Decay-accelerating-factor-deficient erythrocytes during the long-term clinical course of patients with paroxysmal nocturnal hemoglobinuria.
We performed long-term follow-up studies of 5 patients with paroxysmal nocturnal hemoglobinuria (PNH). The percentages of decay-accelerating-factor (DAF)-deficient erythrocytes were almost stable or slowly increased during the 2-4 years' observation periods when the effects of treatment with drugs or blood transfusion and pregnancy were excluded. However, a tendency of increase in the percentages of DAF-deficient erythrocytes was observed along with the duration of the disease. Our findings suggest that the growth advantage of PNH clones beyond the normal hematopoiesis may be different from the other clonal stem cell disorders.
Mahbub
B
B
Second Department of Interanl Medicine, Shinshu University School of Medicine, Matsumoto, Japan.
Nishimura
J
J
Kitani
T
T
Kitano
K
K
D000328:Adult; D015703:Antigens, CD / Q000378:metabolism*; D018958:CD55 Antigens; D002999:Clone Cells / Q000473:pathology; D004912:Erythrocytes / Q000737:chemistry*; D005260:Female; D005500:Follow-Up Studies; D017261:Glycosylphosphatidylinositols / Q000032:analysis / Q000172:deficiency / Q000235:genetics; D006098:Granulocytes / Q000737:chemistry; D006410:Hematopoiesis; D006412:Hematopoietic Stem Cells / Q000473:pathology; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood* / Q000473:pathology; D006801:Humans; D008297:Male; D008562:Membrane Glycoproteins / Q000378:metabolism*; D008875:Middle Aged; D017452:Receptors, IgG / Q000032:analysis
D015703:Antigens, CD; D018958:CD55 Antigens; D017261:Glycosylphosphatidylinositols; D008562:Membrane Glycoproteins; D017452:Receptors, IgG