TYPES OF MDS
Myelodysplastic syndromes (MDS) involve ineffective blood cell production.4
All blood cells — red blood cells, white blood cells and platelets — are produced
in the bone marrow. With MDS, fewer "good" blood cells circulate in the bloodstream,
which can lead to abnormally low amounts of any of these blood cells.
There are two main classifications healthcare professionals use to determine the
type of MDS a person has: The French-American-British (FAB) classification system
and The World Health Organization (WHO) classification system.7,8
FAB was developed at an international conference with doctors from France, the United
States and Britain. The classification allows doctors to determine a patient’s prognosis
with myelodysplastic syndromes. The World Health Organization (WHO) developed its
tool to better classify patients with MDS.
The FAB classification uses the following categories:
|
FAB Classification7 |
|
Type of MDS |
Percent of blasts in marrow (less
than 5% is normal) |
|
Refractory Anemia (RA) |
Less than 5% blasts in bone marrow (normal count) |
|
Refractory Anemia with Ringed Sideroblasts
(RARS) |
Less than 5% blasts in bone marrow (normal amount), plus more than 15% of
abnormal red blood cells called ringed sideroblasts |
|
Refractory Anemia with Excess Blasts (RAEB) |
5 to 20% blasts in bone marrow |
|
Refractory Anemia with Excess Blasts in Transformation (RAEB-t) |
21-30% blasts in bone marrow |
|
Chronic Myelomonocytic Leukemia (CMML) |
5% to 20% blasts in bone marrow, plus a large number of a type of white
blood cell called monocytes |
The WHO classification uses the following categories:
|
WHO Classification8 |
|
Types of MDS |
Percent of blasts in bone marrow (<5% is considered normal) |
|
Refractory Anemia (RA) |
Less than 5% blasts in bone marrow |
|
Refractory Anemia with Ringed Sideroblasts (RARS) |
Less than 5% blasts in bone marrow
Greater than or equal to 15% ringed sideroblasts |
|
Refractory Cytopenia with Multilineage Dysplasia (RCMD) |
Less than 5% blasts in bone marrow
Less than 15% ringed sideroblasts
2 or more abnormal blood cells lines in bone marrow |
|
Refractory Cytopenia with Multilineage Dysplasia and Ringed Sideroblasts (RCMD-RS) |
Less than 5% blasts in bone marrow
Greater than or equal to 15% ringed sideroblasts
2 or more abnormal blood cells lines in bone marrow |
|
Refractory Anemia with Excess Blasts-1 (RAEB-1) |
5-9% blasts in bone marrow
0-4% blasts in blood |
|
Refractory Anemia with Excess Blasts-2 (RAEB-2) |
10-19% blasts in bone marrow
5-19% blasts in blood |
|
Myelodysplastic Syndrome, unclassified (MDS-U) |
Less than 5% blasts in bone marrow
Abnormal white cells or platelets |
|
Myelodysplastic Syndromes Associated with Isolated del(5q) |
Less than 5% blasts in bone marrow
0-4% blasts in blood
Chromosomes with partial loss of chromosome #5 |
A third MDS-related scoring system, the International Prognostic Scoring System
(IPSS) is used to describe the risk that a patient’s disease will develop into Acute
Myeloid Leukemia. The IPSS score is based on three factors:7
|
|
Percent of blasts in the bone marrow |
|
|
If the patient has 1 or more cytopenias – low red, white and/or platelet blood cell
counts
|
|
|
Changes in bone marrow blood cell chromosomes (cytogenetics) |
|
International Prognostic Scoring System for MDS9 |
|
|
Score Value |
|
Prognostic Variable |
0.0 |
0.5 |
1.0 |
1.5 |
2.0 |
Bone Marrow Blasts (%)
(immature blood cells) |
<5 |
5-10 |
— |
11-20 |
21-30 |
Cytogenetics*
(profile of chromosomal abnormalities) |
Good |
Intermediate |
Poor |
|
|
Cytopenias
(low red, white and/or platelets cell count)
|
0/1 |
2/3 |
|
|
|
*Good = normal, -Y alone, del (5q) alone, del (20q) alone; Poor = complex
(≥ 3 abnormalities) or chromosome 7 anomalies; Intermediate = other abnormalities.
|
Prognostic Score and IPSS Subgroup |
|
Prognostic Score |
IPSS Subgroup |
|
0 |
Low |
|
0.5-1.0 |
Intermediate-1 |
|
1.5-2.0 |
Intermediate-2 |
|
>2.5 |
High |
As the MDS disease progresses from low to high risk, there is a greater likelihood
for patients’ disease to develop into AML or death.
blasts = immature blood cell
sideroblasts =ring shaped iron deposit on red blood cell
To learn more about the steps to understand a FAB, WHO or IPSS diagnosis,
Click here.
References