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We present two cases with rare infectious and oncological complications of CVID, including a patient with <jats:italic>Mycobacterium avium<\/jats:italic> comple<jats:italic>x-intracellular<\/jats:italic> infection and ovarian cancer, and another patient with group B <jats:italic>Streptococcus<\/jats:italic> empyema of the lung with acute myeloid leukaemia. The main objective of this study is to highlight how CVID-induced hypogammaglobulinaemia can lead to rare infections and malignancies. The management of these complications can vary according to severity, but an awareness of their existence is crucial to diagnose them promptly in an already immunocompromised CVID patient.<\/jats:p>","DOI":"10.1136\/bcr-2022-250570","type":"journal-article","created":{"date-parts":[[2022,12,2]],"date-time":"2022-12-02T09:00:24Z","timestamp":1669971624000},"page":"e250570","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Uncommon presentations of common variable immunodeficiency"],"prefix":"10.1136","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6920-9879","authenticated-orcid":false,"given":"Akankcha","family":"Alok","sequence":"first","affiliation":[{"name":"Department of Infectious Disease, University of South Florida, Tampa, Florida, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sadaf","family":"Aslam","sequence":"additional","affiliation":[{"name":"Department of Infectious Disease, University of South Florida, Tampa, Florida, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John N","family":"Greene","sequence":"additional","affiliation":[{"name":"Department of Infectious Disease, Moffitt Cancer Center, Tampa, Florida, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2022,12,2]]},"reference":[{"key":"2025082712362128000_15.12.e250570.1","first-page":"200","article-title":"Agammaglobulinemia","volume":"66","author":"Janeway","year":"1953","journal-title":"Trans Assoc Am Physicians"},{"key":"2025082712362128000_15.12.e250570.2","doi-asserted-by":"crossref","first-page":"1787","DOI":"10.1182\/blood.2019002062","article-title":"Common variable immune deficiency: case studies","volume":"134","author":"Cunningham-Rundles","year":"2019","journal-title":"Blood"},{"key":"2025082712362128000_15.12.e250570.3","doi-asserted-by":"crossref","first-page":"707","DOI":"10.1590\/S0001-37652004000400007","article-title":"Clinical and laboratory aspects of common variable immunodeficiency","volume":"76","author":"Kokron","year":"2004","journal-title":"An Acad Bras Cienc"},{"key":"2025082712362128000_15.12.e250570.4","doi-asserted-by":"crossref","first-page":"S123","DOI":"10.1016\/j.jaci.2003.12.436","article-title":"Mycobacterium simiae infection in a patient with common variable immunodeficiency","volume":"113","author":"Arora","year":"2004","journal-title":"J Allergy Clin Immunol"},{"key":"2025082712362128000_15.12.e250570.5","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-007-0527-7"},{"key":"2025082712362128000_15.12.e250570.6","doi-asserted-by":"publisher","DOI":"10.1086\/587669"},{"key":"2025082712362128000_15.12.e250570.7","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2020.01245","article-title":"Low IgA associated with oropharyngeal microbiota changes and lung disease in primary antibody deficiency","volume":"11","author":"Berbers","year":"2020","journal-title":"Front Immunol"},{"key":"2025082712362128000_15.12.e250570.8","doi-asserted-by":"crossref","first-page":"504","DOI":"10.1016\/j.jaip.2020.11.006","article-title":"COVID-19 infection in 10 common variable immunodeficiency patients in New York City","volume":"9","author":"Cohen","year":"2021","journal-title":"J Allergy Clin Immunol Pract"},{"key":"2025082712362128000_15.12.e250570.9","unstructured":"Pescador Ruschel MA , Vaqar S . 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