scholarly journals Lower response to BNT162b2 vaccine in patients with myelofibrosis compared to polycythemia vera and essential thrombocythemia

2021 ◽  
Vol 14 (1) ◽  
Author(s):  
Fulvia Pimpinelli ◽  
Francesco Marchesi ◽  
Giulia Piaggio ◽  
Diana Giannarelli ◽  
Elena Papa ◽  
...  
Keyword(s):  
Prospective Study ◽  
Polycythemia Vera ◽  
Essential Thrombocythemia ◽  
Active Treatment ◽  
Myeloproliferative Neoplasm ◽  
Large Prospective Study ◽  
Lower Response ◽  
Ongoing Treatment ◽  
Vaccine Immunogenicity

AbstractIn a population of 42 Philadelphia negative myeloproliferative neoplasm patients, all on systemic active treatment, the likelihood of responding to anti-SARS-CoV-2 BNT162b2 vaccine at 2 weeks after the second dose was significantly lower in the ten patients with myelofibrosis compared to the 32 with essential thrombocythemia (n = 17) and polycythemia vera (n = 15) grouped together, both in terms of neutralizing anti-SARS-CoV-2 IgG titers and seroprotection rates (32.47 AU/mL vs 217.97 AU/mL, p = 0.003 and 60% vs 93.8%, p = 0.021, respectively). Ruxolitinib, which was the ongoing treatment in five patients with myelofibrosis and three with polycythemia vera, may be implicated in reducing vaccine immunogenicity (p = 0.076), though large prospective study is needed to address this issue.

scholarly journals Distinct clustering of symptomatic burden among myeloproliferative neoplasm patients: retrospective assessment in 1470 patients

Blood ◽  
2014 ◽  
Vol 123 (24) ◽  
pp. 3803-3810 ◽  
Author(s):  
Holly L. Geyer ◽  
Robyn M. Scherber ◽  
Amylou C. Dueck ◽  
Jean-Jacques Kiladjian ◽  
Zhijian Xiao ◽  
...  
Keyword(s):  
Polycythemia Vera ◽  
Essential Thrombocythemia ◽  
Myeloproliferative Neoplasm ◽  
Prognostic Scores ◽  
Retrospective Assessment ◽  
Key Points

Key Points Distinct clusters exist within polycythemia vera, essential thrombocythemia, and myelofibrosis. Clusters are not direct surrogates for current prognostic scores.

Does Occurrence of Other Malignancy after Essential Thrombocythemia and Polycythemia Vera Influence Their Rate of Transformation to Myelofibrosis?

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 5189-5189
Author(s):  
Lucia Masarova ◽  
Naval Daver ◽  
Naveen Pemmaraju ◽  
Prithviraj Bose ◽  
Sherry Pierce ◽  
...  
Keyword(s):  
Polycythemia Vera ◽  
Essential Thrombocythemia ◽  
Research Funding ◽  
Hematologic Malignancy ◽  
Myeloproliferative Neoplasm ◽  
Rank Test ◽  
Control Group ◽  
Transformation Rate ◽  
Similar Time ◽  
Female Ratio

Abstract Introduction: Other malignancies (OM) were reported to be increased in patients with essential thrombocythemia "ET" and polycythemia vera "PV". However, the effect of coexistent OM on the rate of disease transformation to myelofibrosis "MF" is unknown. Objective: To determine the occurrence of OM in patients with ET and PV and their influence on transformation rate to overt MF. Methods: Fisher's exact test, and Mann-Whitney test were used to compare categorical and continuous variable, respectively. Control group without OM matched for age, MPN diagnosis type and year was created to compare transformation rates, expressed by Kaplan-Meier curve and Log rank test. Results: We perform a retrospective chart review for 783 patients referred to our institution between years 1960 - 2013 with diagnosis of Philadelphia-negative myeloproliferative neoplasm "MPN", of which 256 had ET, 165 PV and 362 post ET- or post PV- MF. Overall, 107 (13.7%) patients had OM prior to MF transformation, accounting for total of 125 cases. See table for OM types and distribution. In total, 9 patients had more than 1 OM with the most common being prostatic carcinoma (n=6), melanoma (n=4) and renal cell carcinoma (n=2). Relapsed/refractory OM were noticed in 15 patients, 3 of these had recurrence only prior MPN. None of alive patients with refractory OM experienced progression after MF transformation. Median age at diagnosis of MPN and OM (59 and 62 years), median age at the time of first event (OM or MPN; 51 and 53 years), and male to female ratio (1:1) were similar. Time to secondary OM was shorter than time to secondary MPN (6.5 vs 9 years, p>0.05). After median follow up of 30 months (range, 1-150), 49 (46%) patients had died. Eleven (23%) patients died because of OM progression, most commonly due to lung carcinoma and melanoma. No MPN related deaths were observed, however, 2 patients progressed to acute leukemia. Median overall survival "OS" for MPN was 160 months (range, 1-240) and was not different for patients with and without MF (212 and 173 months; p>0.05). No significant differences were detected between patients with and without MF in terms of age at the time of MPN diagnosis, gender, MPN characteristics (cytogenetic, molecular mutations) or time between OM and MPN. However, patients who transformed to MF were older at the time of OM (53 vs 59 years, p= 0.041, 95% CI: 1.2-11.7). Similarly, within a group of MF, patients with OM prior to MPN were younger at the time of OM (53 vs 62 years, p<0.001, 95%CI 5.0-23.5) and older at the time of MPN (67 vs 55 years, p=0.001, 95%CI 8.3-19.4) that patients who had OM after previous MPN. Overall time to transformation to MF was similar between ET and PV, and between studied cohort and control group (6 years, range 0.5-29, p>0.05, chi square 1.14). However, patients who developed OM after MPN had longer time to transformation (14 years, range 1-36, p=0.011, HR 1.9, 95%CI of 1.3 -4.4), which remained significant when control group was added, p=0.024 [Fig.] Conclusions: OM diagnosed after ET or PV does not seem to influence rate of disease transformation to MF. Table. ET/PV, n=421 ET/PV -> MF, n= 362 OM characteristics OM prior MPN, n OM after MPN, n OM prior MPN, n OM after MPN, n No. of patients with OM 29 26 19 33 No. of OM 30 26 26 43 Breast 5 4 ( 1*) 2 (1*) 3 Female reproductive 5 1 4 Prostate 7 1 6 (1*) 14 (1*) Gastrointestinal tract 2 4 6 3 Genitourinary tract 3 (1*) 4 2 Lungs 1 3 (3*) Melanoma 3 (1*) 2 4 9 (1*) Soft tissue 1 2 (1*) 1 Thyroid 3 2 (1*) 1 2 Head and neck 1 (1*) Hematologic malignancy 3 5 (1*) 6 (1*) (*) stands for relapsed or refractory disease Disclosures Pemmaraju: Stemline: Research Funding; Incyte: Consultancy, Honoraria; Novartis: Consultancy, Honoraria, Research Funding; LFB: Consultancy, Honoraria. Cortes:Pfizer: Consultancy, Research Funding; Teva: Consultancy, Research Funding; BMS: Consultancy, Research Funding; Novartis: Consultancy, Research Funding; ARIAD Pharmaceuticals Inc.: Consultancy, Research Funding.

scholarly journals Hemorrhage in Essential Thrombocythemia or Polycythemia Vera: Epidemiology, Location, Risk Factors, and Lessons Learned from the Literature

2020 ◽  
Author(s):  
Christophe Nicol ◽  
Karine Lacut ◽  
Brigitte Pan-Petesch ◽  
Eric Lippert ◽  
Jean-Christophe Ianotto
Keyword(s):  
Risk Factors ◽  
Polycythemia Vera ◽  
Essential Thrombocythemia ◽  
Myeloproliferative Neoplasm ◽  
Young Patients ◽  
Lessons Learned ◽  
Published Data ◽  
Bleeding Events ◽  
Future Studies ◽  
Increased Risk

AbstractHemorrhage is a well-known complication of essential thrombocythemia (ET) and polycythemia vera (PV), but evidence-based data on its management and prevention are lacking to help inform clinicians. In this review, appropriate published data from the past 15 years regarding bleeding epidemiology, classification, location, and risk factors are presented and discussed. Research was conducted using the Medline database. The bleeding classifications were heterogeneous among the collected studies. The median incidences of bleeding and major bleeding were 4.6 and 0.79% patients/year, in ET patients and 6.5 and 1.05% patients/year in PV patients, respectively. The most frequent location was the gastrointestinal tract. Bleeding accounted for up to 13.7% of deaths, and cerebral bleeding was the main cause of lethal hemorrhage. Thirty-nine potential risk factors were analyzed at least once, but the results were discrepant. Among them, age >60 years, bleeding history, splenomegaly, myeloproliferative neoplasm subtype, and platelet count should deserve more attention in future studies. Among the treatments, aspirin seemed to be problematic for young patients with ET (especially CALR-mutated ET patients) and anagrelide was also identified as a bleeding inducer, especially when associated with aspirin. Future studies should analyze bleeding risk factors in more homogeneous populations and with common bleeding classifications. More tools are needed to help clinicians manage the increased risk of potentially lethal bleeding events in these diseases.

scholarly journals Excess Mortality in Polycythemia Vera and Essential Thrombocythemia

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 3042-3042
Author(s):  
Arturo Pereira ◽  
Carlos Besses Raebel ◽  
Juan Carlos Hernandez Boluda ◽  
Francisca Ferrer Marin ◽  
Carmen Garcia-Hernandez ◽  
...  
Keyword(s):  
Polycythemia Vera ◽  
Board Of Directors ◽  
Essential Thrombocythemia ◽  
Excess Mortality ◽  
Myeloproliferative Neoplasms ◽  
Thrombotic Event ◽  
Myeloproliferative Neoplasm ◽  
Treatment Modalities ◽  
Advisory Committees ◽  
Age Related

Abstract Background and objective. An important proportion of patients with polycythemia vera (PV) and essential thrombocythemia (ET) are diagnosed in the seventh and eighth decades of life. Because of the chronic course of PV and ET and the advanced age of patients, many will actually die from age-related ailments instead of the myeloproliferative neoplasm, so the disease's impact on life-expectancy remains largely unknown. This registry‐based study was aimed at investigating the excess mortality attributable to PV and ET in a large series of patients diagnosed and managed according to modern criteria. Methods. We queried the databases of the Spanish Group for Chronic Myeloproliferative Neoplasms (GEMFIN) to retrieve patients diagnosed with PV or ET after 1980. Criteria for diagnosis and treatment modalities were the internationally recommended ones in each period. Excess mortality was defined as the complementary of the ratio between the actuarial survival observed in the cohort of patients and the expected survival derived from the general Spanish population matched to the patients by age, sex, and calendar year at diagnosis, and was estimated by the Dickman's method (Stat Med 2004;23:51). Spanish life-tables stratified by age, sex, and calendar year were obtained from the Human Mortality Database (www.mortality.org). Results. A total of 3,268 patients were included in the study (PV: 1,731; ET: 1,537). Median age (interquartile range) and sex distribution in the PV and ET groups were 67 (55-75) years, 48% females, and 62 (48-74) years, 61% females, respectively. At the study closing date, 270 (16%) patients with PV and 202 (13%) with ET had died. Estimated median survival was 20.2 years for PV patients and 23.8 years for ET patients. Transformation into acute myeloid leukemia/myelodysplastic syndrome was diagnosed in 76 (4.4%) patients with PV and 63 (4.1%) with ET. Three hundred and seventy-one patients (170 ET, 201 PV) had one or more episodes of thrombosis over the course of the disease. No significant excess mortality attributable to ET was detected at 20 years from diagnosis (figure 1a). In PV, mortality did not significantly deviate from matched general population until 18 years from diagnosis (figure 1b). In both diseases, excess mortality significantly increased after the first recorded thrombotic event (figure 1c). Conclusion. From the population viewpoint, ET and PV do not carry an increased excess mortality until late in the second follow-up decade or later. Nevertheless, appearance of a thrombotic event significantly increases the subsequent excess mortality. Figure 1 Figure 1. Disclosures Hernandez Boluda: Incyte: Consultancy; Novartis: Consultancy. Ferrer Marin:Novartis: Consultancy, Research Funding; Incyte: Consultancy. Gómez-Casares:Bristol-Myers Squibb: Speakers Bureau; Incyte: Speakers Bureau; Novartis: Speakers Bureau. Cervantes:Celgene: Membership on an entity's Board of Directors or advisory committees; Novartis: Honoraria, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Hospital Clinic Barcelona: Employment.

The Myeloproliferative Neoplasm Symptom Assessment Form (MPN-SAF): International Prospective Validation and Reliability Trial in 402 patients

Blood ◽  
2011 ◽  
Vol 118 (2) ◽  
pp. 401-408 ◽  
Author(s):  
Robyn Scherber ◽  
Amylou C. Dueck ◽  
Peter Johansson ◽  
Tiziano Barbui ◽  
Giovanni Barosi ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Polycythemia Vera ◽  
Essential Thrombocythemia ◽  
Myeloproliferative Neoplasms ◽  
Intraclass Correlation ◽  
Myeloproliferative Neoplasm ◽  
Symptom Assessment ◽  
The United States ◽  
Life Questionnaire

Abstract Symptomatic burden in myeloproliferative neoplasms is present in most patients and compromises quality of life. We sought to validate a broadly applicable 18-item instrument (Myeloproliferative Neoplasm Symptom Assessment Form [MPN-SAF], coadministered with the Brief Fatigue Inventory) to assess symptoms of myelofibrosis, essential thrombocythemia, and polycythemia vera among prospective cohorts in the United States, Sweden, and Italy. A total of 402 MPN-SAF surveys were administered (English [25%], Italian [46%], and Swedish [28%]) in 161 patients with essential thrombocythemia, 145 patients with polycythemia vera, and 96 patients with myelofibrosis. Responses among the 3 administered languages showed great consistency after controlling for MPN subtype. Strong correlations existed between individual items and key symptomatic elements represented on both the MPN-SAF and the European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire-C30. Enrolling physicians' blinded opinion of patient symptoms (6 symptoms assessed) were highly correlated with corresponding patients' responses. Serial administration of the English MPN-SAF among 53 patients showed that most MPN-SAF items are well correlated (r > 0.5, P < .001) and highly reproducible (intraclass correlation coefficient > 0.7). The MPN-SAF is a comprehensive and reliable instrument that is available in multiple languages to evaluate symptoms associated with all types of MPNs in clinical trials globally.

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