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04-Jan-2001
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Arch Hellen Med, 17(4), July-August 2000, 383-389
ORIGINAL PAPER
Detection of CD55 and/or CD59 deficient red cell populations
in patients with aplastic anemia and myelodysplastic syndromes
E. TERPOS,1 M. SAMARKOS,1 C.
MELETIS,2 V. KOMNINAKA,1
E. APOSTOLIDOU,1 O. BENOPOULOU,1 K. KOROVESIS,1
D. MAVROGIANNI,1
K. ANARGYROU,1 N. VINIOU,1 E. VARIAMI,1
K. KONSTANTOPOULOS,1 J. MELETIS1
1First
Department of Internal Medicine, University of Athens, School of Medicine, Laiko
General Hospital, Athens
2Department of Electrical and Computer Engineering, National Technical
University of Athens, Greece
OBJECTIVE Paroxysmal
nocturnal hemoglobinuria (PNH) is an acquired clonal stem cell disorder characterized
by intravascular hemolysis, venous thrombosis, marrow hypoplasia, frequent episodes
of infection and, rarely, leukemic conversion. At the cellular level PNH is
characterized by the decrease or absence of glycosylphosphatidylinositol (GPI)-anchored
molecules, such as CD55 and CD59, on the cell surface. Paroxysmal nocturnal
hemoglobinuria-like clones have been described in various hematological disorders.
The link between PNH and aplastic anemia (AA) is established but the relationship
of PNH with myelodysplastic syndromes (MDS) remains unclear.
METHOD In this study the presence of CD55
and/or CD59 defective (PNH-like) red cell populations was evaluated in 19 patients
with AA, 118 with MDS and 7 with PNH, and in 121 healthy blood donors, using
the sephacryl gel test microtyping system.
RESULTS Red cell populations deficient in
both molecules CD55 and CD59 were detected in 36.8% of AA patients, in 17.7%
of MDS patients and in all PNH patients. CD55 deficient red cell populations
were found in 15.7% of patients with AA and in 12.7% of patients with MDS. CD59
deficient populations were found in 10.5% of patients with AA and in 2.5% of
patients with MDS.
CONCLUSIONS These results indicate an association
between PNH, AA and MDS. Further investigation is necessary to elucidate the
mechanisms of this association, and to define classification criteria for borderline
cases, where diagnosis is difficult.
Key words: Aplastic anemia, CD55 (DAF), CD59 (MIRL), GPI-anchor proteins, Myelodysplastic syndromes, Paroxysmal nocturnal hemoglobinuria.