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Disturbed patterns of globin chain synthesis in childhood monosomy 7 myeloproliferative syndrome
Author(s) -
Sheffer R.,
Cividalli G.,
Zaharan Y.,
Tamari H.,
Okon E.
Publication year - 1988
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.1365-2141.1988.tb04214.x
Subject(s) - monosomy , clone (java method) , down syndrome , globin , alpha (finance) , malignant transformation , cancer research , genetics , medicine , immunology , biology , karyotype , gene , chromosome , construct validity , nursing , patient satisfaction
Summary Two children with typical clinical and haematological features of monosomy 7 myeloproliferative syndrome are presented. Both children displayed decreased production of beta‐globin chains and unbalanced high alpha/non‐alpha synthetic ratios similar to those characteristic of homozygous β‐thalassaemia. These provide further evidence for the involvement of the erythroid line as part of the malignant clone, indicating neoplastic transformation of a pluripotential stem cell in this disease.