Details

Autor(en) / Beteiligte

• Campbell, Peter J
• Green, Anthony R

Titel

The Myeloproliferative Disorders

Ist Teil von

• The New England journal of medicine, 2006-12, Vol.355 (23), p.2452-2466

Ort / Verlag

Boston, MA: Massachusetts Medical Society

Erscheinungsjahr

2006

Link zum Volltext

Quelle

MEDLINE

Beschreibungen/Notizen

• The discovery of an identical mutation (V617F) of the JAK2 gene in patients with polycythemia vera, essential thrombocythemia, and myelofibrosis — the principal Philadelphia chromosome–negative myeloproliferative disorders — has greatly advanced our understanding of these conditions. This article reviews the legacy of this discovery and how it has changed our view of the origins, interrelations, and management of the myeloproliferative disorders. The discovery of an identical mutation (V617F) of the JAK2 gene in patients with the principal Philadelphia chromosome–negative myeloproliferative disorders has greatly advanced our understanding of these conditions. This article reviews the legacy of this discovery and how it has changed our view of the origins, interrelations, and management of the myeloproliferative disorders. The myeloproliferative disorders comprise several clonal hematologic diseases that are thought to arise from a transformation in a hematopoietic stem cell. The main clinical features of these diseases are the overproduction of mature, functional blood cells and a long clinical course. Chronic myeloid leukemia (not discussed in detail here) is a myeloproliferative disorder that is defined by its causative molecular lesion, the BCR-ABL fusion gene, which most commonly results from the Philadelphia translocation (Ph). 1 The three main Ph-negative myeloproliferative disorders — polycythemia vera, essential thrombocythemia, and idiopathic myelofibrosis — are the focus of this article. Less common conditions that are . . .