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

Hernández‐Boluda, Juan‐Carlos; Pereira, Arturo; Correa, Juan-Gonzalo; Alvarez‐Larrán, Alberto; Ferrer‐Marín, Francisca; Raya, José María; Martínez‐López, Joaquín; Pérez‐Encinas, Manuel; Estrada, Natàlia; Vélez, Patricia; Fox, Laura; García‐Gutiérrez, Valentín; Payer, Ángel Ramírez; Kerguelen, Ana; Cuevas, Beatriz; Durán, Maria Antònia; Ramı́rez, Marı́a José; Gómez‐Casares, María Teresa; Mata-Vázquez, María Isabel; Mora, Elvira; Martínez‐Valverde, Clara; Gómez, Montse; Cervantes, Francisco

doi:10.1038/leu.2017.297

Cited by 24 publications

(17 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Altogether, these considerations reflect the uncertainty about prognostic scores alone in defining transplant indication. In this regard, it should be underlined that the predictive efficacy of MYSEC-PM score in ruxolitinibtreated patients has been validated in a retrospective study [24] and that two recent reports from the EBMT and the Spanish registry confirmed the efficacy of MYSEC-PM, as well as its superiority over DIPSS or IPSS scores, in predicting posttransplant survival [27,28]. Of note, about 60% of patients in both these series belonged to the low or intermediate-1 MYSEC-PM risk group, and downgrading from higher IPSS categories was mainly related to the large effect of age in the MYSEC-PM model: in fact, only a minority of patients in a transplant age (i.e., <70 years) are categorized in intermediate-2 or high MYSEC-PM risk.…”

Section: Pretransplant Evaluation: Prognostic Scores and Consideratiomentioning

confidence: 96%

“…Of note, about 60% of patients in both these series belonged to the low or intermediate-1 MYSEC-PM risk group, and downgrading from higher IPSS categories was mainly related to the large effect of age in the MYSEC-PM model: in fact, only a minority of patients in a transplant age (i.e., <70 years) are categorized in intermediate-2 or high MYSEC-PM risk. This large contribution of age to the scoring system may thus decrease its sensitivity to the other diseaserelated adverse risk factors (e.g., circulating blasts), which could be important for transplant decisions [28].…”

Section: Pretransplant Evaluation: Prognostic Scores and Consideratiomentioning

confidence: 99%

See 1 more Smart Citation

The role of allogeneic stem-cell transplant in myelofibrosis in the era of JAK inhibitors: a case-based review

Tiribelli

Palandri

Sant’Antonio

et al. 2019

Bone Marrow Transplant

View full text Add to dashboard Cite

Allogeneic hematopoietic stem-cell transplantation (HSCT) is, at present, the only potentially curative therapy for myelofibrosis (MF). Despite many improvements, outcomes of HSCT are still burdened by substantial morbidity and high transplant-related mortality. Allogeneic transplant is generally considered in intermediate-2 and high-risk patients aged <70 years, but the optimal selection of patients and timing of the procedure remains under debate, as does as the role of JAK inhibitors in candidates for HSCT. Starting from a real-life clinical case scenario, herein we examine some of the crucial issues of HSCT for MF in light of recent refinements on MF risk stratification, data on the use of ruxolitinib before and after transplant and findings on the impact of different conditioning regimens and donor selection.

show abstract

Section: Pretransplant Evaluation: Prognostic Scores and Consideratiomentioning

confidence: 96%

Section: Pretransplant Evaluation: Prognostic Scores and Consideratiomentioning

confidence: 99%

The role of allogeneic stem-cell transplant in myelofibrosis in the era of JAK inhibitors: a case-based review

Tiribelli

Palandri

Sant’Antonio

et al. 2019

Bone Marrow Transplant

View full text Add to dashboard Cite

show abstract

“…4 For patients with post-PV/ET MF, the MF secondary to PV, and ET prognostic model (MYSEC-PM) has been defined and validated for risk stratification. 5–7 This new score assigns 2 points to hemoglobin level <11 g/dl, circulating blasts ⩾3% and CALR-unmutated genotype, 1 point to platelet count <150 × 10 9 /l and constitutional symptoms, and 0.15 points to any year of age. Patients are allocated into four risk categories with different survival: low (median survival not reached), intermediate-1 (9.3 years), intermediate-2 (4.4 years), and high (2 years) risk.…”

Section: Prognosis and Risk Stratificationmentioning

confidence: 99%

Allogeneic hematopoietic stem-cell transplantation for myelofibrosis

Zhang

Yang

Feng

2020

Therapeutic Advances in Hematology

View full text Add to dashboard Cite

Myelofibrosis is one of the Philadelphia chromosome (Ph)-negative myeloproliferative neoplasms with heterogeneous clinical course. Though many treatment options, including Janus kinase (JAK) inhibitors, have provided clinical benefits and improved survival, allogeneic hematopoietic stem-cell transplantation (AHSCT) remains the only potentially curative therapy. Considering the significant transplant-related morbidity and mortality, it is crucial to decide who to proceed to AHSCT, and when. In this review, we discuss recent updates in patient selection, prior splenectomy, conditioning regimen, donor type, molecular mutation, and other factors affecting AHSCT outcomes. Relapse is a major cause of treatment failure; we also describe recent data on minimal residual disease monitoring and management of relapse. In addition, emerging studies have reported pretransplant therapy with ruxolitinib for myelofibrosis showing favorable results, and further research is needed to explore its use in the post-transplant setting.

show abstract

“…points allocated for all the other risk factors on a nomogram to quickly find the prognostic category for a given patient. The MYSEC-PM has been independently validated, but the heavy reliance on age could be a shortcoming of this model [68].…”

Section: Mysec-pmmentioning

confidence: 99%

Mutational profiling in myelofibrosis: implications for management

Bose

Verstovšek

2019

Int J Hematol

View full text Add to dashboard Cite

Mutational profiling, usually by targeted next-generation sequencing, is increasingly performed on patients with myeloproliferative neoplasm-associated myelofibrosis (MF), whether primary (PMF) or post-polycythemia vera/essential thrombocythemia (post-PV/ET MF). "Driver" mutations in JAK2, MPL and indels in CALR underlie the vast majority of cases of PMF and post-ET MF; the remainder (≈ 10%) lack identifiable driver mutations, but other clonal markers are usually detectable. Nearly all patients with post-PV MF carry activating JAK2 mutations. In both PMF and post-ET MF, type 1/-like CALR mutations confer a favorable prognosis. Since both type 1/-like and type 2/-like CALR mutations have essentially the same functional consequence, this is a subject of intense research. Additional, "non-driver" mutations, mostly affecting genes encoding epigenetic modifiers or spliceosome components, e.g., ASXL1, EZH2, TET2, DNMT3A, SRSF2 and U2AF1, are frequently found; some of these are associated with inferior survival and have been incorporated into prognostic models. Some mutations, e.g., IDH1/2, are relatively infrequent in chronic phase but are substantially more common in blast phase, and are now therapeutically targetable. While mutational information does not currently influence choice of drug therapy in chronic-phase MF, the presence of a "high molecular risk" genotype is now routinely taken into account for transplant decision-making.

show abstract

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

Cited by 24 publications

References 11 publications

The role of allogeneic stem-cell transplant in myelofibrosis in the era of JAK inhibitors: a case-based review

The role of allogeneic stem-cell transplant in myelofibrosis in the era of JAK inhibitors: a case-based review

Allogeneic hematopoietic stem-cell transplantation for myelofibrosis

Mutational profiling in myelofibrosis: implications for management

Contact Info

Product

Resources

About