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CZECH MEDICAL ASSOCIATION J. Ev. PURKYNĚ |
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Česky / Czech version | Vnitř. Lék. 46, 2000, No. 6, p. 319 - 322 |
Stem Cell Transplantation for
Paroxysmal Nocturnal Hemoglobinuria Čermák J., Vítek A., Marinov I., Písačka M., Brabec V. Ústav hematologie a krevní transfuze, Praha, ředitel RNDr. MUDr. P. Jarolím, DrSc. |
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Summary: Paroxysmal nocturnal hemoglobinuria (PNH) represents a rare clonal disorder of hematopoiesis
clinically characterized by acquired hemolytic anemia, intravascular hemolysis, hemoglobinuria
and frequent occurrence of venous thrombosis. Stem cell transplantation is indicated in patients
with severe bone marrow aplasia, repeated massive hemolysis or recurrent life threatening
thrombotic complications. Almost 90 transplanted patients with PNH have been published. We
report a case of successful allogeneic peripheral blood stem cell transplantation performed in a 24
years old woman with a severe form of PNH with frequent episodes of massive intravascular
hemolysis. The patient is now alive completely engrafted 900 days after transplantation without
signs of chronic GVHD and without recurrent infections. This case represents the first successful-
ly transplanted patient with PNH in our country.
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