Figure 3.
Treatment regimens used in WHIM patients. An exhaustive literature review of journal articles published since the first description of the disorder in 1964 identified 70 cases of WHIM syndrome. In 57 cases, CXCR4 mutations were reported, and 3 cases were defined as having the wild-type CXCR4 (see Table 2 for details). The other 10 cases were clinically diagnosed as WHIM before 2003. The treatments that were used include G-CSF or, rarely, granulocyte-macrophage colony-stimulating factor (GM-CSF), immunoglobulin replacement therapy (intravenously [IVIG] or subcutaneously [scIG]), and antibiotic prophylaxis. The association of these therapeutic strategies is reported. Occasionally, G-CSF was used for a short course during acute infection, to the study neutrophils response, or as a premedication before chemotherapy for lymphoma.