Blood, Vol. 92 No. 5 (September 1), 1998:
pp. 1799-1806
Lymphocyte Subset Analysis and Glycosylphosphatidylinositol
Phenotype in Patients With Paroxysmal Nocturnal Hemoglobinuria
Stephen. J. Richards,
Derek. R. Norfolk,
David M. Swirsky, and
Peter Hillmen
From the Haematological Malignancy Diagnostic Service, Leeds General
Infirmary, Leeds; and the Department of Haematology, Imperial College
School of Medicine, The Hammersmith Hospital, London, UK.
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 × 109/L; normal range, 0.2 to 0.4 × 109/L) and
low absolute numbers of B cells (median, 0.05 × 109/L;
normal range, 0.06 to 0.65 × 109/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.
© 1998 by The American Society of Hematology.
