Haematology 7: Myelodysplastic Syndrome Flashcards

1
Q

What are the haematological features of Myelodysplastic syndrome ?

A

Cytopaenias

Qualitative abnormalities of erythrocytes, myelocytes and megakaryocytes maturation

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2
Q

Name the characteristic bilobed neutrophil seen in MDS ?

A

Pseudo Pelger-huet anomaly

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3
Q

List some abnormal cells seen with microscopy in MDS ?

A

Pseudo Pelger huet neutrophils
Ringed sideroblasts
Micro megakaryoblasts

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4
Q

Which disease does MDS often progress to ?

A

AML

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5
Q

Which drug can be used to treat MDS 5q- syndrome ?

A

Lenalidomide

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6
Q

List 3 Primary causes of bone marrow failure ?

A

Fanconi’s anaemia
Diamond blackfan anaemia
Kostmann’s syndrome

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7
Q

Which 2 antibiotics can cause bone marrow failure ?

A

Chloramphenicol

Sulphonamide

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8
Q

How is fanconi anaemia inherited ?

A

Mainly autosomal recessive but some are X linked

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9
Q

List 4 congenital abnormalities seen in children with Fanconi anaemia ?

A

Absent radii
Abnormal thumbs
Cafe au lait spots
Short stature

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10
Q

What is the pathognomonic triad of clinical signs of Dyskeratosis Congenital (DC)?

A

Skin pigmentation
Nail dystrophy
Leukoplakia

These children look very old! The disease causes premature aging

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11
Q

What is the genetic cause of Dyskeratosis congenita ?

A

Mutation in Genes that encode proteins involved in maintenance of telomerases. These telomerases get shortened at the ends. This causes premature ageing

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