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Platelet acquired defect in PDGF and β thromboglobulin content in hairy cell leukaemia: improvement after interferon therapy
Author(s) -
Dupuy E.,
Sigaux F.,
Bryckaert M. C.,
Tobelem G.,
Castaigne S.,
Flandrin G.,
Degos L.,
Caen J. P.
Publication year - 1987
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 - 10.1111/j.1365-2141.1987.tb06143.x
Subject(s) - platelet , radioimmunoassay , myelofibrosis , medicine , platelet derived growth factor , interferon , platelet derived growth factor receptor , endocrinology , granule (geology) , chemotherapy , platelet factor 4 , immunology , growth factor , biology , bone marrow , receptor , paleontology
Summary To investigate the platelet contribution to the development of myelofibrosis in hairy cell leukaemia (HCL), we have studied two platelet α granule components in 15 patients with HCL before chemotherapy: mitogenic activity was measured by 1 H thymidine incorporation in BALB/C 3T3 cells and β thromboglobulin (βTG) assayed by radioimmunoassay (RIA). Platelet mitogenic activity and βG content were significantly decreased in the patients as compared to the control subjects. The nine patients who were treated with recombinant human interferon (IFNαA) were restudied after 4 months of therapy. The levels of both mitogenic activity and βG platelet content were significantly increased after IFNα‐treatment with a complete response in five of the nine treated patients, a partial response in two and no response in the two others. HCL seemed therefore to be responsible for an acquired platelet α granule defect. As in the grey platelet syndrome a relationship between this abnormal platelet granule storage and the development of myelofibrosis is suggested in HCL.