Myelodysplasia

From Ganfyd

Introduction

Also known as Myelodysplastic syndromes. A collection of haematological malignancies seen mostly in the elderly. Characterised by a reduction of quality and quantity of the myeloid cell lines - red cells, granulocytes/monocytes and platelets. May transform to an Acute leukaemia. The opposite of myeloproliferative disorders although matters are not that simple.

FAB Classification

• Refractory anaemia (RA)
• Refractory anaemia with ringed sideroblasts (RARS)
• Chronic myelomonocytic leukaemia (CMML)
• Refractory anaemia with excess blasts (RAEB)
• Refractory anaemia with excess blasts in transformation (RAEB-T)

Aetiology

• Age - Mean age 60-80 year old
• Exposure to chemicals and toxins can lead to secondary MDS

Clinical

• Anaemia unresponsive to haematinics - Causes tiredness and fatigue
• Neutropenia - Recurrent infections excpt CMML where WCC may be high
• Low platelets - Bleeding problems not common
• Splenomegaly commoner in CMML
• Lymphadenopathy - not common

Exclude exposure to toxins or drugs that may cause a pancytopenia. Ensure no B12 or folate deficiency.

Investigations

• Blood film
• Bone marrow examination
• Flow cytometry

Complications

• Transformation to acute leukaemia (AML)
• Bone marrow failure

Management

• Hormonal therapy
• Differentiating agents
• Chemotherapy
• Bone marrow transplant
• Haemeopoietic growth factors
• Amifostine
• Cyclosporin A
• Anti-thymocyte globulin
• Topetecan