Article
Myelodysplasia in autosomal dominant and sporadic monocytopenia immunodeficiency syndrome: diagnostic features and clinical implications.
Haematologica - 1 Aug 2011
Calvo Katherine R, Vinh Donald C, Maric Irina, Wang Weixin, Noel Pierre, Stetler-Stevenson Maryalice, Arthur Diane C, Raffeld Mark, Dutra Amalia, Pak Evgenia, Myung Kyungjae, Hsu Amy P, Hickstein Dennis D, Pittaluga Stefania, Holland Steven M
Abstract excerpt
A novel, genetic immunodeficiency syndrome has been recently described, herein termed "MonoMAC". It is characterized by severe circulating monocytopenia, NK- and B-lymphocytopenia, severe infections with M. avium complex (MAC), and risk of progression to myelodysplasia/acute myelogenous leukemia. Detailed bone marrow analyses performed on 18 patients further define this disorder. The majority of patients had...
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