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Cytogenetic abnormalities in paroxysmal nocturnal haemoglobinuria usually occur in haematopoietic cells that are glycosylphosphatidylinositol‐anchored protein (GPI‐AP) positive
Author(s) -
Sloand Elaine M.,
Fuhrer Monika,
Keyvanfar Keyvan,
Mainwaring Lori,
Maciejewski Jaroslaw,
Wang Yu,
Johnson Sarah,
Barrett A. John,
Young Neal S.
Publication year - 2003
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.1365-2141.2003.04562.x
Subject(s) - clone (java method) , haematopoiesis , cd34 , biology , karyotype , bone marrow , stem cell , abnormality , bone marrow failure , immunology , chromosome instability , cancer research , chromosome , genetics , gene , medicine , psychiatry
Summary. Some patients with paroxysmal nocturnal haemoglobinuria (PNH) have bone marrow findings characteristic of myelodysplastic syndrome. We studied nine PNH patients to determine whether these karyotypic abnormalities were more likely to occur in glycosylphosphatidylinositol‐anchored protein (GPI‐AP)‐negative cells. Abnormal chromosome patterns were evident only in the GPI‐AP‐positive populations of the PNH clone in 8 of 9 cases studied. Purified GPI‐AP‐negative CD34 cells gave rise only to cells of normal karyotype, whereas the progeny of the GPI‐AP‐positive CD34 cells showed the karyotypic abnormality. These findings suggest that environmental factors, but not genetic instability of the GPI‐AP‐deficient clone, foster development or survival of haematopoietic cells with chromosomal abnormalities.