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2. Myelofibrosis

Myelofibrosis Myelofibrosis - Symptoms, diagnosis and treatment | BMJ Best Practice You'll need a subscription to access all of BMJ Best Practice Search  Myelofibrosis Last reviewed: February 2019 Last updated: July 2018 Summary Myelofibrosis is a reactive and reversible process common to many malignant and benign bone marrow disorders. Primary myelofibrosis (PMF) is a chronic progressive myeloproliferative disorder with a median survival (around 5.5 years) much shorter than that of other (...) . Haematopoietic stem cell transplant is the only treatment option with a potential for cure. Definition Myelofibrosis is a reactive and reversible process common to many malignant and benign bone marrow disorders. It is characterised by abnormal production of red blood cells, white blood cells, and platelets, in association with marrow fibrosis (scarring) and extramedullary haematopoiesis. Mesa RA, Verstovsek S, Cervantes F, et al. Primary myelofibrosis (PMF), post polycythemia vera myelofibrosis (post-PV MF

2018 BMJ Best Practice

3. An Efficacy and Safety Trial of Fedratinib in Subjects With DIPSS, Intermediate or High-Risk Primary Myelofibrosis, Post-Polycythemia Vera Myelofibrosis, or Post-Essential Thrombocythemia Myelofibrosis and Previously Treated With Ruxolitinib

An Efficacy and Safety Trial of Fedratinib in Subjects With DIPSS, Intermediate or High-Risk Primary Myelofibrosis, Post-Polycythemia Vera Myelofibrosis, or Post-Essential Thrombocythemia Myelofibrosis and Previously Treated With Ruxolitinib An Efficacy and Safety Trial of Fedratinib in Subjects With DIPSS, Intermediate or High-Risk Primary Myelofibrosis, Post-Polycythemia Vera Myelofibrosis, or Post-Essential Thrombocythemia Myelofibrosis and Previously Treated With Ruxolitinib - Full Text (...) View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. An Efficacy and Safety Trial of Fedratinib in Subjects With DIPSS, Intermediate or High-Risk Primary Myelofibrosis, Post-Polycythemia Vera Myelofibrosis, or Post-Essential

2018 Clinical Trials

4. Dose-Finding Study of Pacritinib in Patients With Primary Myelofibrosis, Post-Polycythemia Vera Myelofibrosis, or Post-Essential Thrombocythemia Myelofibrosis Previously Treated With Ruxolitinib

Dose-Finding Study of Pacritinib in Patients With Primary Myelofibrosis, Post-Polycythemia Vera Myelofibrosis, or Post-Essential Thrombocythemia Myelofibrosis Previously Treated With Ruxolitinib Dose-Finding Study of Pacritinib in Patients With Primary Myelofibrosis, Post-Polycythemia Vera Myelofibrosis, or Post-Essential Thrombocythemia Myelofibrosis Previously Treated With Ruxolitinib - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer (...) to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Dose-Finding Study of Pacritinib in Patients With Primary Myelofibrosis, Post-Polycythemia Vera Myelofibrosis, or Post-Essential Thrombocythemia Myelofibrosis Previously Treated With Ruxolitinib The safety and scientific validity of this study is the responsibility

2017 Clinical Trials

5. Captopril mitigates splenomegaly and myelofibrosis in the Gata1 low murine model of myelofibrosis (PubMed)

Captopril mitigates splenomegaly and myelofibrosis in the Gata1 low murine model of myelofibrosis Allogeneic stem cell transplantation is currently the only curative therapy for primary myelofibrosis (MF), while the JAK2 inhibitor, ruxolitinib. Has been approved only for palliation. Other therapies are desperately needed to reverse life-threatening MF. However, the cell(s) and cytokine(s) that promote MF remain unclear. Several reports have demonstrated that captopril, an inhibitor

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2018 Journal of cellular and molecular medicine

7. Application of current prognostic models for primary myelofibrosis in the setting of post polycythemia vera or post-essential thrombocythemia myelofibrosis. (PubMed)

Application of current prognostic models for primary myelofibrosis in the setting of post polycythemia vera or post-essential thrombocythemia myelofibrosis. 28819279 2018 06 04 2018 11 13 1476-5551 31 12 2017 12 Leukemia Leukemia Application of current prognostic models for primary myelofibrosis in the setting of post-polycythemia vera or post-essential thrombocythemia myelofibrosis. 2851-2852 10.1038/leu.2017.268 Tefferi A A Division of Hematology, Department of Internal Medicine, Mayo Clinic (...) Medicine, Mayo Clinic, Rochester, MN, USA. eng Journal Article 2017 08 18 England Leukemia 8704895 0887-6924 EC 2.7.10.2 Janus Kinase 2 IM Aged Female Humans Janus Kinase 2 metabolism Male Middle Aged Polycythemia Vera metabolism pathology Primary Myelofibrosis metabolism pathology Prognosis Thrombocythemia, Essential metabolism pathology 2017 8 19 6 0 2018 6 5 6 0 2017 8 19 6 0 ppublish 28819279 leu2017268 10.1038/leu.2017.268 PMC5729329 Leukemia. 2008 Feb;22(2):437-8 17728787 Blood. 2010 Mar 4;115(9

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2017 Leukemia

8. Performance of the myelofibrosis secondary to PV and ET-prognostic model (MYSEC-PM) in a series of 262 patients from the Spanish registry of myelofibrosis. (PubMed)

Performance of the myelofibrosis secondary to PV and ET-prognostic model (MYSEC-PM) in a series of 262 patients from the Spanish registry of myelofibrosis. 28935991 2019 01 02 2019 01 02 1476-5551 32 2 2018 02 Leukemia Leukemia Performance of the myelofibrosis secondary to PV and ET-prognostic model (MYSEC-PM) in a series of 262 patients from the Spanish registry of myelofibrosis. 553-555 10.1038/leu.2017.297 Hernández-Boluda J-C JC Hematology Department, Hospital Clínico, Valencia, Spain (...) Department, Hospital La Fe, Valencia, Spain. Martínez-Valverde C C Hematology Department, Hospital Sant Pau, Barcelona, Spain. Gómez M M Hematology Department, Hospital Clínico, Valencia, Spain. Cervantes F F Hematology Department, Hospital Clínic, Barcelona, Spain. eng Letter Research Support, Non-U.S. Gov't 2017 09 22 England Leukemia 8704895 0887-6924 IM Adult Aged Aged, 80 and over Female Humans Male Middle Aged Polycythemia Vera pathology Primary Myelofibrosis pathology Prognosis Registries

2017 Leukemia

9. The Amelioration of Myelofibrosis with Thrombocytopenia by a JAK1/2 Inhibitor, Ruxolitinib, in a Post-polycythemia Vera Myelofibrosis Patient with a JAK2 Exon 12 Mutation (PubMed)

The Amelioration of Myelofibrosis with Thrombocytopenia by a JAK1/2 Inhibitor, Ruxolitinib, in a Post-polycythemia Vera Myelofibrosis Patient with a JAK2 Exon 12 Mutation Less than 5% of patients with polycythemia vera (PV) show JAK2 exon 12 mutations. Although PV patients with JAK2 exon 12 mutations are known to develop post-PV myelofibrosis (MF) as well as PV with JAK2V617F, the role of JAK inhibitors in post-PV MF patients with JAK2 exon 12 mutations remains unknown. We describe how

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2017 Internal Medicine

10. Myelofibrosis-Induced Erythropoietin-Resistant Anemia Due to Severe Refractory Hyperparathyroidism (PubMed)

Myelofibrosis-Induced Erythropoietin-Resistant Anemia Due to Severe Refractory Hyperparathyroidism 29988982 2019 02 26 2468-0249 3 4 2018 Jul Kidney international reports Kidney Int Rep Myelofibrosis-Induced Erythropoietin-Resistant Anemia Due to Severe Refractory Hyperparathyroidism. 1010-1014 10.1016/j.ekir.2018.04.003 Sethi Sidharth Kumar SK Kidney Institute, Medanta, The Medicity, Gurgaon, Haryana, India. Bansal Shyam Bihari SB Kidney Institute, Medanta, The Medicity, Gurgaon, Haryana

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2018 Kidney international reports

11. The characteristics of vessel lining cells in normal spleens and their role in the pathobiology of myelofibrosis (PubMed)

The characteristics of vessel lining cells in normal spleens and their role in the pathobiology of myelofibrosis The CD34-CD8α+, sinusoid lining, littoral cells (LCs), and CD34+CD8α-, splenic vascular endothelial cells (SVECs) represent 2 distinct cellular types that line the vessels within normal spleens and those of patients with myelofibrosis (MF). To further understand the respective roles of LCs and SVECs, each was purified from normal and MF spleens, cultured, and characterized. Gene

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2018 Blood advances

12. Development of a prognostically relevant cachexia index in primary myelofibrosis using serum albumin and cholesterol levels (PubMed)

Development of a prognostically relevant cachexia index in primary myelofibrosis using serum albumin and cholesterol levels 30097464 2019 03 18 2473-9537 2 15 2018 08 14 Blood advances Blood Adv Development of a prognostically relevant cachexia index in primary myelofibrosis using serum albumin and cholesterol levels. 1980-1984 10.1182/bloodadvances.2018018051 Tefferi Ayalew A Division of Hematology, Department of Internal Medicine. Nicolosi Maura M Division of Hematology, Department

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2018 Blood advances

13. Imetelstat, a telomerase inhibitor, is capable of depleting myelofibrosis stem and progenitor cells (PubMed)

Imetelstat, a telomerase inhibitor, is capable of depleting myelofibrosis stem and progenitor cells Clinical trials of imetelstat therapy have indicated that this telomerase inhibitor might have disease-modifying effects in a subset of patients with myelofibrosis (MF). The mechanism by which imetelstat induces such clinical responses has not been clearly elucidated. Using in vitro hematopoietic progenitor cell (HPC) assays and in vivo hematopoietic stem cell (HSC) assays, we examined

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2018 Blood advances

14. Janus kinase-1 and Janus kinase-2 inhibitors for treating myelofibrosis. (PubMed)

Janus kinase-1 and Janus kinase-2 inhibitors for treating myelofibrosis. Myelofibrosis is a bone marrow disorder characterized by excessive production of reticulin and collagen fiber deposition caused by hematological and non-hematological disorders. The prognosis of myelofibrosis is poor and treatment is mainly palliative. Janus kinase inhibitors are a novel strategy to treat people with myelofibrosis.To determine the clinical benefits and harms of Janus kinase-1 and Janus kinase-2 inhibitors (...) for treating myelofibrosis secondary to hematological or non-hematological conditions.We searched the Cochrane Central Register of Controlled Trials (CENTRAL, the Cochrane Library 2014, Issue 11), Ovid MEDLINE (from 1946 to 13 November 2014), EMBASE (from 1980 to 12 January 2013), and LILACS (from 1982 to 20 November 2014). We searched WHO International Clinical Trials Registry Platform and The metaRegister of Controlled Trials. We also searched for conference proceedings of the American Society

2015 Cochrane

15. MIPSS70: Mutation-Enhanced International Prognostic Score System for Transplantation-Age Patients With Primary Myelofibrosis

MIPSS70: Mutation-Enhanced International Prognostic Score System for Transplantation-Age Patients With Primary Myelofibrosis Purpose To develop a prognostic system for transplantation-age patients with primary myelofibrosis (PMF) that integrates clinical, cytogenetic, and mutation data. Patients and Methods The study included 805 patients with PMF age ≤ 70 years recruited from multiple Italian centers and the Mayo Clinic (Rochester, MN), forming two independent learning and validation cohorts

2018 EvidenceUpdates

16. A rare cause of cytopenia in a patient with systemic lupus erythematosus: Autoimmune myelofibrosis (PubMed)

A rare cause of cytopenia in a patient with systemic lupus erythematosus: Autoimmune myelofibrosis Hematological abnormalities are very common in the course of systemic lupus erythematosus (SLE). Myelofibrosis is a bone marrow disorder in which there is excessive fibrous tissue formation in the bone marrow. Various benign and malignant disorders can cause or be associated with a diffuse increase in the bone marrow reticular tissue. Some diseases such as infections, neoplasms, and autoimmune (...) diseases may also induce bone marrow fibrosis (secondary myelofibrosis). Cytopenia from autoimmune myelofibrosis (AIMF) in SLE is a rare condition. Here we present a case of AIMF associated with SLE and aim to emphasize on the other cause of cytopenia in SLE.

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2017 European journal of rheumatology

17. Therapy-related myelofibrosis does not appear to exist (PubMed)

Therapy-related myelofibrosis does not appear to exist There is no evidence to support the existence of therapy-related myelofibrosis.Therapy for previous malignancy has no impact on myelofibrosis prognosis.

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2017 Blood advances

18. Impact of genomic alterations on outcomes in myelofibrosis patients undergoing JAK1/2 inhibitor therapy (PubMed)

Impact of genomic alterations on outcomes in myelofibrosis patients undergoing JAK1/2 inhibitor therapy In myelofibrosis (MF), driver mutations in JAK2, MPL, or CALR impact survival and progression to blast phase, with the greatest risk conferred by triple-negative status. Subclonal mutations, including mutations in high-molecular risk (HMR) genes, such as ASXL1, EZH2, IDH1/2, and SRSF2 have also been associated with inferior prognosis. However, data evaluating the impact of next-generation

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2017 Blood advances

19. Discrepancies of applying primary myelofibrosis prognostic scores for patients with post polycythemia vera/essential thrombocytosis myelofibrosis (PubMed)

Discrepancies of applying primary myelofibrosis prognostic scores for patients with post polycythemia vera/essential thrombocytosis myelofibrosis 27354022 2018 01 19 2018 11 13 1592-8721 101 10 2016 10 Haematologica Haematologica Discrepancies of applying primary myelofibrosis prognostic scores for patients with post polycythemia vera/essential thrombocytosis myelofibrosis. e405-e406 Gowin Krisstina K Mayo Clinic Arizona, Department of Hematology, Scottsdale, AZ, USA. Coakley Maria M Department (...) diagnosis Primary Myelofibrosis blood diagnosis Prognosis Retrospective Studies Risk Factors Thrombocythemia, Essential blood diagnosis post essential thrombocytosis myelofibrosis post polycythemia vera primary myelofibrosis prognostic scores 2016 6 30 6 0 2018 1 20 6 0 2016 6 30 6 0 ppublish 27354022 haematol.2016.149013 10.3324/haematol.2016.149013 PMC5046661 Mod Pathol. 2013 Dec;26(12):1577-85 23787440 Blood. 1996 Aug 1;88(3):1013-8 8704209 Blood. 2009 Mar 26;113(13):2895-901 18988864 Am J Hematol

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2016 Haematologica

20. Myelofibrosis osteoclasts are clonal and functionally impaired. (PubMed)

Myelofibrosis osteoclasts are clonal and functionally impaired. Bone marrow (BM) sclerosis is commonly found in patients with late stage myelofibrosis (MF). Because osteoclasts (OC) and osteoblasts play a key role in bone remodeling, and MF monocytes, the OC precursors, are derived from the neoplastic clone, we wondered whether decreased OC numbers or impairment in their osteolytic function affects the development of osteosclerosis. Analysis of BM biopsies from 50 MF patients showed increased

2019 Blood

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