Varying populations of CD59‐negative, partly positive, and normally positive blood cells in different cell lineages in peripheral blood of paroxysmal nocturnal hemoglobinuria patients

Abstract

CD59-antigen expression on the surface membranes of erythrocytes, granulocytes, monocytes, lymphocytes, and platelets was determined by flow cytometry in 34 healthy controls and 17 patients with paroxysmal nocturnal hemoglobinuria (PNH). In all PNH patients, CD59-negative erythrocytes accounted for > 10% of the total erythrocyte population. Two erythrocyte populations (CD59-negative and normally positive or CD59-negative and partly positive), three populations (CD59-negative, partly positive, and normally positive), and one population (CD59-negative) were demonstrated in ten, six, and one patients, respectively. However, CD59-negative granulocytes did not account for > 10% of the total granulocytes in two patients, and one of them had only a CD59 normally positive granulocyte population. A particular granulocyte population extended over both CD59-negative and partly positive areas was shown in two patients. Two populations (CD59-negative and normally positive) and one population (CD59-negative) were demonstrated in monocytes and lymphocytes. CD59-negative lymphocytes accounted for > 50% of the total lymphocytes in only two patients. Three patients had a CD59 normally positive lymphocyte population. Percentages of CD59-positive platelet population in normal controls were widely various. Therefore, it was usually difficult to discriminate between PNH-affected and normal platelets. Thus, the flow cytometric profiles of CD59-antigen expression varied not only between PNH patients but between cell lineages. The present results and our prior study indicate that CD59 flow cytometry using erythrocytes and granulocytes is most suitable for diagnosing PNH.

Glycosylphosphatidyl‐inositol (GPI)‐linked protein deficiency on the platelets of patients with aplastic anaemia and paroxysmal nocturnal haemoglobinuria: two distinct patterns correlating with expression on neutrophils

“…Measuring populations of platelets deficient in GPI-linked proteins by flow cytometry is a sensitive method of studying the relationship between these proteins and thrombosis. Published works using flow cytometry show such variability of percentage CD59 expression as to make discrimination between PNH and normal platelets impossible (Fujioka & Yamada, 1994). Vu et al (1996) reported they could not discriminate between PNH II and PNH III platelets due to low mean fluorescence intensity (MFI).…”

“…Measurement of GPI-linked protein-deficient populations of platelets would therefore be important for studying the relationship with thrombosis. Previous reports of CD59 expression on platelets have been unable to discriminate between PNH and normal platelets (Fujioka & Yamada, 1994;Vu et al, 1996). In the present study, platelet CD55 and CD59 patterns of expression were analysed in patients with PNH and AA, and normal controls.…”

Hematopoietic progenitor cells in the blood and bone marrow in various hematologic disorders

“…Absence of red cell surfaces of complement regulatory proteins such as CDS9 and decay-accelerating factor (DAF) is responsible for the hypersensitivity to complement of the red cells [18,19]. DAF, CDS9 and some other membrane proteins are inserted to the red cell surface via a glycosylphosphatidylinositol (GPI) moiety.…”

The Glycosylphosphatidylinositol Anchor and Paroxysmal Nocturnal Haemoglobinuria/Aplasia Model

“…Red cells and granulocytes display complete (type III) or partial (type II) deficiency of GPI-linked proteins [30]. Type III red cells are most sensitive to the effects of complement.…”