WHIM syndrome; warts, hypogammaglobulinemia, infections, & myelokathexis
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Pathology
Genetics
- associated with defects in CXCR4
Clinical manifestations
- extensive human papillomavirus (HPV) infection
Laboratory
- complete blood count: neutropenia, lymphopenia
- serum protein electrophoresis: hypogammaglobulinemia
- bone marrow aspirates from affected individuals contain abundant mature myeloid cells (myelokathexis)
Complications
- life-threatening bacterial infection
- HPV
Management
More general terms
References
- ↑ OMIM https://mirror.omim.org/entry/193670
- ↑ 2.0 2.1 Ingram I FDA Approves First Drug for WHIM Syndrome CXCR4 antagonist mavorixafor effectively boosts neutrophils and lymphocytes to reduce infections. https://www.medpagetoday.com/allergyimmunology/allergy/109871