Disturbed patterns of globin chain synthesis in childhood monosomy 7 myeloproliferative syndrome

R. Sheffer; G. Cividalli; Y. Zaharan; H. Tamari; E. Okon

doi:10.1111/j.1365-2141.1988.tb04214.x

Disturbed patterns of globin chain synthesis in childhood monosomy 7 myeloproliferative syndrome

R. Sheffer, G. Cividalli, Y. Zaharan, H. Tamari, E. Okon

Research output: Contribution to journal › Article › peer-review

Abstract

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.

Original language	English
Pages (from-to)	357-362
Number of pages	6
Journal	British Journal of Haematology
Volume	68
Issue number	3
DOIs	https://doi.org/10.1111/j.1365-2141.1988.tb04214.x
State	Published - 1988
Externally published	Yes

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title = "Disturbed patterns of globin chain synthesis in childhood monosomy 7 myeloproliferative syndrome",

abstract = "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.",

author = "R. Sheffer and G. Cividalli and Y. Zaharan and H. Tamari and E. Okon",

year = "1988",

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AU - Tamari, H.

AU - Okon, E.

PY - 1988

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N2 - 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.

AB - 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.

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Disturbed patterns of globin chain synthesis in childhood monosomy 7 myeloproliferative syndrome

Abstract

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