DÄ internationalArchive26/2008Congenital and Acquired Polycythemias: Key Role of HIF-2a

Correspondence

Congenital and Acquired Polycythemias: Key Role of HIF-2a

Dtsch Arztebl Int 2008; 105(26): 480; DOI: 10.3238/arztebl.2008.0480b

Tsamaloukas, A G

This excellent review article follows on seamlessly from August 2007's "Essential Thrombocythemia."
It remains to be seen whether the two other chronic myeloproliferative disorders – chronic myeloid leukemia and myelofibrosis – will also be reviewed so comprehensively.
In figure 2, the authors illustrate the cycle of erythropoiesis via erythropoietin and the central role of HIF-2a in cellular oxygen sensing. Further to the mutations described by the authors, a mutation of the HIF-2A gene (G1909T) (1) and of the prolyl hydroxylase domain-containing protein 2 (PHD2) in other familial erythrocytoses has been described (2). This is particularly notable because initial clinical studies have started and have been published on the basis of our understanding of the regulation of erythropoietin production. Oral medications are used to inhibit PHD2 and treat anemias. In light of the current discussion about erythropoiesis stimulating factors, molecular insights into the key role of HIF-2a will enable the deduction of new strategies to stimulate erythropoiesis and therefore potentially new treatment options for anemia (3). DOI: 10.3238/arztebl.2008.0480b

Dr. med. Antonis G. Tsamaloukas
Schulstr. 16–18, 40721 Hilden, Germany
tsamaloukas@onkologe-hilden.de
1.
Percy MJ et al.: A gain-of-function mutation in the HIF2A gene in familial erythrocytosis. N Engl J Med 2008; 358: 162–8. MEDLINE
2.
Percy MJ et al.: A family with erythrocytosis establishes a role for prolyl hydroxylase domain protein 2 in oxygen homeostasis. Proc Natl Acad Sci USA 2006; 103: 654–9. MEDLINE
3.
Bunn HF: New agents that stimulate erythropoiesis. Blood 2007; 109: 868–73. MEDLINE
1. Percy MJ et al.: A gain-of-function mutation in the HIF2A gene in familial erythrocytosis. N Engl J Med 2008; 358: 162–8. MEDLINE
2. Percy MJ et al.: A family with erythrocytosis establishes a role for prolyl hydroxylase domain protein 2 in oxygen homeostasis. Proc Natl Acad Sci USA 2006; 103: 654–9. MEDLINE
3. Bunn HF: New agents that stimulate erythropoiesis. Blood 2007; 109: 868–73. MEDLINE