Section editor
	Benjamin Tillman, MD Vanderbilt University Nashville, TN, USA LinkedIn

WHIM: Warts, Hypogammaglobulinemia, Infections, Myelokathexis

Last updated on 2024-09-06: 2 regimens on this page 2 variants on this page

All lines of therapy

Mavorixafor monotherapy

Study	Dates of enrollment [xx]	Evidence	Comparator	Comparative Efficacy
Badolato et al. 2024 (4WHIM)	2019-10-24 to 2021-09-09	Phase 3 (E-esc)	Placebo	Superior time (hours) above ANC threshold 500/uL

Mavorixafor (Xolremdi) by the following age- and weight-based criteria:
- Adults: 400 mg PO once per day
- Adolescents more than 50 kg: 400 mg PO once per day
- Adolescents up to 50 kg: 200 mg PO once per day

Continued indefinitely

4WHIM: Badolato R, Alsina L, Azar A, Bertrand Y, Bolyard AA, Dale D, Deyà-Martínez À, Dickerson KE, Ezra N, Hasle H, Kang HJ, Kiani-Alikhan S, Kuijpers TW, Kulagin A, Langguth D, Levin C, Neth O, Olbrich P, Peake J, Rodina Y, Rutten CE, Shcherbina A, Tarrant TK, Vossen MG, Wysocki CA, Belschner A, Bridger GJ, Chen K, Dubuc S, Hu Y, Jiang H, Li S, MacLeod R, Stewart M, Taveras AG, Yan T, Donadieu J. A phase 3 randomized trial of mavorixafor, a CXCR4 antagonist, for WHIM syndrome. Blood. 2024 Jul 4;144(1):35-45. link to original article link to PMC article dosing details in manuscript have been reviewed by our editors PubMed NCT03995108

Study	Dates of enrollment	Evidence
McDermott et al. 2019	Not reported	Pilot, fewer than 20 pts

Various durations

Case series: McDermott DH, Pastrana DV, Calvo KR, Pittaluga S, Velez D, Cho E, Liu Q, Trout HH 3rd, Neves JF, Gardner PJ, Bianchi DA, Blair EA, Landon EM, Silva SL, Buck CB, Murphy PM. Plerixafor for the treatment of WHIM syndrome. N Engl J Med. 2019 Jan 10;380(2):163-170. link to original article link to PMC article dosing details in manuscript have been reviewed by our editors PubMed