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Diamond‐Blackfan syndrome: A possible role of cellular factors for erythropoietic suppression
Author(s) -
Sawada Kenichi,
Koyanagawa Yoshinori,
Sakurama Shoki,
Nakagawa Shoichi,
Konno Takahiko
Publication year - 1985
Publication title -
scandinavian journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0036-553X
DOI - 10.1111/j.1600-0609.1985.tb01565.x
Subject(s) - peripheral blood mononuclear cell , buffy coat , bone marrow , rosette (schizont appearance) , immunology , cell growth , antiserum , biology , microbiology and biotechnology , antibody , in vitro , biochemistry
CFU‐E growth from fractionated bone marrow cells of 5 children with Diamond‐Blackfan syndrome was studied. In all patients, CFU‐E growth was reduced in mononuclear cell‐rich fraction. In 2 of the 5 patients, CFU‐E growth was returned to normal by the depletion of E‐rosette forming cells or monocytes from mononuclear cell‐rich fraction. In the patient whose CFU‐E growth returned to normal by the depletion of E‐rosette forming cells, co‐cultivation between bone marrow buffy coat cells and autologous bone marrow E‐rosette forming cells resulted in a significant decrease of CFU‐E growth, and there was a significant increase in CFU‐E growth by treatment with monoclonal antiserum to OKT 4. We concluded that immunologic causes such as cellular factors may play a role, at least in part, in the pathogenesis of Diamond‐Blackfan syndrome.