Haem - MDS + Aplastic Anemia Flashcards
Define a myelodysplastic syndrome
Ineffective proliferation and differentiation
Clinical features of MDS
Cytopenias –> fatigue, bleeding, infection
What do defective blood cells in MDS look like?
RBCs = ring sideroblasts
WBCs = Pseudo-Pelger Huet anomaly
Hypogranulation
Plts = micromegakaryocytes
% blasts in MDS?
<20%
Pseudo-Pelger Huet anomaly - what is it? in whom is it seen?
Hypo segmented neutrophils!
MDS
How is MDS prognosis calculated? what does it determine?
IPSS!!
% blasts, cytopenias, Karyotype
Determines survival + risk of AML transformation
MDS - how do patients die?
1/3 = infection
1/3 bleeding
1/3 leukaemia
Tx of MDS?
Definitive = allogenic SCT or chemo
Supportive - EPO, blood products, G-CSF
What is aplastic anemia
BM is unable to produce adequate numbers of blood cels
- typically RBCs but can have pancytopenia
Diagnosis of aplastic anaemia - how?
FBC - cytopenias
Marrow - hypocellular
Classification of types of aplastic anaemia?
Primary vs Secondary
Causes of primary aplastic anemia
Idiopathic (70%)
Inherited - Fanconi’s anemia, Dyskeratosis Congenita
Causes of secondary aplastic anemia
Malignant infiltration SLE Hepatitis Drugs - NSAIDs, chloramphenicol Chemo Radio
Management of aplastic anaemia = 4 points
- Supportive - transfusions, Abx, iron chelation
- Promoting marrow recovery - oxymethalone, GFs
- Immunosuppressants - anti-thymocyte globulin, cyclosporin
- SCT
Supportive tx used in aplastic anemia
Transfusions
Abx
Iron chelation therapy