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Validation of the Myelofibrosis Secondary to PV and ET – Prognostic Model in Patients with Post-PV and Post-Essential Thrombocythemia
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Lucia Masarova, M.D. presents from ASH 2017 on the characteristics of patients with elevated blasts in chronic myelofibrosis and the effect of ruxolitinib.

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Hello. I am Dr. Lucia Masarova from MD Anderson Cancer Center in Houston Texas. And I will discuss our work presented as poster here in Atlanta at the American Society of Hematology. Validating the myelofibrosis secondary to ET and PV prognostic model or later referred as MYSEC-PM. In patients with post polycythemia vera and post essential thrombocythemia myelofibrosis, and the group from MD Cancer Center.

Patients with post ET and post PV myelofibrosis have spent different outcome in patients with primary myelofibrosis. Recently, a novel model called MYSEC-PM, done by Francesco Passamonti, was reported redefining outcome of these patients, and was proven to be better than currently existing IPSS, and DIPSS models, which were primary developed for patients with primary myelofibrosis. Therefore, we aimed to validate this model on our group of patients from MD Anderson Cancer Center, including 178 newly diagnosed patients with post ET and post PVMF, as done in the original report, as well as on all referred patients which included patients presented later during the disease, with the median follow up, or time from the presentation, of 18 months.

In reference, we summarized that we retrospectively reviewed medical charts of all newly diagnosed and later referred patients between the past three years referred to MD Anderson Cancer Center. We have applied the MYSEC-PM model as published, and compare the prognostic utility to IPSS and DIPSS risk model. The results are showing a clinical characteristics and demographics of all patients shown in Table 1. There were no differences in clinical parameters between patients who were newly diagnosed or later referred during the disease.

Most of the patients presented in intermediate risk 1 and 2 IPSS, as well as DIPSS models. First we evaluated the prognostic utility of MYSEC-PM on newly diagnosed patients as shown in the Graph 1 from the top to the bottom, and overall survivor results summarized in Table 2 and Table 3.

IPSS model, shown in the Graph 1, in the middle, did not discriminate different overall survival between patients in the low and intermediate-1 risk, as well as between those in high and intermediate-2. Risk stratification according to MYSEC-PM. However, that does distinguish these patients, and we've seen four different risk categories with different overall survival between low, intermediate 1, 2, and high risk patients as summarized in Table 2.

Similarly, when patients with original intermediate 1 and 2 risks according to IPSS were reclassified based on MYSEC-PM model. These patients again fall into four distinct categories with overall survival, shown in Table 3 and Graph 1 at the bottom.

These results validated the MYSEC-PM model of newly diagnosed patients in our group. Later we evaluated the same model on all referred patients during the course of the disease, as shown in the Graph 2, from the top to the bottom, and again results with median survival summarized in Table 2 and Table 3.
The IPSS model, Graph 2 in the middle, did not show distinct overall survival between patients with intermediate 1, intermediate 2, and high risk DIPSS scores. Only patients with low risk had distinct and improved survival.

Stratification occurred into MYSEC-PM. These show better stratification of patients between intermediate-2 and high risk. However, fail to distinguish different survival between patients with intermediate-1 and intermediate-2 MYSEC-PM.

Similar findings were found when patients with intermediate DIPSS were re-stratified according to MYSEC-PM, and again, patients with intermediate-1 and intermediate-2 MYSEC-PM, had similar overall survival. These results with exact times of survival are shown in Table 2 and Table 3.

In conclusion, our data validated MYSEC-PM prognostics utility on overall survival in patients with newly diagnosed post ET and post PV myelofibrosis, and the results were comparable to those in the original report. However, the model failed to distinguish different overall survival between patients in intermediate-1 and intermediate-2 DIPSS risk score during the course of the disease. Where other factors not accounted for in the model may play a significant role.

Thank you for your attention and watching my presentation.