Plerixafor on a WHIM - Promise or Fantasy of a New CXCR4 Inhibitor for This Rare, but Important Syndrome?
Skin Therapy Lett
; 27(2): 1-5, 2022 Mar.
Article
en En
| MEDLINE
| ID: mdl-35385630
ABSTRACT
Warts, Hypogammaglobulinemia, Infections and Myelokathexis (WHIM) is a primary immunodeficiency syndrome. Patients with WHIM syndrome are more susceptible to human papillomavirus (HPV) infections and commonly present to a dermatologist with recalcitrant to treatment warts. Other cardinal features of WHIM syndrome include recurrent sinopulmonary bacterial infections, neutropenia/lymphopenia, low levels of immunoglobulins (IgG, IgA, IgM) and myelokathexis. Research demonstrated that truncating gain-of-function mutations of the C-X-C chemokine receptor type 4 gene (CXCR4) are responsible for this disease. Plerixafor, a specific small molecule antagonist of CXCR4, is currently used for peripheral blood hematopoietic stem cell (HSC) mobilization in stem cell transplant recipients. It has recently shown promise for the treatment of WHIM syndrome in phase I/II clinical trials. In this paper we review the emerging patient clinical data for this medication and highlight the role of CXCR4 in other important skin diseases including keratinocyte carcinomas, psoriasis and cutaneous T-cell lymphoma.
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Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Verrugas
/
Infecciones por Papillomavirus
/
Agammaglobulinemia
/
Compuestos Heterocíclicos
/
Neutropenia
Límite:
Humans
Idioma:
En
Revista:
Skin Therapy Lett
Asunto de la revista:
DERMATOLOGIA
Año:
2022
Tipo del documento:
Article
País de afiliación:
Canadá