Caution:The relative risk assessment of MPN phenotypes as well as degree of myelofibrosis risk based on the following DIPSS scales is data based but survival estimates are highly variable based on individual genetics, clinical condition and the impact of new treatment protocols. Sharing results and questions with your hematologist is essential to develop a full picture of your status, prognosis, and treatment options
The MAGIC toolbox, including the MPN Assessment Graphic Internet Calculator, is a convenient and graphic means to summarize broad clinical experience in assessing overall risk-associated score for myelofibrosis, polycythemia vera and essential thrombocythemia.
MAGIC is an interactive MPN risk assessment tool, The actual determination of MPN risk of progression or assessment of the lethality of an MPN state is determined by multiple means such as blood tests, physical examination, bone marrow biopsy, genomic testing, pathology reports, etc.
What MAGIC offers is an overview of some major peer-reviewed tests constructed and validated by MPN specialists. These test results have been found to statistically associate with MPN disease status. Taken into consideration with other clinical and genetic results – as well as patient reported symptoms – these tests help form the basis for diagnosis, anticipated prognosis and a treatment plan
Much about myeloproliferative neoplasm remains mysterious. Individual variations in response to the onset of disease and to treatment are great.
What MAGIC can provide is a snapshot of a patient’s current status based on available testing data.
For Myelofibrosis
MAGIC relies on two commonly used and validated scales developed by the International Working Group on Myeloproliferative Neoplasms Research and Treatment (IWG-MRT). Together, the DIPSS (Dynamic International Prognostic Scoring System)* and the DIPSS+ assess the current degree of myelofibrosis risk.*
There are four MF Risk Levels (Low, Intermediate-1, Intermediate-2, and High). Each Risk Level carries a mean approximation of overall survival (OS) expressed in years and months. The OS is only a guideline. According to the mean OS calculation, as many patients with that specific risk profile will live longer than predicted as those who die earlier.
Myelofibrosis is a complex disease whose etiology and progression is only partially understood. Beyond the clinical elements measured by our scales, some factors influencing survival include genetics, environment, cytogenetics, co-morbidities, diet, lifestyle, epigenetics, geography, occupation, and, emotional, social and psychological elements.
The MAGIC results are significant, however, in revealing a patient’s underlying general risk status and can serve as a guide to undertake minimal or more aggressive therapies.
The Symptom Scales for Polycythemia Vera and Essential Thrombocythemia and MF.
MAGIC includes reference to two clinically validated patient reported symptom scales, the MF-SAF and the MPN-SAF TSS, the myelofibrosis symptom assessement form and the MPN symptom assessment form — Total System Score.
The myelofibrosis brief inventory symptom scale (Mesa et al., 2009, Leukemia Research) is “an evidence-based brief inventory to measure quality of life and symptomatic response to treatment in myelofibrosis.”
h
This upgrade of MPN Risk Assessment incorporates a mutational status component.
The Mutational Component
MAGIC provides the option for patients to select their known current MPN mutations. Calculation of the weight of these mutational selections is not incorporated within MAGIC which currently relies only on the clinically validated DIPSS scales to determine Risk Levels and Overall Survival averages. The mutational landscape however provides vital prognostic information that may be applicable to selection of therapeutic options.
A brief description of the most common MPN mutations( as 9f Summer, 2022) and the role these mutations play is included in the MAGIC Toolbox Lnks to on-line calculators that incorporate clinical, cytogenetic and mutational data are also provided.
Much has been learned about the role mutations play in the creation and driving of myeloproliferative neoplasms. Two recent studies – Vainchenker, Kralovics et al. and Grinfeld, Nangalia, et al. – were a primary basis for selecting commonly occurring driving and passenger mutations. And while there are both deadly and relatively benign mutations there remains some question as to the specific impact of an individual mutation. However, the presence of multiple mutations arising in an unstable genetic environment that includes MPN driving mutations generally predict unfavorable outcomes.
. Details and a full listing of all these mutations notes can be found in Notes on Individual Mutations, an integral resource for MAGIC .
Additional Risk Calculators
Additional risk calculators in MAGIC –MIPSS70, IPSET . MYSEC and the MPN Personal Risk Calculator can be round here
NCCN Graphic courtesy of Aaron Gerds, MD, Cleveland Clinic
(Table appeared in Cruz et al. “Myelofbrosis: Best Practices…” Expert Rev Hem 2019)
The real value of the MAGIC calculator lies in sharing its results with the patient’s hematologist as an aid to developing an optimal treatment plan. If genomic testing has not yet been done and mutational load is unknown MAGIC will still yield significant Risk Level results.
