Passamonti et al reported the Dynamic International Prognostic Scoring System (DIPSS) for predicting survival for a patient with primary myelofibrosis. This can help to identify patients who may benefit from more aggressive management. The work was based on a study by the International Working Group for Myelofibrosis Research and Treatment (IWGMRT).
NOTE: Cervantes et al reported an earlier version of the score, with the only differences being a different weighting for hemoglobin and different risk group cutoffs. The previous version was used in newly-diagnosed patients, while the DIPSS can be used anytime during the patient's clinical course.
Parameters at diagnosis:
(1) age in years
(2) constitutional symptoms (unexplained fever, excessive sweating for > 1 month, and/or weight loss > 10% = B symptoms for malignant lymphoma)
(3) hemoglobin in g/dL
(4) white blood cell count
(5) percent blasts in the peripheral blood
|
Parameter |
Finding |
Points |
|---|---|---|
|
age in years |
<= 65 years of age |
0 |
|
|
> 65 years of age |
1 |
|
constitutional symptoms |
absent |
0 |
|
|
present |
1 |
|
hemoglobin |
>= 10 g/dL |
0 |
|
|
< 10 g/dL |
2 |
|
white blood cell count |
<= 25,000 per µL |
0 |
|
|
> 25,000 per µL |
1 |
|
percent blasts |
<= 1% |
0 |
|
|
> 1% |
1 |
Because allogeneic stem cell transplant (SCT) is limited to patients < 65 years of age, the authors also developed an "age-adjusted" DIPSS for patients younger than 65 years of age.
|
Parameter |
Finding |
Points |
|---|---|---|
|
constitutional symptoms |
absent |
0 |
|
|
present |
2 |
|
hemoglobin |
>= 10 g/dL |
0 |
|
|
< 10 g/dL |
2 |
|
white blood cell count |
<= 25,000 per µL |
0 |
|
|
> 25,000 per µL |
1 |
|
percent blasts |
<= 1% |
0 |
|
|
> 1% |
2 |
total DIPSS score =
= SUM(points for all 5 parameters)
total age-adjusted DIPSS score =
= SUM(points for all 4 parameters)
Interpretation:
• minimum score: 0
• maximum DIPSS score: 6
• maximum age-adjusted DIPSS score: 7
• The higher the score the worse the patient's prognosis.
|
DIPSS |
Risk Group |
10 Year Survival |
20 Year Survival |
|---|---|---|---|
|
0 |
low |
91% |
84% |
|
1 or 2 |
intermediate-1 |
61% |
47% |
|
3 or 4 |
intermediate-2 |
11% |
2% |
|
5 or 6 |
high |
1% |
0% |
|
Age-adjusted DIPSS |
Risk Group |
10 Year Survival |
20 Year Survival |
|---|---|---|---|
|
0 |
low |
88% |
77% |
|
1 or 2 |
intermediate-1 |
46% |
34% |
|
3 or 4 |
intermediate-2 |
33% |
0% |
|
5, 6 or 7 |
high |
3% |
0% |
Purpose: To evaluate a patient with primary myelofibrosis at any time during its course using the prognostic score of Passamonti et al.
Specialty: Hematology Oncology
Objective: severity, prognosis, stage
ICD-10: D47.4,
