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Reduced interferon‐alpha production by Epstein‐Barr virus transformed B‐lymphoblastoid cell lines and lectin‐stimulated lymphocytes in congenital dyserythropoietic anaemia type I
Author(s) -
WICKRAMASINGHE S. N.,
HASAN R.,
SMYTHE J.
Publication year - 1997
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.1997.1993016.x
Subject(s) - phytohaemagglutinin , pokeweed mitogen , alpha interferon , lymphoblast , alpha (finance) , biology , virology , lymphocyte , epstein–barr virus , immunology , virus , cell culture , interferon , interferon alfa , microbiology and biotechnology , medicine , concanavalin a , in vitro , biochemistry , genetics , construct validity , nursing , patient satisfaction
The concentrations of interferon‐α (IFN‐α) in supernatants from cultures of Epstein‐Barr virus (EBV) transformed B‐lymphoblastoid cell lines derived from seven patients with congenital dyserythropoietic anaemia (CDA) type I were below the 95% confidence limits for those derived from six healthy subjects. In contrast, the concentrations of IFN‐α in supernatants from cultures of EBV‐transformed lymphoblastoid cell lines derived from four patients with other types of CDA and four patients with hereditary sideroblastic anaemia were normal. Supernatants from cultures of peripheral blood lymphocytes stimulated with phytohaemagglutinin or pokeweed mitogen contained less IFN‐α when the cells were derived from patients with CDA type I than when derived from healthy subjects. Since patients with CDA type I show a substantial haematological response to treatment with IFN‐α, the data suggest that impaired IFN‐α production may be an important pathogenetic mechanism in CDA type I.