Haematology 13 - Myelodysplastic syndromes and aplastic anaemias Flashcards

1
Q

What are the possible causes of death in myelodysplastic syndromes?

A

1/3 die of bleeding
1/3 die of infection
1/3 die of AML

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are the 2 possible curative treatments for myelodysplastic syndromes, and what is the biggest issue with them?

A
  1. Allogenic stem cell transplant
  2. Intensive chemotherapy
    Sadly, most patients can’t benefit from either for one reason or another
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

In myelodysplastic syndrome patients who are not suitable for curative treatment, how should disease be managed?

A
Supportive treatments include: 
- Blood products
- Antibiotics
- GF
Can add biological modifiers: 
- Immunosuppressive therapy
- Azacytidine
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

How does azacytidine work in the treatment of myelodysplastic syndromes?

A

Hypomethylating agent

Causes blood count to rise

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Recall 3 drugs that can cause bone marrow failure

A
  1. Cytotoxic drugs (eg chemo)
  2. Antibiotics (particularly chloramphenicol)
  3. Thiazide diuretics
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the age distribution of aplastic anaemia?

A

Bimodal
Peak 1: 15-24 years
Peak 2: >60 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are the possible causes of aplastic anaemia

A
Idiopathic in 75%
Inherited causes: 
- Fanconi's anaemia
- Dyskeratosis anaemia
- Schwachman-Diamond syndrome
Acquired causes: 
- Herpes viruses
- SLE
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the 2 classifications of aplastic anaemia, and how is classification decided?

A

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 well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

How should idiopathic aplastic anaemia be treated?

A

For all patients: androgens (oxymethalone)
For older patients: immunosuppression
- anti-lymphocyte globulin
- ciclosporin
For younger patients: stem cell transplant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Recall some symtoms of Fanconi’s anaemia

A

Short stature, hypogonadism, thumb abnormality, cafe au lait spots

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is the triad of clinical features of dyskeratosis congenita?

A
  1. Skin pigmentation
  2. Nail dystrophy
  3. Oral leukoplakia
    “SNOB” = the above triad + BM failure - useful mnemonic
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is the genetic basis of dyskeratosis congenita?

A

Telomere shortening

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is the pseudo-pelger-huet anomaly?

A

Hyposegmented neutrophils seen in myelodysplastic syndromes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Recall the options for treatment in essential thrombocytosis

A
  1. Aspirin (to reduce thrombus formation)
  2. Anagrelide (reduced formation of platelets from megakaryocytes)
  3. Hydroxycarbamide
How well did you know this?
1
Not at all
2
3
4
5
Perfectly