[Experimental therapy of hypoplastic paroxysmal nocturnal hemoglobinuria]

Immun Infekt. 1995 Apr;23(2):65-6.
[Article in German]

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) and aplastic anemia are associated either as a PNH-aplasia syndrome or the emergence of glycosylphosphatidylinositol-(GPI)deficient blood cells in patients with severe aplastic anemia (SAA). It could be demonstrated that SAA patients with GPI-deficient cells in comparison to those without have a worse response to classical immunosuppressive therapy. Therefore, we treated a female PNH patient with severe thrombocytopenia, anemia and granulopenia with G-CSF and cyclosporine. Within 8 weeks, a trilineage response of hematopoiesis was observed. In addition, the proportion of normal to GPI-deficient granulocytes and monocytes increased significantly.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antigens, CD / metabolism
  • Antigens, CD34
  • CD59 Antigens
  • Cyclosporine / administration & dosage*
  • Female
  • Glycosylphosphatidylinositols / deficiency
  • Granulocyte Colony-Stimulating Factor / administration & dosage*
  • Hemoglobinuria, Paroxysmal / drug therapy*
  • Humans
  • Membrane Glycoproteins / metabolism
  • Thrombocytopenia / drug therapy

Substances

  • Antigens, CD
  • Antigens, CD34
  • CD59 Antigens
  • Glycosylphosphatidylinositols
  • Membrane Glycoproteins
  • Granulocyte Colony-Stimulating Factor
  • Cyclosporine