[MIM 193 670]

Acronym for Warts Hypogammaglobulinemia Infections Myelokathexis.

(Warts - hypogammaglobulinemia - infections - myelokathexis)

Prevalence: < 1/106. Autosomal dominant (usually) transmission of a mutation of gene CXCR4. This produces a form of immune deficiency associating in varying ways : chronic neutropenia, myelokathesis  (abnormal retention of mature neutrophils in the bone marrow) and hypogammaglobulinemia. These patients are very sensitive to human papillomavirus infections (PVH virus, warts, mucosal cancers) and to bacterial infections.

Long term complications : bronchiectasis and COPD ; ano-genital cancers.

A few published cases associated with Tetralogy of Fallot.

Anesthetic implications:

neutropenia, strict asepsis

References:

-        Bock I, Dugué F, Loppinet E, Bellanné-Chantelot C, Bénet B.
Diagnostic de WHIM évoqué devant l’observation de myélokathexis.
Ann Biol Clin 2014 ; 72 : 111-9.

Updated: November 2017