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[PMID]:29396713
[Au] Autor:Kiladjian JJ; Guglielmelli P; Griesshammer M; Saydam G; Masszi T; Durrant S; Passamonti F; Jones M; Zhen H; Li J; Gadbaw B; Perez Ronco J; Khan M; Verstovsek S
[Ad] Endereço:Centre d'Investigations Cliniques (CIC1427), Hôpital Saint-Louis, AP-HP, INSERM, CLIP2 "Saint-Louis - Paris Nord," Early Phase Research Center, Université Paris Diderot, 1, Avenue Claude Vellefaux, 75010, Paris, France. jean-jacques.kiladjian@aphp.fr.
[Ti] Título:Efficacy and safety of ruxolitinib after and versus interferon use in the RESPONSE studies.
[So] Source:Ann Hematol;97(4):617-627, 2018 Apr.
[Is] ISSN:1432-0584
[Cp] País de publicação:Germany
[La] Idioma:eng
[Ab] Resumo:Ruxolitinib was well tolerated and superior to best available therapy (including interferon [IFN]) in controlling hematocrit without phlebotomy eligibility, normalizing blood counts, and improving polycythemia vera-related symptoms in the Study of Efficacy and Safety in Polycythemia Vera Subjects Who Are Resistant to or Intolerant of Hydroxyurea: JAK Inhibitor INC424 (INCB018424) Tablets Versus Best Available Care (RESPONSE) studies. This ad hoc analysis focuses on ruxolitinib in relation to IFN in the RESPONSE studies, with attention on the following: (1) safety and efficacy of ruxolitinib and best available therapy in patients who received IFN before study randomization, (2) safety and efficacy of IFN during randomized treatment in best available therapy arm, and (3) use of ruxolitinib after crossover from best available therapy in IFN-treated patients. IFN exposure before randomization had little effect on the efficacy or safety of ruxolitinib. In the randomized treatment arms, ruxolitinib was superior to IFN in efficacy [hematocrit control (RESPONSE = 60% of ruxolitinib vs 23% of IFN patients; RESPONSE-2 = 62% of ruxolitinib vs 15% of IFN patients)] and was tolerated better in hydroxyurea-resistant or hydroxyurea-intolerant patients. After crossing over to receive ruxolitinib, patients who had initially received IFN and did not respond had improved hematologic and spleen responses (62% of patients at any time after crossover) and an overall reduction in phlebotomy procedures. Rates and incidences of the most common adverse events decreased after crossover to ruxolitinib, except for infections (primarily grade 1 or 2). These data suggest that ruxolitinib is efficacious and well tolerated in patients who were previously treated with IFN. The RESPONSE (NCT01243944) and RESPONSE-2 (NCT02038036) studies were registered at clinicaltrials.gov .
[Mh] Termos MeSH primário: Antineoplásicos/uso terapêutico
Interferons/uso terapêutico
Janus Quinases/antagonistas & inibidores
Policitemia Vera/tratamento farmacológico
Inibidores de Proteínas Quinases/uso terapêutico
Pirazóis/uso terapêutico
[Mh] Termos MeSH secundário: Adulto
Idoso
Antineoplásicos/efeitos adversos
Sangria/efeitos adversos
Terapia Combinada/efeitos adversos
Estudos Cross-Over
Monitoramento de Medicamentos
Resistência a Múltiplos Medicamentos
Resistência a Medicamentos Antineoplásicos
Feminino
Seres Humanos
Hidroxiureia/efeitos adversos
Hidroxiureia/uso terapêutico
Interferons/efeitos adversos
Janus Quinases/metabolismo
Masculino
Meia-Idade
Policitemia Vera/metabolismo
Policitemia Vera/fisiopatologia
Policitemia Vera/terapia
Padrões de Prática Médica
Inibidores de Proteínas Quinases/efeitos adversos
Pirazóis/efeitos adversos
Reprodutibilidade dos Testes
Esplenomegalia/etiologia
Esplenomegalia/prevenção & controle
[Pt] Tipo de publicação:CLINICAL TRIAL; CLINICAL TRIAL, PHASE III; COMPARATIVE STUDY; EQUIVALENCE TRIAL; JOURNAL ARTICLE; RANDOMIZED CONTROLLED TRIAL
[Nm] Nome de substância:
0 (Antineoplastic Agents); 0 (INCB018424); 0 (Protein Kinase Inhibitors); 0 (Pyrazoles); 9008-11-1 (Interferons); EC 2.7.10.2 (Janus Kinases); X6Q56QN5QC (Hydroxyurea)
[Em] Mês de entrada:1803
[Cu] Atualização por classe:180309
[Lr] Data última revisão:
180309
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:180204
[Cl] Clinical Trial:ClinicalTrial
[St] Status:MEDLINE
[do] DOI:10.1007/s00277-017-3225-1


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[PMID]:29285580
[Au] Autor:Hidalgo López JE; Carballo-Zarate A; Verstovsek S; Wang SA; Hu S; Li S; Xu J; Zuo W; Tang Z; Yin CC; Medeiros LJ; Bueso-Ramos CE; Tang G
[Ad] Endereço:Department of Hematopathology, Unit 0072, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX, 77030, USA.
[Ti] Título:Bone marrow findings in blast phase of polycythemia vera.
[So] Source:Ann Hematol;97(3):425-434, 2018 Mar.
[Is] ISSN:1432-0584
[Cp] País de publicação:Germany
[La] Idioma:eng
[Ab] Resumo:Approximately 10% of patients with polycythemia vera (PV) transform to acute leukemia (blast phase) at 10 years after initial diagnosis of PV. The bone marrow pathologic, cytogenetic, and molecular features of blast phase have not been well characterized. In this study, we reviewed 422 PV patients over a period of 11 years and identified 58 patients who developed acute myeloid leukemia (blast phase) during the course of disease. We found that blast phase of PV was characterized by overt myelodysplasia (n = 51, 88%); moderate to severe myelofibrosis (33 of 45, 73%); an abnormal karyotype (n = 51, 88%) that was often complex karyotype (n = 42, 72%); and gene mutations involving TP53 (55%), TET2 (27%), and DNMT3A (25%). Patients with blast phase of PV had an aggressive clinical course, with a median overall survival of 4 months after onset of blast phase. Eleven patients had close follow-up from polycythemic phase to blast phase: Four patients showed dysplastic changes in the polycythemic phase, and three of them transformed to blast phase without a "middle phase" of post-PV myelofibrosis.We conclude that blast phase of PV is characterized by myelodysplasia, moderate to severe fibrosis, a high frequency of an abnormal and often complex karyotype, and frequentTP53mutation.
[Mh] Termos MeSH primário: Crise Blástica/patologia
Medula Óssea/patologia
Leucemia Mieloide Aguda/patologia
Policitemia Vera/patologia
[Mh] Termos MeSH secundário: Adulto
Idoso
Idoso de 80 Anos ou mais
Crise Blástica/genética
Medula Óssea/metabolismo
Transformação Celular Neoplásica/genética
Transformação Celular Neoplásica/patologia
Análise Citogenética
Progressão da Doença
Feminino
Seres Humanos
Leucemia Mieloide Aguda/genética
Masculino
Meia-Idade
Policitemia Vera/genética
Estudos Retrospectivos
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180215
[Lr] Data última revisão:
180215
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171230
[St] Status:MEDLINE
[do] DOI:10.1007/s00277-017-3211-7


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[PMID]:29181548
[Au] Autor:Senín A; Fernández-Rodríguez C; Bellosillo B; Camacho L; Longarón R; Angona A; Besses C; Álvarez-Larrán A
[Ad] Endereço:Hematology Department, Hospital del Mar-IMIM (Institut Hospital del Mar d'Investigacions Mèdiques), Universidad Autónoma de Barcelona, Passeig Marítim 25-29, 08003, Barcelona, Spain.
[Ti] Título:Non-driver mutations in patients with JAK2V617F-mutated polycythemia vera or essential thrombocythemia with long-term molecular follow-up.
[So] Source:Ann Hematol;97(3):443-451, 2018 Mar.
[Is] ISSN:1432-0584
[Cp] País de publicação:Germany
[La] Idioma:eng
[Ab] Resumo:JAK2V617F monitoring and NGS of non-driver genes was performed in 100 patients with polycythemia vera (PV) or essential thrombocythemia (ET) with long molecular follow-up. Patients who did not progress to myelofibrosis (MF) or acute myeloid leukemia (AML) after more than 10 years (n = 50) showed a low frequency of mutations at first sample (18%) and an incidence rate of 1.7 new mutations × 100 person-years. Mutations were detected at first sample in 83% of PV/ET patients who later progressed to AML (n = 12) with these patients having a rate of 25.6 mutations × 100 person-years. Presence of mutations at diagnosis was the unique risk factor for acquiring a new genetic event (HR 2.7, 95% CI 1.1-6.8, p = 0.03) after correction for age, PV diagnosis, and total duration of hydroxyurea (HU) exposure. Patients with additional mutation at first sample showed a higher probability of developing cytopenia under HU therapy and a higher risk of AML (HR 12.2, 95% CI 2.6-57.1, p = 0.001) with mutations in ASXL1 (p < 0.0001), TP53 (p = 0.01), SRSF2 (p < 0.0001), IDH1/2 (p < 0.0001), and RUNX1 (p < 0.0001) being associated with a higher probability of AML. Myelofibrotic transformation was more frequent in patients with additional mutations, especially in SF3B1 (p = 0.02) and IDH1/2 (p < 0.0001) although a persistently high or a progressive increase of the JAK2V617F allele burden while receiving cytoreduction was the strongest predictor of MF transformation (HR 10.8, 95% CI 2.4-49.1, p = 0.002). In conclusion, NGS may be useful to identify a minority of PV and ET patients with high genetic instability and increased risk of AML transformation.
[Mh] Termos MeSH primário: Transformação Celular Neoplásica/genética
Janus Quinase 2/genética
Mutação de Sentido Incorreto
Policitemia Vera/genética
Trombocitemia Essencial/genética
[Mh] Termos MeSH secundário: Adulto
Idoso
Idoso de 80 Anos ou mais
Alelos
Substituição de Aminoácidos
Análise Citogenética
Progressão da Doença
Feminino
Seguimentos
Frequência do Gene
Seres Humanos
Masculino
Meia-Idade
Fenilalanina/genética
Policitemia Vera/patologia
Trombocitemia Essencial/patologia
Valina/genética
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
47E5O17Y3R (Phenylalanine); EC 2.7.10.2 (JAK2 protein, human); EC 2.7.10.2 (Janus Kinase 2); HG18B9YRS7 (Valine)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180215
[Lr] Data última revisão:
180215
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171129
[St] Status:MEDLINE
[do] DOI:10.1007/s00277-017-3193-5


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[PMID]:28464892
[Au] Autor:Li B; Gale RP; Xu Z; Qin T; Song Z; Zhang P; Bai J; Zhang L; Zhang Y; Liu J; Huang G; Xiao Z
[Ad] Endereço:MDS and MPN Centre, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, 288 Nanjing Road, Tianjin, 300020, China.
[Ti] Título:Non-driver mutations in myeloproliferative neoplasm-associated myelofibrosis.
[So] Source:J Hematol Oncol;10(1):99, 2017 May 02.
[Is] ISSN:1756-8722
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:We studied non-driver mutations in 62 subjects with myeloproliferative neoplasm (MPN)-associated myelofibrosis upon diagnosis, including 45 subjects with primary myelofibrosis (PMF) and 17 with post-polycythemia vera or post-essential thrombocythemia myelofibrosis (post-PV/ET MF). Fifty-eight subjects had ≥1 non-driver mutation upon diagnosis. Mutations in mRNA splicing genes, especially in U2AF1, were significantly more frequent in PMF than in post-PV/ET MF (33 vs. 6%; P = 0.015). There were also striking differences in clonal architecture. These data indicate different genomic spectrums between PMF and post-PV/ET MF.
[Mh] Termos MeSH primário: Mutação
Policitemia Vera/genética
Mielofibrose Primária/genética
[Mh] Termos MeSH secundário: Adulto
Idoso
Idoso de 80 Anos ou mais
Apoptose/genética
Adesão Celular/genética
Ciclo Celular/genética
Montagem e Desmontagem da Cromatina/genética
Células Clonais
Metilação de DNA/genética
Reparo do DNA/genética
Feminino
Seres Humanos
Masculino
Meia-Idade
Policitemia Vera/complicações
Mielofibrose Primária/etiologia
Processamento de RNA/genética
Transdução de Sinais/genética
Fator de Processamento U2AF/genética
Trombocitemia Essencial/complicações
Trombocitemia Essencial/genética
Adulto Jovem
[Pt] Tipo de publicação:COMPARATIVE STUDY; LETTER
[Nm] Nome de substância:
0 (Splicing Factor U2AF); 0 (U2AF1 protein, human)
[Em] Mês de entrada:1801
[Cu] Atualização por classe:180112
[Lr] Data última revisão:
180112
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170504
[St] Status:MEDLINE
[do] DOI:10.1186/s13045-017-0472-5


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[PMID]:28934680
[Au] Autor:Verstovsek S; Mesa RA; Salama ME; Li L; Pitou C; Nunes FP; Price GL; Giles JL; D'Souza DN; Walgren RA; Prchal JT
[Ad] Endereço:The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd., Houston, TX 77030, USA. Electronic address: sverstov@mdanderson.org.
[Ti] Título:A phase 1 study of the Janus kinase 2 (JAK2) inhibitor, gandotinib (LY2784544), in patients with primary myelofibrosis, polycythemia vera, and essential thrombocythemia.
[So] Source:Leuk Res;61:89-95, 2017 Oct.
[Is] ISSN:1873-5835
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Mutations in Janus kinase 2 (JAK2) are implicated in the pathogenesis of Philadelphia-chromosome negative myeloproliferative neoplasms, including primary myelofibrosis, polycythemia vera, and essential thrombocythemia. Gandotinib (LY2784544), a potent inhibitor of JAK2 activity, shows increased potency for the JAK2 mutation. The study had a standard 3+3 dose-escalation design to define the maximum-tolerated dose. Primary objectives were to determine safety, tolerability, and recommended oral daily dose of gandotinib for patients with JAK2 -positive myelofibrosis, essential thrombocythemia, or polycythemia vera. Secondary objectives included estimating pharmacokinetic parameters and documenting evidence of efficacy by measuring clinical improvement. Thirty-eight patients were enrolled and treated (31 myelofibrosis, 6 polycythemia vera, 1 essential thrombocythemia). The maximum-tolerated dose of gandotinib was 120mg daily, based on dose-limiting toxicities of blood creatinine increase or hyperuricemia at higher doses. Maximum plasma concentration was reached 4h after single and multiple doses, and mean half-life on day 1 was approximately 6h. Most common treatment-emergent adverse events were diarrhea (55.3%) and nausea (42.1%), a majority of which were of grade 1 severity. Best response of clinical improvement was achieved by 29% of myelofibrosis patients. A ≥50% palpable spleen length reduction was observed at any time during therapy in 20/32 evaluable patients. Additionally, ≥50% reduction in the Total Symptom Myeloproliferative Neoplasm Symptom Assessment Form Score was seen in 11/21 (52%) and 6/14 patients (43%) receiving ≥120mg at 12 and 24 weeks respectively. Gandotinib demonstrated an acceptable safety and tolerability profile, and findings at the maximum-tolerated dose of 120mg supported further clinical testing. Clinicaltrials.gov identifier: NCT01134120.
[Mh] Termos MeSH primário: Antineoplásicos/uso terapêutico
Imidazóis/uso terapêutico
Janus Quinase 2/antagonistas & inibidores
Policitemia Vera/tratamento farmacológico
Mielofibrose Primária/tratamento farmacológico
Pirazóis/uso terapêutico
Piridazinas/uso terapêutico
Trombocitemia Essencial/tratamento farmacológico
[Mh] Termos MeSH secundário: Adulto
Idoso
Feminino
Seres Humanos
Masculino
Dose Máxima Tolerável
Meia-Idade
Inibidores de Proteínas Quinases/uso terapêutico
[Pt] Tipo de publicação:CLINICAL TRIAL, PHASE I; JOURNAL ARTICLE; MULTICENTER STUDY
[Nm] Nome de substância:
0 (Antineoplastic Agents); 0 (Imidazoles); 0 (LY2784544); 0 (Protein Kinase Inhibitors); 0 (Pyrazoles); 0 (Pyridazines); EC 2.7.10.2 (JAK2 protein, human); EC 2.7.10.2 (Janus Kinase 2)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171017
[Lr] Data última revisão:
171017
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170922
[St] Status:MEDLINE


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[PMID]:28859112
[Au] Autor:Andersen M; Sajid Z; Pedersen RK; Gudmand-Hoeyer J; Ellervik C; Skov V; Kjær L; Pallisgaard N; Kruse TA; Thomassen M; Troelsen J; Hasselbalch HC; Ottesen JT
[Ad] Endereço:Department of Science and Environment, Roskilde University, Roskilde, Denmark.
[Ti] Título:Mathematical modelling as a proof of concept for MPNs as a human inflammation model for cancer development.
[So] Source:PLoS One;12(8):e0183620, 2017.
[Is] ISSN:1932-6203
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:The chronic Philadelphia-negative myeloproliferative neoplasms (MPNs) are acquired stem cell neoplasms which ultimately may transform to acute myelogenous leukemia. Most recently, chronic inflammation has been described as an important factor for the development and progression of MPNs in the biological continuum from early cancer stage to the advanced myelofibrosis stage, the MPNs being described as "A Human Inflammation Model for Cancer Development". This novel concept has been built upon clinical, experimental, genomic, immunological and not least epidemiological studies. Only a few studies have described the development of MPNs by mathematical models, and none have addressed the role of inflammation for clonal evolution and disease progression. Herein, we aim at using mathematical modelling to substantiate the concept of chronic inflammation as an important trigger and driver of MPNs.The basics of the model describe the proliferation from stem cells to mature cells including mutations of healthy stem cells to become malignant stem cells. We include a simple inflammatory coupling coping with cell death and affecting the basic model beneath. First, we describe the system without feedbacks or regulatory interactions. Next, we introduce inflammatory feedback into the system. Finally, we include other feedbacks and regulatory interactions forming the inflammatory-MPN model. Using mathematical modeling, we add further proof to the concept that chronic inflammation may be both a trigger of clonal evolution and an important driving force for MPN disease progression. Our findings support intervention at the earliest stage of cancer development to target the malignant clone and dampen concomitant inflammation.
[Mh] Termos MeSH primário: Inflamação/genética
Modelos Teóricos
Transtornos Mieloproliferativos/genética
Neoplasias/genética
[Mh] Termos MeSH secundário: Carcinogênese/genética
Evolução Clonal/genética
Progressão da Doença
Seres Humanos
Inflamação/patologia
Janus Quinase 2/genética
Transtornos Mieloproliferativos/patologia
Neoplasias/patologia
Células-Tronco Neoplásicas/patologia
Policitemia Vera/genética
Policitemia Vera/patologia
Trombocitemia Essencial/genética
Trombocitemia Essencial/patologia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
EC 2.7.10.2 (Janus Kinase 2)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171013
[Lr] Data última revisão:
171013
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170901
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0183620


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[PMID]:28778261
[Au] Autor:Tefferi A; Vannucchi AM
[Ad] Endereço:Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN. Electronic address: tefferi.ayalew@mayo.edu.
[Ti] Título:Genetic Risk Assessment in Myeloproliferative Neoplasms.
[So] Source:Mayo Clin Proc;92(8):1283-1290, 2017 Aug.
[Is] ISSN:1942-5546
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo: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.
[Mh] Termos MeSH primário: Transtornos Mieloproliferativos/diagnóstico
Transtornos Mieloproliferativos/genética
Medição de Risco
[Mh] Termos MeSH secundário: Seres Humanos
Janus Quinase 2
Mutação
Transtornos Mieloproliferativos/mortalidade
Fenótipo
Policitemia Vera/genética
Prognóstico
Trombocitemia Essencial/genética
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
EC 2.7.10.2 (JAK2 protein, human); EC 2.7.10.2 (Janus Kinase 2)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170822
[Lr] Data última revisão:
170822
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170806
[St] Status:MEDLINE


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[PMID]:28700486
[Au] Autor:Guo Y; Xu W; Dong L; Huang N; Bi K
[Ad] Endereço:Department of Hematology, Shandong Provincial Qianfoshan Hospital Affiliated to Shandong University, Jinan, Republic of China.
[Ti] Título:Progression of primary myelofibrosis to polycythemia vera: A case report.
[So] Source:Medicine (Baltimore);96(28):e7464, 2017 Jul.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: This case report describes the progression of primary myelofibrosis (PMF) to polycythemia vera (PV), and discuss its potential mechanisms. PATIENT CONCERNS: The patient was admitted because of abdominal discomfort and enlarged spleen for 19 months. DIAGNOSIS: A case of PMF progressed to PV was retrospectively analyzed. There were 19 months between the diagnosis of PMF and PV. The JAK2 V617F mutation was positive before and after the diagnosis of PV; however, new chromosomal abnormalities were detected during the progression. INTERVENTIONS: For treatment of PMF, the danazol, calcitriol, and thalidomide were given. Then, the use of thalidomide and calcitriol was stopped, and hydroxyurea was started. For treatment of PV, interferon treatment was given, whereas hydroxyurea was continued. OUTCOMES: After 30 months of the progression (at the recent follow-up), this patient had no obvious symptoms or thrombosis. LESSONS: PMF rarely progresses to PV, however, the progression will significantly improve the quality of life and prognosis.
[Mh] Termos MeSH primário: Policitemia Vera/fisiopatologia
Mielofibrose Primária/fisiopatologia
[Mh] Termos MeSH secundário: Progressão da Doença
Feminino
Seres Humanos
Meia-Idade
Policitemia Vera/diagnóstico
Policitemia Vera/tratamento farmacológico
Policitemia Vera/genética
Mielofibrose Primária/diagnóstico
Mielofibrose Primária/tratamento farmacológico
Mielofibrose Primária/genética
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1707
[Cu] Atualização por classe:170730
[Lr] Data última revisão:
170730
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170713
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000007464


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[PMID]:28699191
[Au] Autor:Barbui T; Vannucchi AM; Finazzi G; Finazzi MC; Masciulli A; Carobbio A; Ghirardi A; Tognoni G
[Ad] Endereço:F.R.O.M. Research Foundation, Papa Giovanni XXIII Hospital, Bergamo, Italy.
[Ti] Título:A reappraisal of the benefit-risk profile of hydroxyurea in polycythemia vera: A propensity-matched study.
[So] Source:Am J Hematol;92(11):1131-1136, 2017 Nov.
[Is] ISSN:1096-8652
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:The use of hydroxyurea (HU) as first line therapy in polycythemia vera (PV) has been criticized because no solid demonstration that this drug prevents thrombosis or prolongs survival has been so far produced. Here we present the outcomes of a large cohort of patients with PV included in the European Collaborative Low-dose Aspirin (ECLAP) study. We selected 1,042 patients who, during the follow-up, had received only phlebotomy (PHL) or HU to maintain the hematocrit level < 45%. To assure comparability, we conducted a propensity score matching analysis. The two groups (PHL n = 342 and HU n = 681) were well balanced for the parameters included in the propensity score (overall balance: χ = 2.44, P = 0.964). Over a comparable period of follow-up (PHL = 29.9 vs. HU = 34.7 months), we documented an advantage of HU over PHL consistently significant with respect to the incidence of fatal/non-fatal cardiovascular (CV) events (5.8 vs. 3.0 per 100 person-years in PHL vs. HU group, P = 0.002) and myelofibrosis transformation that was only experienced by patients of PHL group. Evolution to acute leukemia was registered in three patients (two in PHL and one in HU group). The excess of mortality and total CV events in the PHL patients was restricted to the high-risk group, and, compared with HU cases, was significant higher in the PHL patients who failed to reach the hematocrit target < 0.45% (P = 0.000). In conclusion, this analysis provides reliable and qualified estimates of the therapeutic profile of HU and PHL treatments for future experimental studies and for the management of PV in clinical practice.
[Mh] Termos MeSH primário: Antineoplásicos/uso terapêutico
Hidroxiureia/uso terapêutico
Policitemia Vera/tratamento farmacológico
[Mh] Termos MeSH secundário: Antineoplásicos/administração & dosagem
Antineoplásicos/efeitos adversos
Biomarcadores
Terapia Combinada
Comorbidade
Feminino
Seguimentos
Hematócrito
Seres Humanos
Hidroxiureia/administração & dosagem
Hidroxiureia/efeitos adversos
Masculino
Flebotomia/métodos
Policitemia Vera/diagnóstico
Policitemia Vera/mortalidade
Pontuação de Propensão
Fatores de Risco
Análise de Sobrevida
Resultado do Tratamento
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Antineoplastic Agents); 0 (Biomarkers); X6Q56QN5QC (Hydroxyurea)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171024
[Lr] Data última revisão:
171024
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170713
[St] Status:MEDLINE
[do] DOI:10.1002/ajh.24851


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Texto completo
[PMID]:28685840
[Au] Autor:Kvasnicka HM; Orazi A; Thiele J; Barosi G; Bueso-Ramos CE; Vannucchi AM; Hasserjian RP; Kiladjian JJ; Gianelli U; Silver R; Mughal TI; Barbui T
[Ad] Endereço:Senckenberg Institute of Pathology, University of Frankfurt, Germany.
[Ti] Título:European LeukemiaNet study on the reproducibility of bone marrow features in masked polycythemia vera and differentiation from essential thrombocythemia.
[So] Source:Am J Hematol;92(10):1062-1067, 2017 Oct.
[Is] ISSN:1096-8652
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:The purpose of the study was to assess consensus and interobserver agreement among an international panel of six hematopathologists regarding characterization and reproducibility of bone marrow (BM) histologic features used to diagnose early stage myeloproliferative neoplasms, in particular differentiation of so-called masked/prodromal polycythemia vera (mPV) from JAK2-mutated essential thrombocythemia (ET). The six members of the hematopathology panel evaluated 98 BM specimens independently and in a blinded fashion without knowledge of clinical data. The specimens included 48 cases of mPV according to the originally published hemoglobin threshold values for this entity (male: 16.0-18.4 g/dL, female: 15.0-16.4 g/dL), 31 cases with overt PV according to the updated 2016 WHO criteria, and 19 control cases. The latter group included cases of JAK2-mutated ET, primary myelofibrosis, myelodysplastic syndrome, and various reactive conditions. Inter-rater agreement between the panelists was very high (overall agreement 92.6%, kappa 0.812), particularly with respect to separating mPV from ET. Virtually all cases of mPV were correctly classified as PV according to their BM morphology. In conclusion, a central blinded review of histology slides by six hematopathologists demonstrated that highly reproducible specific histological pattern characterize PV and confirmed the notion that there are no significant differences between mPV and overt PV in relation to BM morphology.
[Mh] Termos MeSH primário: Medula Óssea/patologia
Policitemia Vera/patologia
Trombocitemia Essencial/patologia
[Mh] Termos MeSH secundário: Diagnóstico Diferencial
Europa (Continente)
Feminino
Hemoglobinas/análise
Seres Humanos
Janus Quinase 2/genética
Masculino
Mutação
Policitemia Vera/sangue
Policitemia Vera/genética
Reprodutibilidade dos Testes
Trombocitemia Essencial/sangue
Trombocitemia Essencial/genética
Organização Mundial da Saúde
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Hemoglobins); EC 2.7.10.2 (JAK2 protein, human); EC 2.7.10.2 (Janus Kinase 2)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170925
[Lr] Data última revisão:
170925
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170708
[St] Status:MEDLINE
[do] DOI:10.1002/ajh.24837



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