Myelodysplasia

Question 1

Define Myelodysplasia?

Answer 1

A series of haematological conditions characterised by chronic cytopaenia (anaemia, neutropaenia, thrombocytopaenia) and abnormal cellular maturation

Question 2

What are the five subgroups of Myelodysplasia?

Answer 2

Refractory Anaemia (RA)
RA with ringed sideroblasts (RARS)
RA with excess blasts (RAEB)
Chronic myelomonocytic leukaemia (CMML)
RAEB in transformation (RAEB-t)

Question 3

What is the aetiology of Myelodysplasia?

Answer 3

It may be primary (intrinsic bone marrow problem)
It may arise in patients who have received chemotherapy or radiotherapy for previous malignancies
Patients may have chromosoml abnormalities

Question 4

What is the epidemilogy of Myelodysplasia?

Answer 4

Mean age of diagnosis: 65-75 yrs
More common in MALES
2 x as common as AML

Question 5

What are the presenting symptoms of Myelodysplasia?

Answer 5

May be asymptomatic and diagnosed on routine blood counts

Symptoms of Bone Marrow Failure

Question 6

What are the Symptoms of Bone Marrow Failure present in Myelodysplasia?

Answer 6

Anaemia (Fatigue, dizziness)
Neutropaenia (recurrent infections)
Thrombocytopaenia (easy bruising, epistaxis)

Question 7

What’s important to remember with Myelodysplasia?

Answer 7

Check risk factors

Question 8

What are the risk factors for Myelodysplasia?

Answer 8

Occupational exposure to toxic chemicals

Prior chemotherapy or radiotherapy

Question 9

What are the signs of Myelodysplasia on physical examination?

Answer 9

Anaemia (pallor, cardiac flow murmur)
Neutropaenia (infections)
Thrombocytopaenia (purpura or ecchymoses)
Gum hypertrophy
Lympadenopathy
Spleen NOT enlarged (except in CMML)

Question 10

What investigations would you do for Myelodysplasia?

Answer 10

Bloods
Blood Film
Bone marrow aspire or biopsy

Question 11

What bloods do we do for Myelodysplasia and why?

Answer 11

FBC - pancytopaenia

Question 12

What do we see on a Blood Film for Myelodysplasia?

Answer 12

Normocytic or macrocytic red cells
Variable microcytic red cells in RARS 
Low granulocytes 
Granulocytes are not granulated 
High monocytes in CMML

Question 13

What do we see on a Bone Marrow Aspire or Biopsy for Myelodysplasia?

Answer 13

Hypercellularity
Ringed Sideroblasts (haemosiderin deposits in the mitochondria of erythroid precursors forming an apparent ring around the nucleus)
Abnormal granulocyte precursors
10% show marrow fibrosis