Symptomatic Profiles of Patients With Polycythemia Vera: Implications of Inadequately Controlled Disease Hematologic Malignancy

Conclusion The results of this study suggest that patients with PV who have any one of the features in question (known HU use, known phlebotomy, or splenomegaly) have significant PV-associated symptoms. Furthermore, it demonstrates that many PV symptoms remain severe independent of the number of features present.
Source: Journal of Clinical Oncology - Category: Cancer & Oncology Authors: Tags: Translational Oncology, Quality of Life Hematologic Malignancy Source Type: research

Related Links:

This study is registered with ClinicalTrials.gov, NCT01243944.FindingsWe enrolled patients between Oct 27, 2010, and Feb 13, 2013, and the study concluded on Feb 9, 2018. Of 342 individuals screened for eligibility, 222 patients were randomly assigned to receive ruxolitinib (n=110, 50%) or best available therapy (n=112, 50%). The median time since polycythaemia vera diagnosis was 8·2 years (IQR 3·9–12·3) in the ruxolitinib group and 9·3 years (4·9–13·8) in the best available therapy group. 98 (88%) of 112 patients initially randomly assigned to best available therapy cr...
Source: The Lancet Haematology - Category: Hematology Source Type: research
AbstractThromboembolic events and cardiovascular disease are the most prevalent complications in patients with polycythemia vera (PV) compared with other myeloproliferative disorders and are the major cause of morbidity and mortality in this population. Moreover, a vascular complication such as arterial or venous thrombosis often leads to the diagnosis of PV. The highest rates of thrombosis typically occur shortly before or at diagnosis and decrease over time, probably due to the effects of treatment. Important risk factors include age ( ≥ 60 years old) and a history of thrombosis; elevated hematocrit and le...
Source: Annals of Hematology - Category: Hematology Source Type: research
Authors: Foucar CE, Stein BL Abstract Polycythemia vera (PV) is the most common Philadelphia chromosome-negative myeloproliferative neoplasm. Whereas low-risk patients are treated with aspirin and phlebotomy, high-risk patients receive cytoreductive therapy, which most commonly consists of hydroxyurea in the United States. Concerns about the long-term safety of hydroxyurea, as well as a desire for more efficacious and targeted therapy, have led to the development of novel therapies for high-risk patients with PV. Pegylated interferon (IFN) has shown promise in phase 2 studies of PV, and preliminary data from ongoin...
Source: Clinical Advances in Hematology and Oncology - Category: Cancer & Oncology Tags: Clin Adv Hematol Oncol Source Type: research
Discussion: TE was the most common Ph-MPN. The epidemiological and blood count findings were similar to the data reported in the literature. It is important to note that with the 2016 WHO classification new criteria, some of patients diagnosed with ET, now will be in PV cathegory (21 patients in our serie). The distribution of JAK2V617F+ in Ph-MPN was similar to the published data, except for PV, in which we found a lower percentage of JAK2+. Thrombosis were lower than the data reported for PV. It is worrisome that bone marrow biopsy and cytogenetic study were performed only in a low percentage of the patients. The treatme...
Source: Blood - Category: Hematology Authors: Tags: 634. Myeloproliferative Syndromes: Clinical Source Type: research
CONCLUSIONThe considered clinical and biological features, at MPN diagnosis, were not different in cases with SC and controls. During the course of the disease, three factors significantly and independently affected the risk of SC in these MPN patients: 1) patients with PV had a 77% higher risk than those with ET, 2) patients with MPN duration of more than 5 years had a twice higher risk than those with lower duration, 3) for the first time, we documented that in non-skin solid cancers, aspirin treatment reduced SC risk of 38%. Exposure to HU and other cytoreductive drugs was confirmed as a risk factor for non-melanoma ski...
Source: Blood - Category: Hematology Authors: Tags: 634. Myeloproliferative Syndromes: Clinical: Poster III Source Type: research
ConclusionChronic pain (abdominal, bone) is a prevalent and undermanaged feature of MPN disorders. Chronic non-MPN pain is further complicated by the development of the disorder. Many MPN patients lack understanding on its prevalence and furthermore feel ill-equipped to manage it. Patients have, however, found symptomatic benefit from various pharmacological and non-pharmacological treatment modalities that require further exploration. Results from this study suggest MPN patients should be assessed regularly for pain and offered early referral to specialized services for evaluation, source identification and treatment.Tabl...
Source: Blood - Category: Hematology Authors: Tags: 634. Myeloproliferative Syndromes: Clinical: Poster III Source Type: research
ConclusionsIn this analysis of prospectively gathered real-world data, the MPN-SAF TSS results confirm that patients with PV experience a recognizable constellation of symptoms, including fatigue, early satiety, inactivity, and itching. Not surprisingly, PV-related symptoms have a negative impact on QOL. There were moderate correlations (r = 0.5-0.75) between the MPN-SAF TSS and the EORTC QLC-C30 with respect to global health status/QOL, the 5 functional scales, and fatigue, pain, and dyspnea. Consistent with the previous analysis, this analysis provides further evidence that the MPN-SAF TSS represents an accurate, yet sim...
Source: Blood - Category: Hematology Authors: Tags: 902. Health Services Research-Malignant Diseases: Poster I Source Type: research
ConclusionIn HU resistant/intolerant PV pts, clinical benefits of rux treatment (Hct control and CLHM) were durable with long-term therapy. Considering that the OS findings from this analysis are confounded by extensive CO, the observed HR from this analysis represents a conservative estimate of rux benefit. The long-term safety was consistent with previous findings.DisclosuresKiladjian: AOP Orphan: Membership on an entity's Board of Directors or advisory committees, Research Funding; Novartis: Membership on an entity's Board of Directors or advisory committees, Research Funding; Celgene: Membership on an entity's Board of...
Source: Blood - Category: Hematology Authors: Tags: 634. Myeloproliferative Syndromes: Clinical: Poster I Source Type: research
BACKGROUNDRuxolitinib (RUX), a potent Janus kinase (JAK)1/JAK2 inhibitor, is approved for hydroxyurea (HU)-resistant/-intolerant patients (pts) with polycythemia vera (PV) based on findings from the RESPONSE study (NCT01243944). RUX proved superior to best available therapy (BAT) in maintaining hematocrit (Hct) control without phlebotomy eligibility, normalizing blood cell count, reducing spleen volume, and improving symptoms in pts with PV with splenomegaly who are resistant to or intolerant of HU. RESPONSE-2 (NCT02038036) is a global, multicenter, open-label, phase 3 trial comparing RUX with BAT in HU-resistant or -intol...
Source: Blood - Category: Hematology Authors: Tags: 634. Myeloproliferative Syndromes: Clinical: Poster I Source Type: research
Conclusion. No significant difference in overall survival was observed between PV patients treated with initial rIFNa compared to initial HU or PHL-O. Initial treatment with rIFNa was associated with a lower risk of myelofibrotic progression. However, this risk was not statistically significant after adjusting for age at diagnosis, which was a predictor of myelofibrosis progression in this sample cohort. Finally, the risk-adjusted incidence of cardiovascular events was lower during IFNa therapy than HU therapy. A larger cohort is needed to validate these findings and accordingly, the data collection and analysis for our to...
Source: Blood - Category: Hematology Authors: Tags: 634. Myeloproliferative Syndromes: Clinical: Poster III Source Type: research
More News: Cancer & Oncology | Hematology | Laboratory Medicine | Myeloproliferative Disorders | Phlebotomy | Study