Classer les syndromes myéloprolifératifs (SMP) n'est pas inutile.
Rédacteur : Xavier Troussard , France-Hemato, 17 avril 2007
'Il n'est pas inutile de revenir sur la classification des syndromes myéloprolifératifs (SMP). Cette excellente revue générale nous permet de faire le point sur les différentes entités actuellement individualisées. Parmi les SMP classiques, il convient d'identifier la LMC, caractérisée par la présence d'une t(9;22)(q34;q11) ou d'un transcrit de fusion Bcr/Abl : la LMC a largement bénéficié de l'introduction de l'imatinib mesylate. Sont aussi identifiées dans cette catégorie la thrombocythémie essentielle (TE), la polyglobulie primitive (PG) et la myélofibrose avec métaplasie myéloïde (MFMM) : ces trois entités sont caractérisées par la présence de la mutation JAK2V617F (mutation dans l'exon 14 du gène JAK2 localisé en 9p24) dans respectivement 23-57% des cas de TE, 65-97% des cas de PG et 35-57% des cas de MFMM.'
Extrait de http://www.france-hemato.net/rdp2.php?num=3447
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cousineg
- Messages : 1058
- Enregistré le : lun. août 14, 2006 4:36 am
- Localisation : Montréal (Ar. Saint-Laurent)
-
cousineg
- Messages : 1058
- Enregistré le : lun. août 14, 2006 4:36 am
- Localisation : Montréal (Ar. Saint-Laurent)
Re: La classification des syndromes myéloprolifératifs (SMP)
The history of myeloproliferative disorders: before and after Dameshek
A Tefferi ,Leukemia (2008).Volume: 22, Issue: 1, Pages: 3-13
'In 1951, William Dameshek described the concept of 'myeloproliferative disorders (MPDs)' by grouping together chronic myelogenous leukemia (CML), polycythemia vera (PV), essential thrombocythemia (ET), primary myelofibrosis (PMF) and erythroleukemia; he reasoned that a self-perpetuating trilineage myeloproliferation underlined their pathogenesis. Pre-Dameshek luminaries who laid the foundation for this unifying concept include Bennett, Virchow, Heuck, Vaquez, Osler, Di Guglielmo and Epstein. In 1960, Nowell and Hungerford discovered the Philadelphia (Ph) chromosome in CML. In 1967, Fialkow and colleagues used X-linked polymorphisms to establish CML as a clonal stem cell disease. Also in 1967, the PV Study Group was summoned by Louis Wasserman to study the natural history of PV and conduct large-scale clinical trials. In 1972, Janet Rowley deciphered the Ph chromosome as a reciprocal translocation between chromosomes 9 and 22, thus paving the way for its subsequent characterization as an oncogenic BCR-ABL mutation. In 1996, Brian Druker discovered imatinib-a small molecule ABL inhibitor with exceptional therapeutic activity in CML. In 2005, a gain-of-function JAK2 mutation (JAK2V617F) was described in BCR-ABL-negative MPDs, raising the prospect of a CML-like treatment strategy in PV, ET and PMF. The current review considers these and other landmark events in the history of MPDs.'
Extrait de http://www.mendeley.com/research/the-hi ... -dameshek/
A Tefferi ,Leukemia (2008).Volume: 22, Issue: 1, Pages: 3-13
'In 1951, William Dameshek described the concept of 'myeloproliferative disorders (MPDs)' by grouping together chronic myelogenous leukemia (CML), polycythemia vera (PV), essential thrombocythemia (ET), primary myelofibrosis (PMF) and erythroleukemia; he reasoned that a self-perpetuating trilineage myeloproliferation underlined their pathogenesis. Pre-Dameshek luminaries who laid the foundation for this unifying concept include Bennett, Virchow, Heuck, Vaquez, Osler, Di Guglielmo and Epstein. In 1960, Nowell and Hungerford discovered the Philadelphia (Ph) chromosome in CML. In 1967, Fialkow and colleagues used X-linked polymorphisms to establish CML as a clonal stem cell disease. Also in 1967, the PV Study Group was summoned by Louis Wasserman to study the natural history of PV and conduct large-scale clinical trials. In 1972, Janet Rowley deciphered the Ph chromosome as a reciprocal translocation between chromosomes 9 and 22, thus paving the way for its subsequent characterization as an oncogenic BCR-ABL mutation. In 1996, Brian Druker discovered imatinib-a small molecule ABL inhibitor with exceptional therapeutic activity in CML. In 2005, a gain-of-function JAK2 mutation (JAK2V617F) was described in BCR-ABL-negative MPDs, raising the prospect of a CML-like treatment strategy in PV, ET and PMF. The current review considers these and other landmark events in the history of MPDs.'
Extrait de http://www.mendeley.com/research/the-hi ... -dameshek/