Haematology 13 - Myelodysplastic syndromes and aplastic anaemias Flashcards Preview

Year 5 Pathology ICSM > Haematology 13 - Myelodysplastic syndromes and aplastic anaemias > Flashcards

Flashcards in Haematology 13 - Myelodysplastic syndromes and aplastic anaemias Deck (14)
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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
- Antibiotics
- GF
Can add biological modifiers:
- Immunosuppressive therapy
- Azacytidine


How does azacytidine work in the treatment of myelodysplastic syndromes?

Hypomethylating agent
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%
Inherited causes:
- Fanconi's anaemia
- Dyskeratosis anaemia
- Schwachman-Diamond syndrome
Acquired causes:
- 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
- ciclosporin
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?

Telomere shortening


What is the pseudo-pelger-huet anomaly?

Hyposegmented neutrophils seen in myelodysplastic syndromes


Recall the options for treatment in essential thrombocytosis

1. Aspirin (to reduce thrombus formation)
2. Anagrelide (reduced formation of platelets from megakaryocytes)
3. Hydroxycarbamide

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