title: Paroxysmal nocturnal hemoglobinuria clone in bone marrow of patients with pancytopenia.
abstract: The lack of glycosylphosphatidylinositol (GPI)-anchored membrane proteins such as decay-accelerating factor (DAF) and CD59 on blood cells has a diagnostic value in paroxysmal nocturnal hemoglobinuria (PNH). Because PNH often develops in patients with aplastic anemia (AA), we attempted to detect a PNH clone in the bone marrow (BM) of patients with AA and pancytopenia before affected cells were evident in the peripheral blood (PB). We used flow cytometry with monoclonal antibodies against DAF and CD59 for the detection of the clone. Affected cells were observed in the BM of 3 of 7 patients with AA and 1 of 3 patients with pancytopenia of unknown origin, but not in their PB. All 8 patients with apparent PNH had affected cells in their BM and PB. On the basis of the early appearance of the PNH clone in the BM, a prospective 4-month follow-up study of the PB cells was performed. The study showed the release of affected mature cells first in granulocytes, then in monocytes, and finally in lymphocytes. Ham's test was positive before affected erythrocytes were detected by flow cytometry. Our findings indicate that detection of the PNH clone in BM could be predictive of the development of PNH in patients with AA and pancytopenia.
authors.affiliation: Second Department of Internal Medicine, Kumamoto University School of Medicine, College of Medical Science, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Nakakuma
authors.forename: S
authors.initials: S
authors.lastname: Nagakura
authors.forename: N
authors.initials: N
authors.lastname: Iwamoto
authors.forename: T
authors.initials: T
authors.lastname: Kawaguchi
authors.forename: M
authors.initials: M
authors.lastname: Hidaka
authors.forename: K
authors.initials: K
authors.lastname: Horikawa
authors.forename: T
authors.initials: T
authors.lastname: Kagimoto
authors.forename: T
authors.initials: T
authors.lastname: Shido
authors.forename: K
authors.initials: K
authors.lastname: Takatsuki
mesh_terms: D000741:Anemia, Aplastic / Q000473:pathology*; D015703:Antigens, CD / Q000032:analysis*; D015415:Biomarkers / Q000032:analysis; D001853:Bone Marrow / Q000473:pathology*; D018958:CD55 Antigens; D018957:CD59 Antigens; D002999:Clone Cells / Q000473:pathology*; D005260:Female; D005434:Flow Cytometry; D017261:Glycosylphosphatidylinositols / Q000172:deficiency; D006412:Hematopoietic Stem Cells / Q000473:pathology*; D006457:Hemoglobinuria, Paroxysmal / Q000473:pathology*; D006801:Humans; D008562:Membrane Glycoproteins / Q000032:analysis*; D008875:Middle Aged; D010198:Pancytopenia / Q000473:pathology*
chemical_list: D015703:Antigens, CD; D015415:Biomarkers; D018958:CD55 Antigens; D018957:CD59 Antigens; D017261:Glycosylphosphatidylinositols; D008562:Membrane Glycoproteins