Haematology - Myelodysplastic Syndrome Flashcards
What are myelodysplastic syndromes?
A heterogenous group o fprogressive disorders featuring ineffective proliferation and differentiation of abnormally maturing myeloid stem cells
What are characteristics of myelodysplastic syndromes?
- Peripheral cytopenia
- Quantitative abnormalities of cell maturation
- Risk of AML transformation
- Typically seen in elderly
- Sx develop over weeks/months (incidental)
By definition: All patients have <20% blasts (>20% blasts = acute leukaemia)
What are some clinical features of myelodysplastic syndromes?
- BM failure + cytopenias = infection, bleeding, fatigue
- Hypercellular BM
- Macrocytic anaemia
Defective cells:
- RBCs: RING SIDEROBLASTS (Ab nucleated blast surrounded by iron granule ring)
- WBCs: Hypogranulation, PSEUDO-PELGER-HUET anomaly (hyposegmented neutrophil)
- Platelets: Micromegakaryocytes, Hypolobulated nuclei
What is the general treatment of meylodysplastic syndromes?
Supportive:
- Transfusions
- EPO
- G-CSF
- Abx
Biological Modifiers:
- Immunosuppressive drugs
- Lenalidomide
- Azacytidine
Chemotherapy:
- Similar to AML
Allogenic SCT
What scorin system is used for myelodysplastic syndromes and how is it used?
International Prognostic Scoring System (IPSS):
- BM Blasts %
- Karyotype
- Degree of cytopenia
What is the prognosis of myelodysplastic syndromes?
- Risk of progression to AML
Mortality rule of 1/3:
- 1/3 die from infection
- 1/3 die from haemorrhage
- 1/3 die from acute leukaemia
What are the blood and bone marrow features of refractory anaemia?
Blood:
- Anaemia
- No blasts
BM:
- Erythroid dysplasia with <5% blasts
What are the blood + bone marrow features of refractory anaemia with ringed sideroblasts?
Blood:
- Anaemia
- No blasts
BM:
- Erythroid dysplasia with >15% ringed sideroblasts
What are the blood + bone marrow features of refractory cytopenia with multiilneage dysplasia (RCMD)?
Blood:
- Cytopenia in >= 2 cell lines
BM:
- Dysplasia in >10% cells in >= 2 cell lines
What are the blood + bone marrow features of refractory cytopenia with multilineage dysplasia and ringed sideroblasts (RCMD + RS)?
Blood:
- Cytopenias in >= 2 cell lines
BM:
- Dysplasia in >10% cells in >= 2 cell lines
- >15% ringed sideroblasts
What are the blood + bone marrow results of refractory anaemia with excess blasts - 1 (RAEB I)?
Blood:
- Cytopenias
- <5% blasts
- NO AUER RODS
BM:
- Dysplasias
- 5-9% blasts
What are the blood + bone marrow results of refractory anaemia with excess blasts - 2 (RAEB II)?
Blood:
- Cytopenias OR 5-19% blasts OR AUER RODS
BM:
- Dysplasias
- 10-19% blasts OR AUER RODS
What are the blood + bone marrow results of MDS with 5q deletion?
Blood:
- Anaemia
- Normal or increased platelets
BM:
- Megakaryocytes with hypolobulated nuclei
- <5% blasts
What are the blood + bone marrow results of myelodysplasia syndrome (unclassified)?
Blood:
- Complex
- Cytopenias
- No blasts
- No Auer rods
BM:
- Complex
- Myeloid or megakaryocytic dysplasia
- <5% blasts
What are some causes of thrombocytosis?
- Acute infection
- Chronic infection
- Malignancy (5-10%)
- Essential thrombocythaemia
- Polycythaemia rubra vera
