Myelodysplastic syndrome

The word dysplasia is premalignant condition. Any dysplasia is premalignant.It is premalignant so malignancy of myeloid origin may occur. That is it may turn into leukaemia whether acute or chronic.

Here bone marrow dysplasia occur so cytopenia occurs. So we can call MDS as a group of disorder where .

the word.

Myelo means Blood cells.

Dysplasia means abnormal growth.

Here below the classification of MDS:

French American British (FAB) Classification System
Even though the newer WHO system updated the FAB system, the FAB system is still used by some doctors today.

The FAB system is based on four main factors:

* The percentage of blast cells in the bone marrow
* The percentage of peripheral blood blasts
* The percentage of red blood cell precursors with abnormal iron deposits called ring sideroblasts
* The percentage of monocytes in the blood

The FAB system divides MDS into 5 subtypes. These subtypes include:

1. Refractory anemia, also called RA
2. Refractory anemia with ring sideroblasts, also called RARS
3. Refractory anemia with excess blasts, also called RAEB
4. Refractory anemia with excess blasts in transition, also called RAEB-t
5. Chronic Myelomonocytic Leukemia, or CMML

Diagnosis:
BME: cytopenia with dysplastic feature.Hypercelluler marrow.
Blast cell must not more than 30 %. defect in chromosom 5/7

Management:
Treatment is unsatisfactory. Blood transfusion with antibiotics.
Agressive antileukaemic therapy is not effective.
Retenoic acid is not fulfilling the promise.
Low dose cytosin arabinose has little effect
ABMT has 30% success .

International Prognostic Scoring System (IPSS)

The IPSS gives each patient a score to help their doctor understand how quickly their MDS is progressing and what is likely to happen to their disease over time. The score is based on several factors that are linked to MDS. They are:

• Percentage of blasts in the bone marrow
• Changes in cell chromosomes, also called cytogenetics
• Low blood cell counts or cytopenias
  

Each factor gets a score. Together, the scores tell which risk groups you fall into.

Your IPSS score helps your doctor to answer the following questions:

• How severe is your case of MDS?
• How likely is your case to become acute myeloid leukemia or AML if treated only with supportive care?
• How long are you likely to live if you are treated only with supportive care?

Factor	Value	IPSS Score
Blasts in bone marrow	less than 5% 5% to 10% 11% - 20% 21% - 30%	0 0.5 1.5 2.0
Cell DNA changes (cytogenics)	Good Intermediate Poor	0 0.5 2.0
Low blood counts or cytopenias	0 or 1 cytopenia 2 or 3 cytopenias	0 0.5

Using the IPSS, a patient is given a score between 0 and 3.0. A patient can be put into one of four risk groups, two lower-risk and two higher-risk. Scores of less than 1.5 are considered lower-risk.

This score tells you what risk group you fall into. For example:

• If your IPSS score is 0, you are in the low-risk group
• If your IPSS score is 0.5 to 1, you are in the intermediate-1 risk group
• If your IPSS score is 1.5 to 2, you are in the intermediate-2 risk group
  
• If your IPSS score is more than 2.0, you are in the high-risk group

Learn about Treatments for MDS.

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Prognosis:
First two is chronic disorder so less agressive.
Last three is agressive.