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: 1.25). 05/2002; 7(2):69-74. DOI: 10.1080/10245330290028560
Source: PubMed


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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    • "Such low number of positive cases may be also due to in vivo lysis of cells showing altered expression of GPI complex and problems with the detection of single cells. It is worth to remember that the clone with features of PNH can be found in the blood of patients with myeloor lymphoproliferative disorders [7] [11]. Moreover, the presence of CD55-negative and CD59-negative cell clone (most commonly only one marker is lacking) is considered to be a good prognostic factor in myelodysplastic syndromes. "
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    ABSTRACT: PNH is a rare clonal disorder of hematopoietic stem cells, therefore all blood cells lineages are involved. The main feature is an increased sensitivity of erythrocytes to complement-mediated cell lysis due to deficiency of membrane-bound GPI (glycosylphosphatidylinositol)-anchored proteins which normally function as inhibitors of reactive hemolysis. In the present study, we performed flow cytometric analysis using monoclonal antibodies against CD55 and CD59 for the detection of PNH-type clone in the blood of 50 patients (28 females and 22 males, age range 7-67 yrs). In one patient only we found a large population (95%) of granulocytes with decreased expression of both CD55 and CD59 molecules (type I PNH) and in two others with partial loss of CD55 expression (type II PNH). The expression was determined chiefly on granulocytes which in the control group showed reliable and high expression of CD55 and CD59.
    Full-text · Article · Feb 2005 · Folia Histochemica et Cytobiologica
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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.
    Full-text · Article · Jan 2003 · HAEMA
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    ABSTRACT: A 27-year-old Greek male presented to the outpatient department because of progressive weakness and fatigue, dyspnoea on slight exertion and fever. Fever started seven days before, reached 38.5 oC, often peaking twice daily; there were also chills, sweating, and sometimes dry cough. The administration of ampicilline initially and cefuroxime afterwards had no effect. His past medical history was unremarkable. Physical examination on admission revealed only pallor without any sign of infection. There were no hepatosplenomegaly or lymphadenopathy. The body temperature was 37.6 oC, the pulse rate was 125/min and the blood pressure was 110/70 mmHg. His hematological tests showed severe normochromic and normocytic anemia (Ht 19.8%, Hb 6.4 g/dl), leukopenia (white blood cells 1.2x109/l; differential count: neutrophils 18%, lymphocytes 76%, monocytes 5% and eosinophils 1%) and a mild thrombocytopenia with large platelets (78x109/l) although MPV was within normal range. There were no immature forms of red or white series in the blood smears. The reticulocyte count was 0.1%. Coombs reaction was negative and serum haptoglobins’ levels were normal as well as the coagulation parameters. The erythrocyte sedimentation rate was 38 mm/1h. Serum biochemistry was as follows: SGOT 50 IU/l, SGPT 52 IU/l, LDH 263 IU/l, γGT 29 IU/l, ALP 128 IU/l, Bil 1.12 mg/dl, Fe 180 μg/dl. Serum proteins, ferritin, B12 and follate levels, serum electrophoresis and quantitative analysis of immunoglobulins were within normal range. Chest X-rays revealed no abnormality. Blood and urine cultures were unable to detect a bacterial or fungus infection. IgM antibodies titres for EBV, HCV, HSV and VZV were not elevated while IgM antibodies for CMV were positive (titre: 1/480). The tests for rheumatoid factor, antinuclear antibodies, LE cells and cryoglobulins were negative. Antibodies against brucella were not detected. No malaria parasites were present in the peripheral blood smears. Tuberculin skin test was negative. Stool examination for ova and parasites was negative. The abdominal ultrasound was normal. The bone marrow aspirate showed a marked hypoplasia with erythroblastopenia and reduced number of myeloid elements while small foci of sparse cellularity composed mainly of mature lymphocytes were observed. A bone marrow biopsy was performed and showed only yellowish white material, consisting chiefly of fat, fibrous tissue and polyclonal lymphocytes. The detection of CD55 and CD59 red cell populations, using sephacryl gel microtyping system, revealed a 10% double erythrocytic negativity for the above antigens. Although no bacteria were detected cefotaxime, gentamycin and metronidazole were administered and the fever was controlled after three days. Four units of packed erythrocytes were transfused for the correction of anemia. After the febrile episode the administration of the appropriate therapy for basic disorder was effective as the blood cell count normalized and the clinical condition of the patient was excellent.
    Full-text · Article · Jan 2003
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