Clonality in chronic myeloproliferative disorders defined by X-chromosome linked probes: demonstration of heterogeneity in lineage involvement.

Abstract

The restriction fragment length polymorphisms (RFLP) of the X-chromosome phosphoglycerate kinase (PGK) and hypoxanthine phosphoribosyltransferase (HPRT) genes were used to study the clonal basis of the chronic myeloproliferative disorders (CMPD). Analyses were performed on granulocyte and T-lymphocyte fractions obtained from 24 females; 13 had essential thrombocythaemia (ET), eight polycythaemia vera (PV) and three myelofibrosis with myeloid metaplasia (MMM). All 24 of these patients had monoclonal patterns of X-inactivation in the granulocyte fraction. For the T-lymphocyte fraction, non-clonal patterns of X-inactivation were observed in 8/13 patients with ET, 7/8 with PV and 1/3 with MMM, while the remaining eight subjects were found to have monoclonal patterns of X-inactivation. Our findings suggest that the majority of the CMPD in these patients originated from a relatively committed progenitor cell without the capacity to differentiate into T cells, and convincingly demonstrated heterogeneity of lineage involvement.

• Authors: N Tsukamoto, K Morita, T Maehara, K Okamoto, H Sakai, M Karasawa, T Naruse, M Omine
• Journal: British journal of haematology (Br J Haematol) Vol. 86 Issue 2 Pg. 253-8 (Feb 1994) ISSN: 0007-1048 [Print] England
• PMID: 7911034 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Hypoxanthine Phosphoribosyltransferase
• Phosphoglycerate Kinase

Topics

• Adult
• Aged
• Aged, 80 and over
• Chronic Disease
• Clone Cells (pathology)
• Dosage Compensation, Genetic
• Female
• Genetic Linkage
• Granulocytes (pathology)
• Humans
• Hypoxanthine Phosphoribosyltransferase (genetics)
• Middle Aged
• Myeloproliferative Disorders (genetics)
• Phosphoglycerate Kinase (genetics)
• Polycythemia Vera (genetics)
• Polymorphism, Restriction Fragment Length
• Primary Myelofibrosis (genetics)
• T-Lymphocytes (pathology)
• Thrombocythemia, Essential (genetics)
• X Chromosome