Lymphocyte subset analysis and glycosylphosphatidylinositol phenotype in patients with paroxysmal nocturnal hemoglobinuria

Using multicolor flow-cytometry we have examined 19 patients with paroxysmal nocturnal hemoglobinuria (PNH) (18 with active disease and 1 spontaneous remitter) to determine absolute numbers of lymphocyte subsets and the proportion of glycosylphosphatidylinositol (GPI)-deficient clones amongst these...

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Bibliographic Details
Published in:Blood 1998-09, Vol.92 (5), p.1799-1806
Main Authors: STEPHEN, RICHARDS, J, DEREK, NORFOLK, R, SWIRSKY, D. M, HILLMEN, P
Format: Article
Language:English
Subjects:
Adult
Aged
Anemias. Hemoglobinopathies
B-Lymphocytes - chemistry
Biological and medical sciences
Diseases of red blood cells
Female
Flow Cytometry
Glycosylphosphatidylinositols - blood
Glycosylphosphatidylinositols - deficiency
Hematologic and hematopoietic diseases
Hemoglobinuria, Paroxysmal - blood
Hemoglobinuria, Paroxysmal - complications
Humans
Killer Cells, Natural - chemistry
Lymphocyte Count
Lymphocyte Subsets
Lymphopenia - etiology
Male
Medical sciences
Middle Aged
Neutropenia - etiology
Phenotype
T-Lymphocytes - chemistry
Thrombocytopenia - etiology
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Summary:Using multicolor flow-cytometry we have examined 19 patients with paroxysmal nocturnal hemoglobinuria (PNH) (18 with active disease and 1 spontaneous remitter) to determine absolute numbers of lymphocyte subsets and the proportion of glycosylphosphatidylinositol (GPI)-deficient clones amongst these subpopulations. Lymphocyte subsets were abnormal in all patients; the most frequent findings were low absolute numbers of natural killer (NK) cells (median, 0.08 x 10(9)/L; normal range, 0.2 to 0.4 x 10(9)/L) and low absolute numbers of B cells (median, 0.05 x 10(9)/L; normal range, 0.06 to 0.65 x 10(9)/L). GPI-deficient B, T, and NK cells were identified in 88%, 84%, and 89% of patients, respectively. The proportion of GPI-deficient cells within individual lymphoid lineages was highly variable, though in most patients the percentage of GPI-deficient NK cells was considerably higher than B or T cells. These observations can be explained when mechanisms of normal lymphopoiesis are considered. Despite these quantitative and qualitative abnormalities, no patients suffered an excessive number or severity of infections. The detection of PNH clones amongst all lymphocyte lineages may provide important information regarding the natural history of the disease and additional insights into kinetics of adult lymphopoiesis.
ISSN:0006-4971
1528-0020
DOI:10.1182/blood.V92.5.1799