Gennaio 2009 - Volume XXVIII - numero 1

Medico e Bambino


Pillole: per capire

Neutropenia da mielocatessi

Raffaele Badolato

Clinica Pediatrica, Università di Brescia

Indirizzo per corrispondenza: badolato@med.unibs.it

NEUTROPENIA DUE TO MYELOKATHEXIS

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.

Vuoi citare questo contributo?

R. Badolato
Neutropenia da mielocatessi
Medico e Bambino 2009;28(1):35-38 https://www.medicoebambino.com/?id=0901_35.pdf


leggi l'articolo in formato PDF