What causes autoimmune neutropenia?

Updated: Sep 18, 2018
  • Author: Christopher D Braden, DO; Chief Editor: Emmanuel C Besa, MD  more...
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Autoimmune neutropenia is the neutrophil analogue of autoimmune hemolytic anemia and of idiopathic thrombocytopenic neutropenia. It should be considered in the absence of any of the common causes. Antineutrophil antibodies have been demonstrated in these patients. Autoimmune neutropenia may be associated with the following:

  • Rheumatoid arthritis (with or without Felty syndrome)

  • Sjögren syndrome

  • Chronic, autoimmune hepatitis

  • Systemic lupus erythematosus

  • Thymoma

  • Goodpasture disease

  • Granulomatosis with polyangiitis (Wegener granulomatosis)

  • Pure red blood cell (RBC) aplasia, in which there is complete disappearance of granulocyte tissue from the bone marrow; pure RBC dysplasia is a rare disorder due to the presence of antibody-mediated, granulocyte-macrophage colony forming unit (GM-CFU) inhibitory activity, and it is often associated with thymoma

  • Transfusion reactions, which can be caused by the surface antigens of neutrophilia; recipients of repeated granulocyte transfusions could become alloimmunized

  • Large granular lymphocyte proliferation or leukemia

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