G-CSF and cyclosporin induce an increase of normal cells in hypoplastic paroxysmal nocturnal hemoglobinuria.
Four paroxysmal nocturnal hemoglobinuria (PNH) patients with severe thrombocytopenia, hemolytic anemia and neutropenia were treated using a combination of filgrastim (G-CSF) and cyclosporin. In all patients a trilineage response of hematopoiesis was achieved. In addition, the proportion of glycosyl-phosphatidylinositol (GPI)-deficient granulocytes decreased. All patients mobilized CD34+ hematopoietic progenitors into peripheral blood after starting treatment with G-CSF. The majority of early progenitors (CD34+ CD38-) after mobilization into peripheral blood was found to be unaffected by the GPI-anchoring defect. No patient developed leukemia while under therapy. We conclude from these data that the combination of G-CSF and cyclosporin represents an efficient option for the treatment of hypoplastic PNH.
Schubert
J
J
Department of Clinical Immunology, Hannover Medical School, Germany.
Scholz
C
C
Geissler
R G
RG
Ganser
A
A
Schmidt
R E
RE
D000328:Adult; D001772:Blood Cell Count / Q000187:drug effects; D016572:Cyclosporine / Q000494:pharmacology*; D005260:Female; D005500:Follow-Up Studies; D016179:Granulocyte Colony-Stimulating Factor / Q000494:pharmacology*; D006410:Hematopoiesis / Q000187:drug effects; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000494:pharmacology; D010198:Pancytopenia / Q000188:drug therapy
D007166:Immunosuppressive Agents; D016179:Granulocyte Colony-Stimulating Factor; D016572:Cyclosporine