Pillole: per capire
Clinica Pediatrica, Università di Brescia
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Key words: Myelokathexis, Neutropenia, WHIM, Hypogammaglobulinemia, Lymphocytes B
The works describes a case of WHIM (Warts, Hypogammaglobulinemia, Immunodeficit, Myelokathexis) characterized by neutropenia, hypogammaglobulinemia, reduced number of lymphocytes B (C19) in blood stream, and bone marrow rich in element of the myeloid series. The syndrome is due to a mutation of the receptor CXCR4 present both in the myeloid cells and in the limphocytes B for the ligand SDF1 placed on the stromal cells of the bone marrow and in the lymphatic tissue. The expression of the mutant receptor in neutrophyles and in the plasma cells does not stop with cell maturation, thus causing a protracted adhesion of the mature cells to the marrow stroma and lymphatic organs, with consequent myelokathexis, neutropenia and hypogammaglobulinemia.
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