Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Path - Haematology > Myelodysplastic syndromes and Aplastic Anaemia > Flashcards

Myelodysplastic syndromes and Aplastic Anaemia Flashcards

1
Q

What is a myelodysplastic syndrome?

A

An acquired haematopoeic stem cell disorder
Characterised by the development of a clone of marrow stem cells with abnormal maturation, resulting in
- functionally defective blood cells
- numerical reduction in blood cells

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

what are blood features of myelodyasplastic syndromes?

A
  • Peiger-Huet (boiled neutrophils)
  • Dysgranylopoesis of neutrophils (failure of granulation)
  • Dyserythropoesis of RBC (blebbing)
  • Dysplastic megacaryocytes (micro-megakaryocyte)
  • Increased blast cells
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are ringed sideroblasts

A

Accumulation of iron granules around the nucleus of myeloid precursor

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

What are Auer rods diagnostic of?

A

ACUTE MYELOID LEUKAEMIA

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

What prognostic variables are important for MDS?

A 
BM blasts (%)
karyotype 
Hb
Platelet 
Neutrophil
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are MDS causes of death?

A

1/3 from infection
1/3 from bleeding
1/3 from acute leukaemia

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

Which 2 treatments prolong life in MDS?

A

Allogenic stem cell transplant

Chemotherapy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Why are SC transplant/chemo often not appropriate for MDS patients

A

Because most patients are ELDERLY

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

What kind of supportive care is appropriate?

A

Blood products support
Antimicrobial therapy
Growth factors (EPO, G-CSF)

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

Which drugs can be given for MDS?

A

Immunosuppression
Azacytidine
Decitabine
Lenalidomide

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

What is the oral chemotherapy for MDS called?

A

Hydroxyurea= hydroxycarbamide

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are primary causes of BM failure?

A

*Fanconis
*Idiopathic aplastic anaemia
Diamond-Blackfan anaemia
Kostmann syndrome

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

What are secondary causes of BM failure?

A

Surgical sieve

Vascular: Haematological malignancies (e.g. leukaemia, lymphoma, myelofibrosis) 
Infection (parvovirus, viral hepatitis)
AI 
Marrow infiltration 
Drugs, Radiation, Chemicals (benzene)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What kind of drugs can cause BM failure?

A 
Cytotoxic drugs 
Phenylbuzatone, gold salts 
Antibiotics (chloramphenicol, sulphonamide) 
Thiazide diuretics 
Anti thyroid drug (Carbimazole)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the age incidence for aplastic anaemia?

A

BIMODAL
15-24
60+

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is the likely aetiology of aplastic anaemia

A

Idiopathic UNKNOWN

possibly Autoimmune

17
Q

What is the clinical presentation of BM failure?

A

Anaemia
Thrombocytopoenia
Leukopoenia

18
Q

What does a bone marrow slide look like in aplastic anaemia?

A

HYPOCELLULAR (few cells, mainly fat)

19
Q

What are the Camitta Criteria for SEVERE aplastic anaemia?

A

2 out of 3 peripheral blood features:

  • Reticulocytes <1%
  • Neutrophils <0.5
  • Platelets <20
20
Q

What drugs can you use to promote marrow recovery in BM failure?

A

Oxymetholone

Thrombopoetin Receptor Antagonist

21
Q

How is Fanconi Anaemia inherited?

A

Autosomal recessive.
OR
X linked

22
Q

In how many children with FA do congenital abnormalities occur?

A

70%

23
Q

What are abnormalities in children with FA?

A 
Short stature 
Cafe au lait spots 
Short/non-existent thumbs 
Microcephaly/hydrocephaly 
Hypogonadism 
Developmental delay
24
Q

What are common complications of FA?

A 
Aplastic anaemia 
Leukaemia 
Liver disease 
Myelodysplasia 
Cancer
25
Q

What is dyskeratosis congenita?

A

Inherited condition characterised by:

  • marrow failure
  • cancer predisposition
  • somatic abnormalities

SCaM

26
Q

What is triad of somatic abnormalities in dyskeratosis congenital?

A

Skin pigmentation
Nail dystrophy
leukoplakia

27
Q

What occurs to telomeres in dystkeratosis congenital?

A

Their LENGTH is REDUCED

28
Q

what criteria do you use for severe aplastic anaemia?

A

Camitta criteria

29
Q

What features are required for camitta criteria?

A

2 of 3 on peripheral blood

  • low reticulocytes
  • low neutrophils
  • low platelets
30
Q

what is the overview of management for bone marrow failure?

A

supportive (blood/platelet transfusion, antibiotics, iron chelation therapy)

Promote marrow recovery (TPO receptor agonist)

Stem cell transplant

Path - Haematology (17 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions