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We describe a 73-year-old female carrier with an overt CGD phenotype due to skewed lyonisation, complicated by macrophage activation syndrome (MAS)\/haemophagocytic\u2009lymphohistiocytosis (HLH) secondary to <jats:italic>Burkholderiacepacia<\/jats:italic> complex septicaemia that was successfully treated with a combination of three antibiotics, an antifungal, granulocyte colony stimulating factor, intravenous immune globulin (IVIG) and ciclosporin. Fully phenotypic immunodeficiency is possible in X-linked CGD carriers when skewed lyonisation occurs, rendering such patients to all the same sequelae of CGD such as MAS\/HLH. MAS\/HLH should be thoroughly excluded when evaluating \u2018cepacia syndrome\u2019 in non-CGD patients.<\/jats:p>","DOI":"10.1136\/bcr-2019-230434","type":"journal-article","created":{"date-parts":[[2019,8,31]],"date-time":"2019-08-31T07:35:10Z","timestamp":1567236910000},"page":"e230434","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":10,"title":["Macrophage activation syndrome\/haemophagocytic lymphohistiocytosis secondary to <i>Burkholderia cepacia<\/i> complex septicaemia in an elderly female carrier of X-linked chronic granulomatous disease with extreme lyonisation: \u2018cepacia syndrome\u2019 revisited"],"prefix":"10.1136","volume":"12","author":[{"given":"Nicol\u00e1s","family":"Urriola","sequence":"first","affiliation":[{"name":"Immunology, Campbelltown Hospital, Campbelltown, New South Wales, 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