Disturbed patterns of globin chain synthesis in childhood monosomy 7 myeloproliferative syndrome.
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 beta-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.
Sheffer
R
R
Department of Pediatrics, Hadassah University Hospital, Jerusalem, Israel.
Cividalli
G
G
Zaharan
Y
Y
Tamari
H
H
Okon
E
E
D001853:Bone Marrow / Q000473:pathology; D002675:Child, Preschool; D002872:Chromosome Deletion; D002897:Chromosomes, Human, Pair 7; D005260:Female; D005914:Globins / Q000096:biosynthesis*; D006801:Humans; D007223:Infant; D008099:Liver / Q000473:pathology; D008297:Male; D009006:Monosomy; D009196:Myeloproliferative Disorders / Q000097:blood / Q000235:genetics* / Q000473:pathology
D005914:Globins