Flashcards in Haematology 13 - Myelodysplastic syndromes and aplastic anaemias Deck (14)
What are the possible causes of death in myelodysplastic syndromes?
1/3 die of bleeding
1/3 die of infection
1/3 die of AML
What are the 2 possible curative treatments for myelodysplastic syndromes, and what is the biggest issue with them?
1. Allogenic stem cell transplant
2. Intensive chemotherapy
Sadly, most patients can't benefit from either for one reason or another
In myelodysplastic syndrome patients who are not suitable for curative treatment, how should disease be managed?
Supportive treatments include:
- Blood products
Can add biological modifiers:
- Immunosuppressive therapy
How does azacytidine work in the treatment of myelodysplastic syndromes?
Causes blood count to rise
Recall 3 drugs that can cause bone marrow failure
1. Cytotoxic drugs (eg chemo)
2. Antibiotics (particularly chloramphenicol)
3. Thiazide diuretics
What is the age distribution of aplastic anaemia?
Peak 1: 15-24 years
Peak 2: >60 years
What are the possible causes of aplastic anaemia
Idiopathic in 75%
- Fanconi's anaemia
- Dyskeratosis anaemia
- Schwachman-Diamond syndrome
- Herpes viruses
What are the 2 classifications of aplastic anaemia, and how is classification decided?
Severe or non-severe
Decided by *Camitta criteria*:
- Aplastic anaemia is severe if 2 or more of the following peripheral blood features are present:
- Reticulocytes <1%
- Neutrophils <0.5
- Platelets <20
PLUS: Bone marrow cellularity must be <25%
How should idiopathic aplastic anaemia be treated?
For all patients: androgens (oxymethalone)
For older patients: immunosuppression
- anti-lymphocyte globulin
For younger patients: stem cell transplant
Recall some symtoms of Fanconi's anaemia
Short stature, hypogonadism, thumb abnormality, cafe au lait spots
What is the triad of clinical features of dyskeratosis congenita?
1. Skin pigmentation
2. Nail dystrophy
3. Oral leukoplakia
"SNOB" = the above triad + BM failure - useful mnemonic
What is the genetic basis of dyskeratosis congenita?
What is the pseudo-pelger-huet anomaly?
Hyposegmented neutrophils seen in myelodysplastic syndromes