Cancers and Screening Risks Associated With WHIM Syndrome (Chapter 3)

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Jean Donadieu, MD, Service d'Hémato-Oncologie Pédiatrique, Hopital, Paris, discusses cancer and screening risks associated with WHIM syndrome.

In addition to infections and warts, patients with WHIM syndrome may be prone to an increased risk of cancer (carcinoma), particularly when HPV warts are located in the anogenital region, although there have been reports of cancers in other parts of the body such as oral cavity and vulva.

Researchers conducting a long-term follow-up of 18 patients with WHIM syndrome observed HPV-related malignancy in three patients (17%). Although HPV vaccination may be considered (patients with WHIM syndrome can generate protective antibodies with active immunization, but seem to lose this protection over time.

Other non–HPV related malignancies have been reported in patients with WHIM syndrome. A 2019 report from China described a patient with acute myeloid leukemia that developed following Hodgkin’s lymphoma. The French registry reported that of a 14-patient cohort, five developed malignancy at median age of 37 years (2 HPV-induced vulvar cancers, 2 lymphomas [1 bone, 1 skin lymphoma, 1 basal-cell carcinoma]). The 40-year rate of malignancy was calculated to be 46%.

Beyond malignancies and the threat of recurrent infections, patients with WHIM may be subject to additional long-term complications. For example, recurrent otitis media infection can predispose these patients to perforated ear drums and hearing loss. Lung damage resulting from frequent infections can cause chronic bronchiectasis or other serious respiratory problems,[5] and evidence exists that the frequency of irreversible, end-organ damage (e.g., bronchiectasis) may be underestimated.
Category
Oncology
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