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Production and characterization of lymphoblastoid cell lines with the
paroxysmal nocturnal hemoglobinuria phenotype
P Hillmen, M Bessler, DH Crawford and L Luzzatto
Department of Haematology, Royal Postgraduate Medical School, Hammersmith
Hospital, London, UK.
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic disorder
caused by a somatic mutation in a hematopoietic stem cell. The fact that,
in some cases, not only myeloid but also lymphoid cells are affected
suggests that the mutation has occurred in a multipotent stem cell. By
studying the expression of CD59 antigen (membrane inhibitor of reactive
lysis) and of decay accelerating factor (DAF) on the lymphocytes of 16
patients with PNH, we found an abnormal population of lymphocytes (with
absent CD59 and DAF) in 10 cases. From 4 of these patients we were able to
produce Epstein-Barr virus-immortalized lymphoblastoid cell lines (LCLs)
that have a PNH phenotype (absent CD59, DAF, and CD48). PNH LCL cells have
apparently normal DAF messenger RNA despite not having DAF on their
surface. These cell lines will be a valuable resource for further
investigation of the defect or defects underlying PNH.
Volume 81,
Issue 1,
pp. 193-199,
01/01/1993
Copyright © 1993 by The American Society of Hematology
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