Primary Myelofibrosis (MF)

Your patients may have higher risk disease than you realize

MF, similar to other malignancies, is a serious disease that may require active management at diagnosis.¹

^aFive-year overall survival rates were estimated using SEER data obtained from population-based cancer registries of the US population and SEER^*Stat Software version 8.3.2. The analysis included patients with initial/primary site diagnosis between years 2007-2011. Overall survival is defined as the time from diagnosis until death from any cause.

In MF, the presence of any of the following risk factors* indicates that a patient is already intermediate risk^3,4:

Hemoglobin level <10 g/dL
Leukocyte count >25 × 10⁹/L
Age >65 years
Red cell transfusion dependency

Circulating blast cells ≥1%
Platelet count <100 × 10⁹/L
Constitutional symptoms
Unfavorable karyotype

*As included in the Dynamic International Prognostic Scoring System Plus tool.

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Why assess risk
level in patients
with MF?

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In a study of patients with primary MF, approximately 90% (375/428) of evaluable patients were considered to be intermediate or high risk within 1 year of diagnosis⁴

Image of graphic that reads – 90% In a study of patients with primary MF, approximately 90% (375/428) of evaluable patients were considered to be intermediate or high risk within 1 year of diagnosis

In a study of patients with primary MF, approximately 90% (375/428) of evaluable patients were considered to be intermediate or high risk within 1 year of diagnosis⁴

Consider one of your patients with myelofibrosis

Which risk factors^‡ does your patient have?

As the clinical understanding of myelofibrosis has evolved, a variety of prognostic systems have been developed.³

All of the systems utilize a group of risk factors to assign risk level (low, intermediate, or high) which may be useful in counseling patients with MF and deciding on therapeutic approach.^3-5

One of these systems, DIPSS Plus, is based on 8 risk factors, including karyotype, transfusion dependency, and platelet count less than 100 × 10⁹/L.⁴

Select as many risk factors as apply for your patient. When done, click "Submit." To consider another patient, click "Reset."

Hemoglobin level <10 g/dL

Circulating blast cells ≥1%

Leukocyte count >25 × 10⁹/L

Platelet count <100 × 10⁹/L

Age >65 years

Constitutional symptoms

Red cell transfusion dependency

Unfavorable karyotype

^‡As included in the Dynamic International Prognostic Scoring System Plus tool.

SUBMIT RESET Scroll down to see results.

FOR EDUCATIONAL PURPOSES ONLY. This does not take the place of a physician’s clinical judgment. Results provide a suggested approach to risk assessment and are not a substitute for a series of clinical decisions over time for each individual patient.

Dynamic International Prognostic Scoring System Plus⁴

Risk Factors

Risk Stratification

Median Life Expectancy

Low Risk

15.4 years

Intermediate-1

6.5 years

2-3

Intermediate-2

2.9 years

4 or more

High Risk

1.3 years

Adapted from Gangat, J Clin Oncol. 2011; 29:392-397.⁴

Actively manage your patients by intervening at diagnosis.

…of patients with primary MF may be appropriate for treatment with Jakafi^†

^†Based on a study of patients with primary MF, approximately 90% (375/428) of evaluable patients were considered to be intermediate or high risk within 1 year of diagnosis.⁴

New or increasing splenomegaly is considered to be a marker of disease progression in myelofibrosis.⁶

IWG-MRT and ELN response criteria state that a palpable spleen extending ≥5 cm below the left costal margin constitutes progressive disease^6§

Splenomegaly can cause/exacerbate cytopenias due to splenic sequestration⁷
Splenomegaly may cause pain, early satiety, abdominal discomfort, and other symptoms^7,8
In a pooled overall survival analysis of 528 patients from the COMFORT^||¶ trials, larger baseline spleen volume was associated with incremental increases in the risk of death over 3 years, irrespective of treatment^9#

In a study of 1,054 patients with primary myelofibrosis, approximately 90% of patients for whom data were available had palpable splenomegaly at diagnosis^3**

Image of graphic that reads – 90% … In a study of 1,054 patients with primary myelofibrosis, approximately 90% of patients for whom data were available had a palpable splenomegaly at diagnosis

In a study of 1,054 patients with primary myelofibrosis, approximately 90% of patients for whom data were available had palpable splenomegaly at diagnosis^3**

^§Progressive disease assignment for splenomegaly requires confirmation by CT or MRI showing a ≥25% increase in spleen volume from baseline. Baseline values for both physical examination and imaging studies refer to pretreatment baseline and not to posttreatment measurements.⁶

^||COMFORT-I (COntrolled MyeloFibrosis study with ORal JAK inhibitor Treatment-I) was a randomized, double-blind, placebo-controlled phase 3 study with 309 patients with intermediate-2–risk or high-risk myelofibrosis. The primary end point was the proportion of subjects achieving a ≥35% reduction in spleen volume from baseline to week 24 as measured by CT or MRI.^10,11

^¶COMFORT-II (COntrolled MyeloFibrosis study with ORal JAK inhibitor Treatment-II) was a randomized, open-label phase 3 study with 219 patients with intermediate-2–risk or high-risk myelofibrosis. The primary end point was the proportion of patients achieving a ≥35% reduction in spleen volume from baseline at week 48 as measured by CT or MRI.^10,12

^#14% increase in risk of death for each additional 5 dL of spleen volume at baseline (hazard ratio, 1.14; 95% confidence interval, 1.07-1.21).⁹

^**Data were available for 768 patients, 681 of whom had palpable spleen.³

Video

Risk Assessment in Patients With Myelofibrosis (MF)

In this video, Salman Fazal, MD, discusses two commonly employed scoring systems to help estimate prognosis in patients with myelofibrosis (MF)—the International Prognostic Scoring System (IPSS) and Dynamic International Prognostic Scoring System (DIPSS). When evaluated by these risk assessment tools, the majority of patients with MF are found to have either intermediate or high-risk MF. Using IPSS and DIPSS, healthcare providers can accurately determine the risk category of their patients with MF and engage in meaningful discussions about prognosis and treatment options.

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Image of 3 people – Identify Appropriate Patients for Jakafi

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References

Barbui T, Barosi G, Birgegard G, et al. Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European LeukemiaNet. J Clin Oncol. 2011;29(6):761-770.
Surveillance, Epidemiology, and End Results (SEER) Program (www.seer.cancer.gov) SEER^*Stat Database: Incidence - SEER 18 Regs Research Data + Hurricane Katrina Impacted Louisiana Cases, November 2015 Submission (1973-2013 varying) - Linked To County Attributes - Total U.S., 1969-2014 Counties. Accessed January 4, 2017.
Cervantes F, Dupriez B, Pereira A, et al. New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment. Blood. 2009;113(13):2895-2901.
Gangat N, Caramazza D, Vaidya R, et al. DIPSS plus: a refined Dynamic International Prognostic Scoring System for primary myelofibrosis that incorporates prognostic information from karyotype, platelet count, and transfusion status. J Clin Oncol. 2011;29(4):392-397.
Passamonti F, Cervantes F, Vannucchi AM, et al. A dynamic prognostic model to predict survival in primary myelofibrosis: a study by the IWG-MRT (International Working Group for Myeloproliferative Neoplasms Research and Treatment). Blood. 2010;115(9):1703-1708.
Tefferi A, Cervantes F, Mesa R, et al. Revised response criteria for myelofibrosis: International Working Group-Myeloproliferative Neoplasms Research and Treatment (IWG-MRT) and European LeukemiaNet (ELN) consensus report. Blood. 2013;122(8):1395-1398.
Mesa RA. How I treat symptomatic splenomegaly in patients with myelofibrosis. Blood. 2009;113(22):5394-5400.
Scherber RM, Geyer HL, Mesa RA. Quality of life in MPN comes of age as a therapeutic target. Curr Hematol Malig Rep. 2014;9(4):324-330.
Vannucchi AM, Kantarjian HM, Kiladjian JJ, et al; on behalf of the COMFORT Investigators. A pooled analysis of overall survival in COMFORT-I and COMFORT-II, 2 randomized phase III trials of ruxolitinib for the treatment of myelofibrosis. Haematologica. 2015;100(9):1139-1145.
Jakafi Prescribing Information. Wilmington, DE: Incyte Corporation.
Verstovsek S, Mesa RA, Gotlib J, et al. A double-blind, placebo-controlled trial of ruxolitinib for myelofibrosis. N Engl J Med. 2012;366(9):799-807.
Harrison C, Kiladjian J-J, Al-Ali HK, et al. JAK inhibition with ruxolitinib versus best available therapy for myelofibrosis. N Engl J Med. 2012;366(9):787-798.

Intervene at Diagnosis

Your patients may have higher risk disease than you realize

MF, similar to other malignancies, is a serious disease that may require active management at diagnosis.1

In MF, the presence of any of the following risk factors* indicates that a patient is already intermediate risk3,4:

Which risk factors‡ does your patient have?

Dynamic International Prognostic Scoring System Plus4

Actively manage your patients by intervening at diagnosis.

New or increasing splenomegaly is considered to be a marker of disease progression in myelofibrosis.6

Video

Intervene at
Diagnosis

MF, similar to other malignancies, is a serious disease that may require active management at diagnosis.¹

In MF, the presence of any of the following risk factors* indicates that a patient is already intermediate risk^3,4:

Which risk factors^‡ does your patient have?

Dynamic International Prognostic Scoring System Plus⁴

New or increasing splenomegaly is considered to be a marker of disease progression in myelofibrosis.⁶