- Improved antenatal prediction of twin anemia-polycythemia sequence by delta middle cerebral artery peak systolic velocity: a new antenatal classification system. [Journal Article]
- UOUltrasound Obstet Gynecol 2018 Aug 20
- CONCLUSIONS: Delta MCA-PSV > 0.5 MoM has a higher diagnostic accuracy for predicting TAPS compared to the currently used MCA-PSV cut-off values. We therefore propose a new antenatal classification system. In case of a delta MCA-PSV > 0.5 MoM on Doppler ultrasound, but with normal MCA-PSV values in donor or recipient, obstetricians should be aware of the therapeutic implications and neonatal morbidities associated with TAPS. This article is protected by copyright. All rights reserved.
- Patient-Reported Outcomes Data From REVEAL at the Time of Enrollment (Baseline): A Prospective Observational Study of Patients With Polycythemia Vera in the United States. [Journal Article]
- CLClin Lymphoma Myeloma Leuk 2018; 18(9):590-596
- CONCLUSIONS: These data confirm that many patients with PV experience symptoms, QoL impairments, and work productivity impairments that negatively affect their lives. Longitudinal data from REVEAL will be important for evaluating how PROs change over time in these patients.
- Ruxolitinib Rechallenge Can Improve Constitutional Symptoms and Splenomegaly in Patients With Myelofibrosis: A Case Series. [Journal Article]
- CLClin Lymphoma Myeloma Leuk 2018 Jun 28
- CONCLUSIONS: Our study demonstrates that re-exposure to ruxolitinib following a period of treatment cessation in patients with MF can lead to durable responses with regards to both splenomegaly and symptom burden.
- Polycythemia vera: diagnosis, clinical course, and current management [Journal Article]
- TJTurk J Med Sci 2018 Aug 16; 48(4):698-710
- Very important developments related to polycythemia vera (PV) have occurred during the last two decades. The discovery of Janus kinase (JAK) 2 mutations has changed both the diagnosis and clinical ma...
Very important developments related to polycythemia vera (PV) have occurred during the last two decades. The discovery of Janus kinase (JAK) 2 mutations has changed both the diagnosis and clinical management of PV. Currently JAK2 molecular testing is essential in the diagnostic work-up and JAK2 mutation positivity is a major diagnostic criterion. The discovery of JAK2 mutations suggested that abnormal JAK-STAT signaling was a pivotal feature in the pathogenesis of Philadelphia-negative myeloproliferative neoplasms. This idea led to the development of JAK inhibitors. Currently ruxolitinib, a JAK1/JAK2 inhibitor, is also approved for PV patients with hydroxyurea resistance or intolerance. International collaborations have made it possible to describe disease characteristics and evolution better. Presently it is possible to quantify the symptomatic burden of the disease and to estimate prognosis. In spite of these developments, management of PV still largely depends on estimation of thromboembolic risk and trying to decrease the risk with or without cytoreductive medications. Different approaches have been proposed by international disease experts for the diagnosis, thromboembolic risk estimation, and drug selection. This paper aims to review clinical aspects of PV and propose a management algorithm. The authors also point to still unresolved questions and unmet needs in diagnosis and management.
- [Acute myocardial infarction due to polycythemia vera]. [Case Reports]
- RMRev Med Liege 2018; 73(7-8):397-401
- We report the clinical case of a patient presenting with an acute myocardial infarction with ST- segment elevation. The patient is affected by polycythemia vera for many years and doesn't have any ot...
We report the clinical case of a patient presenting with an acute myocardial infarction with ST- segment elevation. The patient is affected by polycythemia vera for many years and doesn't have any other cardiovascular risk factors. The frequency of thrombotic events in polycythemia vera (and more particularly myocardial infarction), their predictive factors, pathophysiology and treatment will be discussed.
- [Detection and Diagnostic Values of JAK2, CALR, MPL Gene Mutations in 208 Cases of BCR/ABL1 Negative Chronic Myeloproliferative Diseases]. [Journal Article]
- ZSZhongguo Shi Yan Xue Ye Xue Za Zhi 2018; 26(4):1122-1128
- CONCLUSIONS: Combined detection of JAK2, CALR and MPL gene mutations can cover the vast majority of patients with BCR/ABL1-negative myeloproliferative neoplasms. For higher frequencies of the mutations of CALR in ET patients, CALR mutation can be used as a new diagnostic marker in ET patients with JAK2 and MPL wild type.
- Multiple Placental Infarcts in a Pregnant Woman with Essential Thrombocythemia: A Case Report. [Journal Article]
- IMIntern Med 2018 Aug 10
- Myeloproliferative neoplasms (MPNs), including polycythemia vera, essential thrombocythemia (ET), and primary myelofibrosis, mainly occur in older patients, but have also been reported in younger pat...
Myeloproliferative neoplasms (MPNs), including polycythemia vera, essential thrombocythemia (ET), and primary myelofibrosis, mainly occur in older patients, but have also been reported in younger patients. A "second peak" occurs in female patients in their thirties, particularly in ET; thus, the management of pregnancy is often discussed. We herein present the case of a 33-year-old woman with a high platelet count and multiple placental infarcts during delivery who was subsequently diagnosed with ET. Although there are no worldwide guidelines for the management of MPNs in pregnancy, the risk of thrombosis is markedly increased in these patients, and antithrombotic therapy should be considered.
- Long-term pediatric hematological morbidity of the early-term newborn. [Journal Article]
- EJEur J Pediatr 2018 Aug 07
- CONCLUSIONS: Early-term delivery appears to be independently associated with pediatric long-term hematological morbidity of the offspring. What is Known? • It has been shown that children born at early term are at increased risk for short-term adverse outcomes including perinatal mortality. • Early-term infants are also at increased risk for long-term morbidity, mainly respiratory. What is New? • Early-term delivery is also independently associated with long-term hematological morbidity of the offspring.
- Contemporary management of patients with BCR-ABL1-negative myeloproliferative neoplasms during pregnancy. [Journal Article]
- ERExpert Rev Hematol 2018 Aug 14; :1-10
- The management of pregnancy during the course of BCR-ABL1-negative myeloproliferative neoplasms (MPN) is an increasingly relevant problem. This is mostly due to earlier and better diagnosis of MPN to...
The management of pregnancy during the course of BCR-ABL1-negative myeloproliferative neoplasms (MPN) is an increasingly relevant problem. This is mostly due to earlier and better diagnosis of MPN together with the trend in modern society toward delaying pregnancy until later life. Areas Covered: The present review aims to provide an overview of the available literature data concerning outcome of pregnancy in MPN. Possible therapeutic modalities are discussed and a management algorithm is suggested. Expert Commentary: Most data are available for women with essential thrombocythemia and we present 793 published pregnancies. Live birth rate is 68.5% with 31.5% miscarriages. Spontaneous abortion is the most frequent complication with 26.5%, followed by stillbirth with 4.8%. Maternal complications are relatively low with 1.8% major thrombotic and 2.4% major bleeding events. In polycythemia vera the situation is clinically more complex and roughly 150 pregnancy reports are available. There is very limited information in primary myelofibrosis with less than 20 reported pregnancies. With active management including control of blood counts, aspirin, low molecular weight heparin and in higher risk cases interferon alpha pregnancy in MPN is manageable with a success rate not far below the normal situation with 80%.
New Search Next
- Normal and pathologic erythropoiesis in adults: from gene regulation to targeted treatment concepts. [Journal Article]
- HHaematologica 2018 Aug 03
- Pathologic erythropoiesis with consecutive anemia is a leading cause of symptomatic morbidity in internal medicine. The etiologies of anemia are complex and include reactive as well as neoplastic con...
Pathologic erythropoiesis with consecutive anemia is a leading cause of symptomatic morbidity in internal medicine. The etiologies of anemia are complex and include reactive as well as neoplastic conditions. Clonal expansion of erythroid cells in the bone marrow may result in peripheral erythrocytosis and polycythemia but can also result in anemia when clonal cells are dysplastic and have a maturation arrest that leads to apoptosis and hinders migration, a constellation typically seen in the myelodysplastic syndromes. Rarely, clonal expansion of immature erythroid blasts results in a clinical picture resembling erythroid leukemia. Although several mechanisms underlying normal and abnormal erythropoiesis and the pathogenesis of related disorders have been deciphered in recent years, little is known about specific markers and targets through which prognosis and therapy could be improved in anemic or polycythemic patients. In order to discuss new markers, targets and new therapeutic approaches in erythroid disorders and the related pathologies, a workshop was organized in Vienna in April 2017. The outcomes of this workshop are summarized in this article, and include a discussion of new diagnostic and prognostic markers, the updated WHO classification, and an overview of new drugs used to stimulate or to interfere with erythropoiesis in various neoplastic and reactive conditions. The use and usefulness of established and novel erythropoiesis-stimulating agents in various indications, including myelodysplastic syndromes and other neoplasms, are also discussed.