WHIM SYNDROME – A LITERATURE REVIEW WITH A DENTAL CONSIDERATION

*Dr. Sowmya Gujjar Vishnu Rao, Dr. Madhusudan Astekar, Dr. Kavya Mathur and Dr. Suveet Mathur

ABSTRACT

WHIM (warts, hypogammaglobulinemia, infections, and myelokathexis) syndrome is an autosomal dominant disease related to a mutation in the chemokine receptor CXCR4 resulting in altered immune function. An increased susceptibility in these patients to human papillomavirus (HPV) manifests as cutaneous warts and, in women, cervical dysplasia and squamous carcinoma. Analysis of the myeloid and plasmacytoid dendritic cell blood counts in WHIM patients revealed a striking defect in the number of plasmacytoid dendritic cells as well as a partial reduction of the number of myeloid dendritic cells. Because of the rarity of the disease and the heterogeneity in clinical presentation, diagnosis is often delayed. Here we put forward a review concerning the WHIM syndrome with its dental consideration.

Keywords: WHIM, CXCR4, autosomal dominant syndrome, human papilloma virus, warts, myelokathexis, hypogammaglobulinemia, carcinoma.