Red Cells with Paroxysmal Nocturnal Hemoglobinuria-phenotype in Patients with Acute Leukemia

First Department of Internal Medicine, University of Athens School of Medicine, Laiko General Hospital, Agiou Thoma 17, 11527, Athens, Greece.
Hematology (Impact Factor: 1.19). 05/2002; 7(2):69-74. DOI: 10.1080/10245330290028560
Source: PubMed

ABSTRACT CD55 and CD59 are complement regulatory proteins that are linked to the cell membrane via a glycosyl-phosphatidylinositol anchor. They are reduced mainly in paroxysmal nocturnal hemoglobinuria (PNH) and in other hematological disorders. However, there are very few reports in the literature concerning their expression in patients with acute leukemias (AL). We studied the CD55 and CD59 expression in 88 newly diagnosed patients with AL [65 with acute non-lymphoblastic leukemia (ANLL) and 23 with acute lymphoblastic leukemia (ALL)] using the sephacryl gel test, the Ham and sucrose lysis tests and we compared the results with patients' clinical data and disease course. Eight patients with PNH were also studied as controls. Red cell populations deficient in both CD55 and CD59 were detected in 23% of ANLL patients (especially of M(0), M(2) and M(6) FAB subtypes), 13% of ALL and in all PNH patients. CD55-deficient erythrocytes were found in 6 ANLL patients while the expression of CD59 was decreased in only 3 patients with ANLL. No ALL patient had an isolated deficiency of these antigens. There was no correlation between the existence of CD55 and/or CD59 deficiency and the percentage of bone marrow infiltration, karyotype or response to treatment. However no patient with M(3), M(5), M(7) subtype of ANLL and mature B- or T-cell ALL showed a reduced expression of both antigens. The deficient populations showed no alteration after chemotherapy treatment or during disease course. This study provides evidence about the lower expression of CD55 and CD59 in some AL patients and the correlation with their clinical data. The possible mechanisms and the significance of this phenotype are discussed.

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    ABSTRACT: Paroxysmal nocturnal haemoglobinuria (PNH) has been inaccurately viewed as a late com- plication of aplastic anaemia due to insensitivity of Ham and sucrose lysis tests. Patients with AA show a deficiency of glycosyl-phosphatidylinositol (GPI)-ancored proteins (PNH phenotype) on their periph- eral blood at a proportion of between 10%-60%, at diagnosis. This deficiency usually affects a small proportion of the studied blood cells. In serial studies, PNH phenotype did not change up to 15 years after diagnosis. We present here a case of a 27-year-old patient with acquired AA, who presented with a high proportion of PNH-like red cell population, which was decreased after immunosuppressive treat- ment.
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    ABSTRACT: Paroxysmal nocturnal hemoglobinuria (PNH) is a hemolytic, clonal and acquired disorder of the hematopoietic stem cell with a deficiency of all glycophosphatidyl-inositol (GPI) linked proteins. The aim of this retrospective study was to analyse haematological and biochemical data from 152 patients referred to our laboratory for diagnosis of PNH by flow cytometry (FC). Patients and healthy donor (152 and 99 respectively) were studied. Ham, sucrose, lactate dehydrogenase (LDH), Iron, haptoglobin (Hp), blood cell morphology and Kaplow cytochemical stain for leukocyte alkaline phosphatase (LAP) were carried out. GPI-proteins anti-CD55 and CD59 in erythrocytes and the former, plus anti CD16b and CD66b on neutrophils were evaluated by FC. Anemia and/or leukopenia and/or thrombocytopenia, increased reticulocyte count and LDH were observed in patients with PNH clone. Some of them had dacriocytes, schistocytes. LAP was low. On average, we detected 50% CD59 (-) erythrocytes and 29, 83, 78% CD55/59 (-), CD16b (-), CD66b (-) neutrophils, respectively. Paroxysmal nocturnal hemoglobinuria clone was detected in 20/152 patients. Negative population's percentages were high in patients with classic PNH, Hematimetry, LAP and adequate use of CF contribute to PNH clone detection in the laboratory.
    International journal of laboratory hematology 11/2013; 36(2). DOI:10.1111/ijlh.12156 · 1.87 Impact Factor
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    ABSTRACT: Background Complement has the potential to provoke severe impairment to host tissues, as shown in autoimmune diseases where complement activation has been associated with diminished CD55 and/or CD59 expression on peripheral blood cell membranes. The aim of this study was to evaluate the presence of CD55- and/or CD59-deficient erythrocytic populations in patients with different rheumatic diseases and to investigate possible correlations with clinical or laboratory parameters. Material and Methods CD55 and CD59 expression was evaluated in erythrocytes of 113 patients with rheumatic diseases, 121 normal individuals, and 10 patients with paroxysmal nocturnal hemoglobinuria (PNH) using the Sephacryl gel microtyping system. Ham and sucrose tests were also performed. Results Interestingly, the majority of patients (104/113, 92%) demonstrated CD55- and/or CD59-deficient erythrocytes: 47 (41.6%) with concomitant deficiency of CD55 and CD59, 50 (44.2%) with isolated deficiency of CD55, and 6 (6.2%) with isolated deficiency of CD59. In normal individuals, only 2 (1%) had concomitant CD55/CD59 negativity and 3 (2%) had isolated CD55 or CD59 deficiency. All PNH patients exhibited simultaneous CD55/CD59 deficiency. Positive Ham and sucrose tests were found only in PNH patients. There was no association between the CD55- and/or CD59-deficient erythrocytes and hemocytopenias or undergoing treatment. However, CD55 expression significantly influenced hemoglobin values (F=6.092, p=0.015). Conclusions This study provides evidence supporting the presence of erythrocytes with CD55 and/or CD59 deficiency in patients with rheumatic diseases. Moreover, CD55 deficiency on red cells influences hemoglobin concentration. Further studies using molecular techniques will clarify the exact pathophysiological mechanisms of this deficiency.
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