Reticular dysgenesis

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Reticular dysgenesis
Classification and external resources
ICD-10 D81.0

Reticular dysgenesis is a rare genetic disorder of the bone marrow resulting in complete absence of granulocytes and decreased number of abnormal lymphocytes. Production of red blood cells (erythrocytes) and megakaryocytes (platelet precursors) is not affected. There is also poor development of the secondary lymphoid organs.

Reticular dysgenesis is the most severe form of severe combined immunodeficiency (SCID). The cause of reticular dysgenesis is the inability of granulocyte precursors to form granules secondary to mitochondrial adenylate kinase 2 malfunction.

Further reading[edit]