Abstract：
:Myeloproliferative neoplasms (MPN) include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). MPN are characterized by clonal proliferation of myeloid progenitors leading to erythrocytosis, thrombocytosis, or leukocytosis, and risk of hemorrhagic and thrombotic events, as well as myelofibrosis and blast transformation. The discovery of somatic mutations in MPN, namely JAK2 V617F, JAK2 exon 12, MPL, and CALR mutations, has permitted a more specific approach to diagnosis and treatment. The prevalence of JAK2 V617F mutations is higher than 95% in PV, 50%-75% in ET and 40%-75% in PMF. JAK2 exon 12 mutations are specific of PV. A 20%-30% of patients with ET and PMF present a CALR mutation. The screening of mutations strengthens the diagnosis of MPN since 97% of MPN have at least 1 somatic mutation. Interestingly, different mutations grant different phenotype and prognosis. Of particular importance, CALR mutations grant a favorable prognosis in ET and PMF, while ASXL1 mutations confer a poorer outcome. In fact, the use of CALR/ASXL1 status for the prognostication of patients has increased clinical value and is now suggested for guidance of therapy in PMF. The increasing importance of mutations in the management of MPN warrants a more frequent revision of current diagnostic criteria and prognostic models and a better understanding of the mechanisms leading to MPN subset differentiation.
journal_name
Semin Hematoljournal_title
Seminars in hematologyauthors
Ferreira Cristina S,Polo B,Lacerda JFdoi
10.1053/j.seminhematol.2018.04.005subject
Has Abstractpub_date
2018-10-01 00:00:00pages
215-222issue
4eissn
0037-1963issn
1532-8686pii
S0037-1963(17)30169-5journal_volume
55pub_type
杂志文章,评审abstract：:Autologous hematopoietic stem cell transplantation (HSCT) has a long tradition in Europe. Initially developed as a tool to restore rapid remission or chronic phase in patients with advanced leukemia without a sibling donor, it evolved over the last three decades to be used as a standard tool in patients with malignanc...
journal_title：Seminars in hematology
pub_type： 历史文章,杂志文章
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abstract：:Thrombus forms on catheters and other indwelling synthetic materials. Systemic heparin infusion decreases, but does not eliminate thrombus formation. The day is approaching when systemic heparinization may no longer be indicated to suppress catheter thrombogenesis. Passivation of catheters by heparin immobilization is...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：
更新日期：1991-10-01 00:00:00
abstract：:Macrocytic anemia, defined as a mean cell volume (MCV) ≥100 fL in adults, has a narrow differential diagnosis that requires evaluation of the peripheral blood smear as well as additional laboratory testing taken in conjunction with clinical information that includes patient history and physical examination findings. T...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
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journal_title：Seminars in hematology
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journal_title：Seminars in hematology
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journal_title：Seminars in hematology
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
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abstract：:Recombinant activated factor VII (rFVIIa; NovoSeven, Novo Nordisk, Denmark) induces hemostasis in life- and limb-threatening bleeds and in major surgery of hemophilia A and B patients, regardless of inhibitor titer. A total of more than 6,500 patients have been treated, and NovoSeven has been administered in more than...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1016/s0037-1963(01)90147-7
更新日期：2001-10-01 00:00:00
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：
更新日期：1990-04-01 00:00:00
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2003.11.009
更新日期：2004-01-01 00:00:00
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1016/s0037-1963(00)90094-5
更新日期：2000-07-01 00:00:00
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journal_title：Seminars in hematology
pub_type： 杂志文章
doi：10.1053/j.seminhematol.2016.04.009
更新日期：2016-04-01 00:00:00
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：
更新日期：1999-04-01 00:00:00
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2016.07.003
更新日期：2016-10-01 00:00:00
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/shem.2002.30909
更新日期：2002-01-01 00:00:00
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：
更新日期：1998-07-01 00:00:00
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：
更新日期：1991-04-01 00:00:00
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1016/s0037-1963(03)00135-5
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：
更新日期：1997-01-01 00:00:00
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1016/s0037-1963(01)90052-6
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abstract：:Allogeneic hematopoietic stem cell transplantation (HSCT) is the only potential cure for most bone marrow (BM) failure syndromes and hemoglobinopathies. Over the past decade, umbilical cord blood (UCB) has been used more frequently as a stem cell source in patients who lack a suitable BM donor. Although graft failure ...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
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更新日期：2010-01-01 00:00:00
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：
更新日期：1987-10-01 00:00:00
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journal_title：Seminars in hematology
pub_type： 杂志文章,评审
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更新日期：2011-04-01 00:00:00
abstract：:This phase I trial of idarubicin (IDA) was conducted in 32 patients with acute leukemia and chronic myelogenous leukemia (CML) in blastic crisis (CML/BC) who either had failed to achieve a complete remission (CR) after initial induction therapy or had relapsed after CR. IDA was administered at dosages ranging from an ...
journal_title：Seminars in hematology
pub_type： 临床试验,杂志文章,评审
doi：
更新日期：1996-10-01 00:00:00
