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Philadelphia-negative chronic myeloproliferative neoplasms

Overview of attention for article published in Revista Brasileira de Hematologia e Hemoterapia, January 2012
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Philadelphia-negative chronic myeloproliferative neoplasms
Published in
Revista Brasileira de Hematologia e Hemoterapia, January 2012
DOI 10.5581/1516-8484.20120034
Pubmed ID

Rosane Isabel Bittencourt<sup></sup>, Jose Vassallo<sup></sup>, Maria de Lourdes Lopes Ferrari Chauffaille<sup></sup>, Sandra Guerra Xavier<sup></sup>, Katia Borgia Pagnano<sup></sup>, Ana Clara Kneese Nascimento<sup></sup>, Carmino Antonio De Souza<sup></sup>, Carlos Sergio Chiattone<sup></sup>


Chronic myeloproliferative diseases without the Philadelphia chromosome marker (Ph-), although first described 60 years ago, only became the subject of interest after the turn of the millennium. In 2001, the World Health Organization (WHO) defined the classification of this group of diseases and in 2008 they were renamed myeloproliferative neoplasms based on morphological, cytogenetic and molecular features. In 2005, the identification of a recurrent molecular abnormality characterized by a gain of function with a mutation in the gene encoding Janus kinase 2 (JAK2) paved the way for greater knowledge of the pathophysiology of myeloproliferative neoplasms. The JAK2 mutation is found in 90-98% of polycythemia vera and in about 50% essential thrombocytosis and primary myelofibrosis. In addition to the JAK2 mutation, other mutations involving TET2 (ten-eleven translocation), LNK (a membrane-bound adaptor protein); IDH1/2 (isocitrate dehydrogenase 1/2 enzyme); ASXL1 (additional sex combs-like 1) genes were found in myeloproliferative neoplasms thus showing the importance of identifying molecular genetic alterations to confirm diagnosis, guide treatment and improve our understanding of the biology of these diseases. Currently, polycythemia vera, essential thrombocytosis, myelofibrosis, chronic neutrophilic leukemia, chronic eosinophilic leukemia and mastocytosis are included in this group of myeloproliferative neoplasms, but are considered different situations with individualized diagnostic methods and treatment. This review updates pathogenic aspects, molecular genetic alterations, the fundamental criteria for diagnosis and the best approach for each of these entities.

Mendeley readers

The data shown below were compiled from readership statistics for 48 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Brazil 2 4%
Unknown 46 96%

Demographic breakdown

Readers by professional status Count As %
Student > Master 8 17%
Researcher 6 13%
Other 4 8%
Student > Postgraduate 4 8%
Student > Doctoral Student 4 8%
Other 17 35%
Unknown 5 10%
Readers by discipline Count As %
Medicine and Dentistry 17 35%
Agricultural and Biological Sciences 9 19%
Biochemistry, Genetics and Molecular Biology 5 10%
Nursing and Health Professions 4 8%
Philosophy 1 2%
Other 6 13%
Unknown 6 13%