Pregnancy and thrombocythemia

Tefferi, Ayalew

doi:10.1002/(sici)1096-8652(199802)57:2<181::aid-ajh19>3.0.co;2-f

Cited by 2 publications

(4 citation statements)

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“…In addition, all 3 disorders might be associated with hepatosplenomegaly (PMF>PV>ET), leukocytosis (PMF>PV>ET), thrombocytosis (ET>PMF>PV), microvascular symptoms (PV>ET>PMF), constitutional symptoms (PMF>PV>ET), thrombohemorrhagic complications (PV>ET>PMF), and variable risk of leukemic transformation (PMF>PV>ET), or fibrotic progression (PV>ET). 5 Patients with PV or PMF might also experience intractable pruritus, whereas increased rates of first trimester miscarriage have been reported in ET 6,7 ; in a recent study, approximately 59% of 292 patients with WHO-defined ET were women and 58% of the study population was younger than 60 years. 8 Additional clinical manifestations in PV include symptoms of hyperviscosity and in PMF, progressive anemia, leukoerythroblastosis, extramedullary hematopoiesis, recurrent splenic infarcts, peripheral edema, early satiety, cachexia, and symptoms of portal hypertension, including ascites and variceal bleeding.…”

Section: Phenotypementioning

confidence: 99%

Genetic Risk Assessment in Myeloproliferative Neoplasms

Tefferi

Vannucchi

2017

Mayo Clinic Proceedings

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The World Health Organization classification system recognizes 4 variants of JAK2 mutation-enriched myeloproliferative neoplasms (for expansion of gene symbols, use search tool at www.genenames.org): essential thrombocythemia (ET), polycythemia vera (PV), primary myelofibrosis (PMF), and prefibrotic PMF. All 4 disorders are characterized by stem cell-derived clonal myeloproliferation with mutually exclusive driver mutations, including JAK2, CALR, and MPL. The median survival is approximately 20 years for ET, 14 years for PV, and 6 years for PMF; age is the most important determinant of survival with the corresponding median of 33, 24, and 15 years in patients younger than 60 years. Genetic information is the second most important prognostic tool and includes karyotype, driver mutational status, and presence of specific other mutations. Karyotype has been shown to carry prognostic relevance in PV (abnormal vs normal) and PMF (unfavorable vs favorable abnormalities). Driver mutational status is prognostically most relevant in PMF; type 1/type 1-like CALR vs other driver mutational status has been associated with superior survival. In ET, arterial thrombosis risk is higher in patients with JAK2 or MPL mutations whereas MPL-mutated patients might be at risk for accelerated fibrotic progression. ASXL1 and SRSF2 mutations have been associated with inferior overall, leukemia-free, or fibrosis-free survival in both PV and PMF, and a recent targeted sequencing study has identified additional other adverse mutations in both these disorders, as well as in ET. Further enhancement of genetic risk stratification in myeloproliferative neoplasms is possible by combining cytogenetic and mutation information and developing a prognostic model that is adjusted for age.

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Section: Phenotypementioning

confidence: 99%

Genetic Risk Assessment in Myeloproliferative Neoplasms

Tefferi

Vannucchi

2017

Mayo Clinic Proceedings

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“…Thrombosis and bleeding are frequent complications during the course of the CMPD's [55][56][57][58][59][60][61][62]. Besides abnormal rheology due to a raised haematocrit in PV-patients these events are primarily attributed to platelet and endothelial cell activation and dysfunction [52][53][54][55].…”

Section: The Rationale For Treatment Of Chronic Myeloproliferative DImentioning

confidence: 99%

“…The diseases arise due to an acquired stem cell insult with subsequent clonal myeloproliferation [56][57][58][59][60][61][62]. In PV and ET thrombohaemorrhagic complications are the major determinants of morbidity [55].…”

Section: Introductionmentioning

confidence: 99%

“…Furthermore, PV may progress to myelofibrosis with myeloid metaplasia (MMM), also called the "spent phase" of the disease, which evolves in approximately 10 -15 % of the patients after an average of 10 years from diagnosis. Before this end stage of PV these patients may remain in a transitional stage of the disease ("transitional myeloproliferative disorder") for several years being featured by pancytosis, huge splenomegaly and bone marrow fibrosis [59,61]. In recent years, it has become evident that ET is a highly heterogeneous disease entity.…”

Section: Introductionmentioning

confidence: 99%

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The Mevalonate Pathway as a Therapeutic Target in the Ph-Negative Chronic Myeloproliferative Disorders

Hasselbalch¹,

Riley

2007

CDT

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The Ph-negative chronic myeloproliferative disorders (CMPDs) polycythaemia vera, essential thrombocytosis and idiopathic myelofibrosis are acquired stem cell disorders, which pathophysiologically are featured by clonal myeloproliferation and accumulation of myeloid cells, the latter being consequent to decreased apoptosis. Myelofibrosis and neoangiogenesis in the bone marrow and spleen are the histopathological hallmarks of idiopathic myelofibrosis but may develop in the other diseases as well. In patients with myelofibrosis elevated levels of circulating CD34+ cells are highly characteristic being partly explained by a proteolytic bone marrow mileu owing to excessive release of various proteases with ensuing extracellular matrix degradation and constitutive mobilisation of CD34+ cells into the peripheral blood. Thrombohaemorrhagic complications are major clinical problems contributing significantly to morbidity and mortality. Based upon in vitro and in vivo studies of the effects of statins (antithrombotic, antiproliferative, antiangiogenic, antiproteolytic) and zoledronic acid (antiproliferative antiproliferative, antiangiogenic, antiproteolytic) this review focusses on the translation of these effects into potential clinical benefits of combinational therapy with these agents in patients with CMPDs.

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Pregnancy and thrombocythemia

Cited by 2 publications

References 4 publications

Genetic Risk Assessment in Myeloproliferative Neoplasms

Genetic Risk Assessment in Myeloproliferative Neoplasms

The Mevalonate Pathway as a Therapeutic Target in the Ph-Negative Chronic Myeloproliferative Disorders

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