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The C-X-C chemokine receptor type 4 (CXCR4) plays a crucial role in modulating cell trafficking in hematopoietic stem cells and clonal B-cells. CXCR4 mutations are found in 28% of patients with lymphoplasmacytic lymphoma (Waldenstrom macroglobulinemia, WM) and are either absent or present in only 7% of patients with other B-cell lymphomas. It is a driver mutation of tumor progression and modulator of drug resistance in lymphoplasmacytic lymphoma. Germline mutations in the C-terminus of CXCR4 have also been reported in warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome, which is a rare, autosomal dominant, primary immunodeficiency disorder.