Pancytopaenia Flashcards

1
Q

Define pancytopaenia:

A

A deficiency of blood cells from ALL lineages

doesn’t tend to include lymphocytes

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2
Q

What are the two main causes of pancytopenia?

A

Reduced production
OR
Increased destruction

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3
Q

What are the main causes of decreased production?

A

Bone marrow failure:

Inherited (rare)
Acquired: primary (stem cell defect) or secondary

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4
Q

What are the clinical features of Fanconi’s Anaemia?

A
Short stature
Skin pigment abnormalities e.g. cafe au lait spots
Radial ray abnormalities
Hypogenitalia
Endocrinopathies
GI defects
CV, Renal and Haem defects
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5
Q

What is an example of an inherited bone marrow failure syndrome?

A

Fanconi’s Anaemia

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6
Q

What is the haematological abnormality that causes Fanconi’s?

A

They are unable to correct inter-strand cross links (DNA damage)

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7
Q

List some examples of acquired Primary bone marrow failures:

A

idiopathic aplastic anaemia
Myelodysplastic syndromes
Acute leukaemia

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8
Q

What is the pathogenesis in aplastic anaemia?

A

Auto-reactive T cells releasing IFN and TNF against the long and short term stem cells, the

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9
Q

How does acute leukaemia lead to pancytopaenia?

A

Proliferation of ABNORMAL cells (blasts) from the stem cells, which fail to differentiate/ mature
therefore normal stem cells cannot develop

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10
Q

What is another name for pancytopaenia when there is clear decrease in the haemopoietic tissue of the bone marrow?

A

Aplastic anaemia

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11
Q

What is the lifetime risk of developing leukemia in a patient with fanconi’s?

A

52% by age 40

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12
Q

What is the risk of bone marrow failure occurring in patient with Fanconi’s?

A

84% by 20 y/o

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13
Q

What is the common age of onset of pancytopaenia in children with Fanconi’s?

A

5-10 y/o

median = 7

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14
Q

Define dysplasia:

A

Disordered growth or development of cells

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15
Q

List some causes of secondary bone marrow failure:

A
  • Drug induced e.g. chemo
  • B12 / folate deficiency
  • Infiltrative e.g. non haem malignancy
  • Viral
  • Storage disorders
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16
Q

Hypersplenism causes pancytopaenia through ______ _____

A

Increased destruction

17
Q

List some causes of hypersplenism:

A
  • portal hypertension
  • congestive cardiac failure
  • rheumatoid arthritis (Felty’s)
  • Splenic lymphoma
18
Q

How does a folate / B12 deficiency lead to pancytopaenia?

A

(Chronic outcome)
B12 / Folate is responsible for lots of cellular DNA synthesis and nuclear maturation, in all lineages. When deficient, the lineages are all affected

19
Q

What are the three blood results of pancytopaenia?

A

Anaemia
Neutropaenia
Thrombocytopaenia

20
Q

List some important investigations for pancytopaenia:

A
FHx
FBC 
Blood film
LFTs
Auto-antibodies / virology
Bone marrow examination
Specialized tests incl. chromosome fragility test
21
Q

What syndrome is chromosome fragility testing done for?

A

Fanconi’s Syndrome

22
Q

Bone marrow with pancytopaenia with be hypocellular in _______?

A

Aplastic anaemia

23
Q

Bone marrow with pancytopaenia will be hypercellular in ________________________?

A

Myelodysplastic syndromes
B12 / folate deficiency
Hypersplenism

24
Q

List some of the supportive management options for pancytopaenia:

A
  • RBC transfusion
  • Platelet transfusion
  • Neutrophil transfusion is not routinely done
  • Prophylaxis antibiotics and anti-fungal
25
Q

What must you do if a patient with pancytopaenia presents with neutropaenic fever?

A
Treat immediately (with whatever guidelines say)
DO NOT wait for micro results
26
Q

When might a bone marrow transplant be a reasonable management of pancytopaenia?

A

In congenital causes of primary bone marrow disorders