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We present the prevalence of cancer in WHIMS patients based on data from the French Severe Chronic Neutropenia Registry and an exhaustive literature review. The median follow\u2010up of the 14 WHIMS \u2018patients was 28.5\u2009years. A central review and viral evaluation of pathological samples were organized, and we conducted a thorough literature review to identify all reports of WHIMS cases. Six French patients were diagnosed with cancer at a median age of 37.6\u2009years. The 40\u2010year risk of malignancy was 39% (95% confidence interval [CI]: 6%\u201374%). We observed two human papillomavirus (HPV)\u2010induced vulvar carcinomas, three lymphomas (two Epstein\u2013Barr virus [EBV]\u2010related) and one basal cell carcinoma. Among the 155 WHIMS cases from the literature, 22 cancers were reported in 16 patients, with an overall cancer 40\u2010year risk of 23% (95% CI: 13%\u201339%). Malignancies included EBV\u2010associated lymphoproliferative disorders and HPV\u2010positive genital and anal cancers as in the French cohort. Worldwide, nine cases of malignancy were associated with HPV and four with EBV. Immunocompromised WHIMS patients appear to be particularly susceptible to developing early malignancy, mainly HPV\u2010induced carcinomas, followed by EBV\u2010related lymphomas.<\/jats:p>","DOI":"10.1111\/bjh.19373","type":"journal-article","created":{"date-parts":[[2024,3,5]],"date-time":"2024-03-05T19:59:18Z","timestamp":1709668758000},"page":"1383-1392","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["<scp>\n                      <i>CXCR4<\/i>\n                      WHIM\n                    <\/scp>\n                    syndrome is a cancer predisposition condition for virus\u2010induced malignancies"],"prefix":"10.1111","volume":"204","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-4313-5812","authenticated-orcid":false,"given":"Cl\u00e9mentine","family":"Moulin","sequence":"first","affiliation":[{"name":"Universit\u00e9 Paris\u2010Cit\u00e9, INSERM U1160, Institut de Recherche Saint\u2010Louis Paris 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chroniques, APHP, H\u00f4pital Trousseau Paris Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alain","family":"Fischer","sequence":"additional","affiliation":[{"name":"Centre de r\u00e9f\u00e9rence des d\u00e9ficits immunitaires h\u00e9r\u00e9ditaires, Unit\u00e9 d'Immuno\u2010H\u00e9matologie P\u00e9diatrique, H\u00f4pital Necker Enfants Malades, APHP Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie","family":"Durin","sequence":"additional","affiliation":[{"name":"Centre de r\u00e9f\u00e9rence des neutrop\u00e9nies chroniques, Registre des neutrop\u00e9nies chroniques, APHP, H\u00f4pital Trousseau Paris Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dana","family":"Ranta","sequence":"additional","affiliation":[{"name":"Service d'h\u00e9matologie, CHU Nancy Nancy 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