Myelodysplasia

Question 1

What is myelodysphasia?

Answer 1

It is a group of acquired bone marrow disorders characterized by increasing bone marrow failure due to a defect in stem cells

Question 2

What is the prognosis of myelodysplasia?

Answer 2

Morbidity and mortality are high. Mean survival is 6 months to 6 years

30% of cases develop acute myeloblastic leukaemia

Question 3

How does myelodysplasia present clinically?

Answer 3

Increased infections

Symptoms of anaemia

Increased bleeding

Question 4

What Ix findings are present in myelodysplasia?

Answer 4

Pancytopenia with reduced reticulocyte count

Ring sideroblasts are present

Marrow cellularity is increased due to dyserythropoiesis

Question 5

How are patients with myelodysplasia Mx?

Answer 5

RBC and platelet transfusion with accompanying Fe chelation therapy

+/- EPO and granulocyte colony stimulating factor

Bone marrow transplantation if HLA-matched donor is available

Prophylactic anti-microbials