Myelodysplastic syndrome, or MDS, is a group of diseases in which the bone marrow doesn’t produce enough healthy blood cells. Red blood cells, white blood cells and platelets originate in the marrow and move into the bloodstream when they mature. In MDS, the marrow produces too many immature cells (blasts).
These blasts die in the marrow or soon after entering the bloodstream, resulting in too few healthy blood cells and low patient blood counts.
In its mildest form, myelodysplastic syndrome may be only anemia, low platelets or low white blood count, but higher-risk types can progress to acute myeloid leukemia (AML). Each year in Spain 3.000 new cases of MDS are diagnosed.
Ninety percent of myelodysplastic syndrome patients are over the age of 60, and 10-20% of diagnosed cases will progress to AML.
Types of Myelodysplastic Syndrome
There are many different types of myelodysplastic syndrome. Over the last 30 years, at least three classification systems have been created in an effort to categorize the various disease types. MD Anderson uses the International Prognostic Scoring System (IPSS), a numerical score to determine a patient's risk for myelodysplastic syndrome:
Risk Level
|
IPSS Score
|
Low risk
|
0
|
Intermediate risk 1
|
0,5 – 1
|
Intermediate risk 2
|
1,5 - 2
|
High risk
|
≥2,5
|
The following factors are used to calculate the IPSS score:
|
0,0
|
0,5
|
1,0
|
1,5
|
2,0
|
% Marrow Blasts
|
<5
|
5-10
|
|
11-20
|
21-30
|
Cytogenetics
|
Normal, -Y del(5)q sola del(20)q sola
|
Otros
|
-7, del(7)q ≥ 3 anormal
|
|
|
Cytopenias:
- Hemoglobin (HGB) < 10 g/dl
- Absolute Neutrophils (ANC) <1.500/ul
- Platelets (PLT) <100K/ul
|
0/1
|
2/3
|
|
|
|
The IPSS score is also used by oncologists to predict age-related survival rates and progression to acute myelogenous leukemia in MDS patients.
|
No. of Patients
|
Low
|
Int-1
|
Int-2
|
High
|
A. Median Survival (yr)
|
Total Patients (%)
|
816
|
267 (33)
|
314 (88)
|
176 (22)
|
59 (7)
|
Median Survival (yr)
|
|
5,7
|
3,5
|
1,2
|
0,4
|
Age (yr)
|
|
|
|
|
|
<60
|
205 (25)
|
11,8
|
5,2
|
1,8
|
0,3
|
>60
|
611
|
4,8
|
2,7
|
1,1
|
0,5
|
<70
|
445 (54)
|
9,0
|
4,4
|
1,3
|
0,4
|
>70
|
271
|
3,9
|
2,4
|
1,2
|
0,4
|
B. 25% AML Evolution (yr)
|
Total Patients (%)
|
759
|
235 (51)
|
295 (39)
|
171 (22)
|
58 (8)
|
Median Survival (yr)
|
|
9,4
|
3,3
|
1,1
|
0,2
|
Age (yr)
|
|
|
|
|
|
<60
|
187 (25)
|
> 9,4 (NA)
|
6,9
|
0,7
|
0,2
|
>60
|
572
|
9,4
|
2,7
|
1,3
|
0,2
|
<70
|
414 (55)
|
> 9,4 (NA)
|
5,5
|
1,0
|
0,2
|
>70
|
345
|
> 5,8 (NA)
|
2,2
|
1,4
|
0,4
|
NR = not reached
While recognizing the usefulness of the IPSS, we developed a new prognostic model specifically for the Low and Int-1 subset of MDS patients, below. This is based on an analysis published in Leukemia, 2008; 22: 538-543.
Adverse Factor
|
Assigned Score
|
Unfavorable cytogenetics
|
1
|
Age > 60 years
|
2
|
Hgb <10 g/dl
|
1
|
Plt <50 K/ul
|
2
|
Plt 50–200 K/ul
|
1
|
BM blasts >4%
|
1
|
In this analysis, diploid and 5q only were favorable cytogenetic, all others were considered as unfavorable cytogenetics.
|
Abbreviations: BM, bone marrow; Hgb, hemoglobin; Plt, platelets.
|
Estimated survival outcomes within each score range and proposed risk categories
Score
|
No. of patients
|
Median (month)
|
Four-year survival (%)
|
Category
|
0
|
11
|
NA
|
78
|
1
|
1
|
58
|
83
|
82
|
1
|
2
|
113
|
51
|
51
|
1
|
3
|
185
|
36
|
40
|
2
|
4
|
223
|
22
|
27
|
2
|
5
|
166
|
14
|
9
|
3
|
6
|
86
|
16
|
7
|
3
|
7
|
13
|
9
|
NE
|
3
|
Abbreviations: NA, not assessable; NR, not reached.
|
There are multiple symptoms of myelodysplastic syndrome, many of them non-specific. Signs may include:
- Easy bruising
- Fatigue
- Weight loss
- Petechiae (tiny red spots just under the skin)
- Fever
- Frequent infections
- Weakness
- Shortness of breath
Having one or more of the symptoms listed above does not necessarily mean you have myelodysplastic syndrome However, it is important to discuss any symptoms with your doctor, since they may indicate other health problems.