiopulmonary bypass. This anaphylaxis reaction was a complication of the pre-existing systemic mastocytosis and could be successfully managed by the administration of epinephrine, antihistamines and corticosteroids. (4) Conclusions: This systematic literature search and case report highlight the importance of careful preoperative planning, as well as coordination between cardiac surgeons, anesthesiologists and hemato-oncological specialists, in patients with rare but complication-prone diseases such as systemic mastocytosis.<\/jats:p>","DOI":"10.3390\/jcm12031156","type":"journal-article","created":{"date-parts":[[2023,2,1]],"date-time":"2023-02-01T06:49:36Z","timestamp":1675234176000},"page":"1156","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["A Practical Approach to Systemic Mastocytosis Complications in Cardiac Surgery: A Case Report and Systematic Review of the Literature"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9541-9888","authenticated-orcid":false,"given":"Mathieu N.","family":"Suleiman","sequence":"first","affiliation":[{"name":"Department of Cardiac Surgery, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, 91054 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valeska","family":"Brueckl","sequence":"additional","affiliation":[{"name":"Department of Haematology and Oncology, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, 91054 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8713-9114","authenticated-orcid":false,"given":"J\u00f6rg","family":"Fechner","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, 91054 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ann-Sophie","family":"Kaemmerer","sequence":"additional","affiliation":[{"name":"Department of Cardiac Surgery, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, 91054 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florian","family":"Wilk","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, 91054 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Weyand","sequence":"additional","affiliation":[{"name":"Department of Cardiac Surgery, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, 91054 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1817-4207","authenticated-orcid":false,"given":"Frank","family":"Harig","sequence":"additional","affiliation":[{"name":"Department of Cardiac Surgery, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, 91054 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,2,1]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1111\/all.12617","article-title":"Drug hypersensitivity in clonal mast cell disorders: ENDA\/EAACI position paper","volume":"70","author":"Bonadonna","year":"2015","journal-title":"Allergy"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"2342","DOI":"10.1038\/leu.2016.190","article-title":"Splenomegaly, elevated alkaline phosphatase and mutations in the SRSF2\/ASXL1\/RUNX1 gene panel are strong adverse prognostic markers in patients with systemic mastocytosis","volume":"30","author":"Jawhar","year":"2016","journal-title":"Leukemia"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"508","DOI":"10.1002\/ajh.26118","article-title":"Systemic mastocytosis in adults: 2021 Update on diagnosis, risk stratification and management","volume":"96","author":"Pardanani","year":"2021","journal-title":"Am. J. Hematol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1111\/j.1540-8191.2011.01404.x","article-title":"Aortic valve replacement in patients with systemic mastocytosis","volume":"27","author":"Wanamaker","year":"2012","journal-title":"J. Card. Surg."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"753","DOI":"10.1097\/ALN.0000000000000031","article-title":"Perioperative management of patients with mastocytosis","volume":"120","author":"Dewachter","year":"2014","journal-title":"Anesthesiology"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1213\/XAA.0000000000000474","article-title":"Kounis Syndrome During Anesthesia: Presentation of Indolent Systemic Mastocytosis: A Case Report","volume":"8","year":"2017","journal-title":"A A Case Rep."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"398","DOI":"10.1097\/00000542-197304000-00018","article-title":"Anesthetic considerations in mastocytosis","volume":"38","author":"Rosenbaum","year":"1973","journal-title":"Anesthesiology"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"446","DOI":"10.1097\/01.mph.0000212950.12856.d4","article-title":"Mast cell disease: Surgical and anesthetic implications","volume":"28","author":"Damodar","year":"2006","journal-title":"J. Pediatr. Hematol. Oncol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1025","DOI":"10.1053\/j.jvca.2014.08.006","article-title":"Aneurysm Repair in a Patient With Systemic Mastocytosis","volume":"29","author":"Duggal","year":"2015","journal-title":"J. Cardiothorac. Vasc. Anesth."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1002\/ccr3.505","article-title":"Systemic mastocytosis presenting as cardiac tamponade with CD25(+) pericardial mast cells","volume":"4","author":"Sukrithan","year":"2016","journal-title":"Clin. Case Rep."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"880","DOI":"10.1053\/j.jvca.2018.08.210","article-title":"Mitral Valve Replacement in a Patient With Systemic Mastocytosis","volume":"33","author":"Montes","year":"2019","journal-title":"J. Cardiothorac. Vasc. Anesth."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"392","DOI":"10.1213\/XAA.0000000000001093","article-title":"Undiagnosed Systemic Mastocytosis Presenting as Postoperative Distributive Shock: A Case Report","volume":"13","author":"Ripoll","year":"2019","journal-title":"A A Pract."},{"key":"ref_13","first-page":"54","article-title":"Case report on aortic valve replacement in adult woman with systemic mastocytosis (RCD code: VIII)","volume":"3","author":"Hryniewiecki","year":"2017","journal-title":"J. Rare Cardiovasc. Dis."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"555649","DOI":"10.19080\/JAICM.2018.04.555649","article-title":"Cardiac Bypass Surgery in a Patient with an Indolent Systemic Mastocytosis","volume":"4","author":"Beckers","year":"2018","journal-title":"J. Anesth. Intensive Care Med."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"689","DOI":"10.1016\/j.healun.2008.03.006","article-title":"Heart transplantation in a patient with systemic mastocytosis","volume":"27","author":"Moro","year":"2008","journal-title":"J. Heart Lung Transplant."},{"key":"ref_16","first-page":"346","article-title":"Anaphylactic risk due to systemic mastocytosis: Perioperative management in cardiac surgery","volume":"66","year":"2019","journal-title":"Rev. Esp. Anestesiol. Reanim."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1051\/ject\/19861812","article-title":"Cardiopulmonary Bypass for A Patient with Systemic Mastocytosis","volume":"18","author":"Trubov","year":"1986","journal-title":"J. Extra-Corpor. Technol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1016\/j.iac.2014.01.003","article-title":"Epidemiology, prognosis, and risk factors in mastocytosis","volume":"34","author":"Brockow","year":"2014","journal-title":"Immunol. Allergy Clin. N. Am."},{"key":"ref_19","unstructured":"AROCK OPM (2022, September 24). Systemic Mastocytosis. Available online: https:\/\/www.orpha.net\/consor\/cgi-bin\/OC_Exp.php?lng=en&Expert=2467."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1016\/j.jaip.2018.09.024","article-title":"The Data Registry of the European Competence Network on Mastocytosis (ECNM): Set Up, Projects, and Perspectives","volume":"7","author":"Valent","year":"2019","journal-title":"J. Allergy Clin. Immunol. Pract."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"2391","DOI":"10.1182\/blood-2016-03-643544","article-title":"The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia","volume":"127","author":"Arber","year":"2016","journal-title":"Blood"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1420","DOI":"10.1182\/blood-2016-09-731893","article-title":"Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts","volume":"129","author":"Valent","year":"2017","journal-title":"Blood"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1111\/bjh.14967","article-title":"How we diagnose and treat systemic mastocytosis in adults","volume":"180","author":"Scherber","year":"2018","journal-title":"Br. J. Haematol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1239","DOI":"10.1007\/s00101-009-1625-x","article-title":"Mastocytosis: A challenge in anaesthesiology","volume":"58","author":"Konrad","year":"2009","journal-title":"Anaesthesist"},{"key":"ref_25","doi-asserted-by":"crossref","unstructured":"Seidel, H., Hertfelder, H.J., Oldenburg, J., Kruppenbacher, J.P., Afrin, L.B., and Molderings, G.J. (2021). Effects of Primary Mast Cell Disease on Hemostasis and Erythropoiesis. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms22168960"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1111\/j.1423-0410.2010.01339.x","article-title":"Comparison of fresh frozen plasma and prothrombin complex concentrate for the reversal of oral anticoagulants in patients undergoing cardiopulmonary bypass surgery: A randomized study","volume":"99","author":"Demeyere","year":"2010","journal-title":"Vox Sang."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"804","DOI":"10.1053\/j.jvca.2011.03.004","article-title":"Recombinant Activated Factor VII Increases Stroke in Cardiac Surgery: A Meta-analysis","volume":"25","author":"Ponschab","year":"2011","journal-title":"J. Cardiothorac. Vasc. Anesth."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1026","DOI":"10.1111\/all.12437","article-title":"Anaphylaxis: Guidelines from the European Academy of Allergy and Clinical Immunology","volume":"69","author":"Muraro","year":"2014","journal-title":"Allergy"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"n71","DOI":"10.1136\/bmj.n71","article-title":"The PRISMA 2020 statement: An updated guideline for reporting systematic reviews","volume":"372","author":"Page","year":"2021","journal-title":"BMJ"}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/12\/3\/1156\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T18:21:07Z","timestamp":1760120467000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/12\/3\/1156"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,2,1]]},"references-count":29,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2023,2]]}},"alternative-id":["jcm12031156"],"URL":"https:\/\/doi.org\/10.3390\/jcm12031156","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,2,1]]}}}