Haem - MDS + Aplastic Anemia Flashcards

1
Q

Define a myelodysplastic syndrome

A

Ineffective proliferation and differentiation

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2
Q

Clinical features of MDS

A

Cytopenias –> fatigue, bleeding, infection

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3
Q

What do defective blood cells in MDS look like?

A

RBCs = ring sideroblasts
WBCs = Pseudo-Pelger Huet anomaly
Hypogranulation
Plts = micromegakaryocytes

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4
Q

% blasts in MDS?

A

<20%

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5
Q

Pseudo-Pelger Huet anomaly - what is it? in whom is it seen?

A

Hypo segmented neutrophils!

MDS

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6
Q

How is MDS prognosis calculated? what does it determine?

A

IPSS!!
% blasts, cytopenias, Karyotype

Determines survival + risk of AML transformation

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7
Q

MDS - how do patients die?

A

1/3 = infection
1/3 bleeding
1/3 leukaemia

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8
Q

Tx of MDS?

A

Definitive = allogenic SCT or chemo

Supportive - EPO, blood products, G-CSF

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9
Q

What is aplastic anemia

A

BM is unable to produce adequate numbers of blood cels

- typically RBCs but can have pancytopenia

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10
Q

Diagnosis of aplastic anaemia - how?

A

FBC - cytopenias

Marrow - hypocellular

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11
Q

Classification of types of aplastic anaemia?

A

Primary vs Secondary

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12
Q

Causes of primary aplastic anemia

A

Idiopathic (70%)

Inherited - Fanconi’s anemia, Dyskeratosis Congenita

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13
Q

Causes of secondary aplastic anemia

A
Malignant infiltration
SLE
Hepatitis
Drugs - NSAIDs, chloramphenicol
Chemo
Radio
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14
Q

Management of aplastic anaemia = 4 points

A
  1. Supportive - transfusions, Abx, iron chelation
  2. Promoting marrow recovery - oxymethalone, GFs
  3. Immunosuppressants - anti-thymocyte globulin, cyclosporin
  4. SCT
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15
Q

Supportive tx used in aplastic anemia

A

Transfusions
Abx
Iron chelation therapy

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16
Q

Drugs used in aplastic anaemia to promote marrow recovery

A

Oxymethalone

Growth factors

17
Q

2 immunosuppressants used in aplastic anemia

A

ATG = anti-thymocyte globulin

Cyclosporin

18
Q

3 complications of ATG and cyclosporin for AA treatment

A

1) AA relapse
2) MDS/leukaemia
3) solid tumours

19
Q

pathophys of idiopathic aplastic anemia

A

Immune attack of stem cells, by CD8+ cytotoxic T cells

20
Q

Normal FBC at birth, pancytopenia at 5-10 yo.

A

Fanconi’s anemia

21
Q

Physical deformities in Fanconi’s anemia

A
Thumb deformities
Microcephaly
Hypogonadism
Short stature
Cafe au lait spots
22
Q

Triad for dyskeratosis congenita

A

Nail dystrophy + oral leukoplakia + skin pigmentation

23
Q

Genetics for dyskeratosis congenita

A

Telomere shortening

X-linked or autosomal dominant