Myelodysplastic syndrome

Question 1

MDS - what investigations would you like to see to confirm the diagnosis and prognostication?

Answer 1

1. FBC - cytopaenia
2. Blood film - dysplastic neutrophils, dysplasia affecting ≥10% of a specific myeloid lineage
3. Bone marrow
   - Biopsy: blasts <20%, BM dysplasia affecting ≥10% of a specific myeloid lineage
   - Flow cytometry, cytogenetics, molecular genetics to _r/o other haematological malignancies, classify MDS and for prognosticatio_n.

For example, 5q deletion (if present, eligible for Lenalidomide)

Question 2

Presentation of MDS? (2)

Answer 2

Usually due to cytopaenia - incidental, infection. bleeding, anaemia.

Transfusion dependence

Question 3

Risk factors for MDS? (2)

Answer 3

Age >65

Previous chemo or RTx - secondary MDS has worse prognosis.

Question 4

What is the staging system for MDS and how is it determined (3)?

Answer 4

It is called IPSS-R (international prognostic scoring system)

Based on

Cytogenetics

% Blasts in BM

Cytopenias (Hb, Plt, Neut)

Question 5

How would you describe a prognosis for a patient diagnosed with MDS?

Answer 5

The main causes of mortality are 1) BM failure & 2) transformation to AML.

Survival depends on the staging: IPSS-R (based on cytogenetics, % blasts and cytopaenias) so I would like to review this information first.

Overall median survival is estimated to be between a few months to 6 years, depending on IPSS-R.

Question 6

What is your approach to managing this patient with MDS?

Answer 6

Goals: maximise self-efficacy, minimise complications from BM failure, improve QOL.

Confirm Dx: BM biopsy (% blasts), Cytogenetics and FBC for prognostication + IPSS-R score.

A: identify & treat depression

Screen for potential complications: infection, bleeding, anaemia & treat/transfuse. Identify & treat iron overload.

T: Non-pharm

• Educate: the major cause of mortality is BM related complications - importance of avoiding contacts, hygiene, vaccination to prevent infection. Monitoring for bleeding, symptomatic anaemia - seek medical attention.
• Provide information pack + link up with MDS support groups
• Regular transfusion requirement: facilitate organising regular transport
• Involve palliative care, discuss ACD

T: Pharm

• Transfusion + Fe chelation therapy if at risk of Fe overload
• G-CSF in consultation with Haematology
• Azacitadine (IPSS-R high-risk group)
• Lenalidomide for 5q del
• Consider ASCT for younger patients

Question 7

Adverse effects of Azacitadine? (3)

Answer 7

BM suppression

Hepatotoxicity

Renal impairment

Question 8

Lenalidomide side effects? (4)

Answer 8

Diarrhoea

Increased risk of VTE

Steven-Johnson syndrome

Teratogenic