Figure 5.

CXCR4 antagonist plerixafor has shown promise in Phase 1 trials in patients with WHIM syndrome. A, Structure of the bicyclam plerixafor. B, Plerixafor durably increases the absolute neutrophil count in WHIM patients. This figure is reproduced from Reference [49]. C, Plerixafor treatment of WHIM patients was associated with clinical improvement. Photographs show clinical manifestation from two patients before and approximately 6 months after twice daily treatment with plerixafor, including partial regression of a heavy wart burden on the hand, reduced myelokathexis in bone marrow and resolved chronic inflammatory lower leg lesions. The ‘before’ photograph of the leg in panel C is reproduced from Reference [87]; the after photograph of the leg shows discoloration at sites of previous inflammation, probably due to hemosiderin deposition that had not yet cleared. See details in Reference [87].