Myelodysplastic Syndromes and Bone Marrow Failure Flashcards
What are myelodysplastic syndromes (MDS)?
Biologically heterogeneous group of acquired haemopoietic stem cell disorders (~ 4 per 100,000 persons).
What is MDS characterised by?
The development of a clone of marrow stem cells with abnormal maturation resulting in functionally defective blood cells AND a numerical reduction.
What is the epidemiology of MDS?
Typically a disorder of the elderly.
Symptoms/signs are those of general marrow failure.
Develops over weeks & months.
What are some blood and bone marrow morphological features of MDS?
- Pelger-Huet anomaly (bilobed neutrophils)
- Dysganulopoieses of neutrophils
- Dyserythropoiesis of red cells
- Dysplastic megakaryocytes – e.g. micromegakaryocytes
- Increased proportion of blast cells in marrow (normal < 5%)
What is this?
Normal neutrophils
What is this?
Pelger-Heut anomaly
What is this?
Refractory anaemia dysgranulopoiesis
What is this?
Myelokathexis
What is this?
Refractory anaemia-dyserythropoiesis
What is this?
Refractory anaemia-dyserythropoiesis
What is this?
Ringed sideroblasts
What is this?
Auer rods
What are Auer rods a sign of?
AML
Which prognostic variables are included in the Revised International Prognostic Scoring System (IPSS-R) in MDS (2012)?
BM blasts (%)
Karyotype
Hb (g/L)
Platelets (x10^9/L)
Neutrophils (x10^9/L)
How is the IPSS-R score interpreted?
The higher the score, the lower the survival and time to progress to AML.