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Haematology SOH > Pancytopenia > Flashcards

Pancytopenia Flashcards

1
Q

Definition

A

A deficiency of blood cells of ALL lineages (but generally excludes lymphocytes)

EG:
- erythrocytes, platelets, granulocytes

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2
Q

Pancytopenia always means that there is bone marrow failure. True or false?

A

False

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3
Q

Causes of pancytopenia result from 1 of 2 things:

A

Decreased production
OR
Increased destruction

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4
Q

Causes of decreased production

A

Bone marrow failure

  • inherited
  • acquired (primary or secondary)
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5
Q

Causes of increased destruction

A

Hypersplenism

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6
Q

Causes of decreased production - INHERITED bone marrow failure

A
  • Cancer pre-disposition
  • Impaired haemopoiesis
  • Congenital anomalies

Arises due to defects in DNA repair/ribosomes

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7
Q

Causes of decreased production - inherited bone marrow failure - give an example

A

Fanconi’s anaemia

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8
Q

Fanconis anaemia - what age do most people present

A

Around the age of 7

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9
Q

Fanconis anaemia - what is happening

A

Unable to correct inter-strand crosslinks (DNA damage)

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10
Q

Fanconis anaemia - haematological findings

A

Macrocytosis ->
Thrombocytopenia ->
Neutrophenia ->
Bone marrow failure

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11
Q

Fanconis anaemia - clinical features

A

Short stature

Skin pigment abnormalities (cafe a lait spots)

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12
Q

Causes of decreased production - ACQUIRED primary bone marrow failure

A

This is an intrinsic bone marrow problem so it is no longer able to make the blood cells as normal

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13
Q

Causes of decreased production - ACQUIRED primary bone marrow failure - idiopathic aplastic anaemia

A

Autoimmune attack against haemopoietic stem cells

End up with an empty marrow

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14
Q

Causes of decreased production - ACQUIRED primary bone marrow failure - name 3 examples

A
  • idiopathic aplastic anaemia
  • myelodysplastic syndromes
  • acute leukaemia
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15
Q

Causes of decreased production - ACQUIRED primary bone marrow failure - idiopathic aplastic anaemia - on examination of bone marrow, what is it like

A

Hypocellular or aplastic

Increased fat spaces

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16
Q

Causes of decreased production - ACQUIRED primary bone marrow failure - myelodysplastic syndromes MDS

A 
Dysplasia due to a defect in stem cells 
Hypercellular marrow (but many of these cells are going through apoptosis so this does not translate to an effective production of blood cells) 
Increased apoptosis
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17
Q

Causes of decreased production - ACQUIRED primary bone marrow failure - usually do to a problem with??

A

Stem cells

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18
Q

Causes of decreased production - ACQUIRED primary bone marrow failure - myelodysplastic syndromes MDS - there is a propensity for evolution into which condition?

A

Acute myeloid leukaemia

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19
Q

Causes of decreased production - ACQUIRED primary bone marrow failure - acute leukaemia - who can acute leukaemia cause pancytopenia

A

Proliferation of abnormal cells (blasts) from stem cells

Failure to differentiate or mature into normal cells

20
Q

Causes of decreased production - ACQUIRED secondary bone marrow failure

A

Drug induced
B12 / folate deficiency
Infiltrative
Metastatic cancer

21
Q

Causes of decreased production - ACQUIRED secondary bone marrow failure - drug induced

A

Chloramphenicol
Chemotherapy
Alcohol

Causes aplasia

22
Q

Causes of decreased production - ACQUIRED secondary bone marrow failure - B12 / folate deficiency

A

Nuclear maturation can affect all lineages

23
Q

Causes of decreased production - ACQUIRED secondary bone marrow failure - infiltrative

A

Non-haemopoietic malignant infiltration

Lymphoma

24
Q

Causes of increased destruction - hypersplenism

A

Increased splenic pool

25
Q

Causes of hypersplenism

A

Splenic congestion

  • portal hypertension
  • congestive heart failure

Systemic illness
- rheumatoid disease

Haematological diseases
- splenic lymphomas

26
Q

Pancytopenia - reduced production - overall cause

A

Primary bone marrow failure

27
Q

Pancytopenia - reduced production - inherited cause

A

Primary bone marrow failure - Fanconi anaemia

28
Q

Pancytopenia - reduced production - acquired causes PRIMARY (3)

A

Idiopathic aplastic anaemia

Myelodysplastic syndromes

Acute leukaemia

29
Q

Pancytopenia - reduced production - acquired causes SECONDARY (5)

A

Drug induced aplasia

Vitamin deficiency (B12, folate)

Infections

Metastatic cancer

Storage disorders

30
Q

What are the 3 constituent parts of pancytopenia?

A

Anaemia
Neutropenia
Thrombocytopenia

31
Q

Pancytopenia - anaemia component - clinical features

A

Fatigue
Shortness of breath
CVS compromise

32
Q

Pancytopenia - neutropenia component - clinical features

A

Infection signs

33
Q

Pancytopenia - thrombocytopenia - clinical features

A

Bleeding

  • purpura
  • petichae
34
Q

How do you establish the cause of pancytopenia

A 
History 
Clinical examination
FBC, blood film 
Additional routine tests 
Bone marrow examination
Specialist tests 
- e.g. if ?Fanconi's anaemia
35
Q

If ?Fanconi’s anaemia, what specialised test do you do?

A

Chromosome fragility testing

36
Q

Marrow cellularity is hypocellular/hypercellular in aplastic anaemia

A

Hypocellular

37
Q

Marrow cellularity is hypocellular/hypercellular in myelodysplastic syndromes

A

Hypercellular

38
Q

Marrow cellularity is hypocellular/hypercellular in B12/folate deficiency

A

Hypercellular

39
Q

Marrow cellularity is hypocellular/hypercellular in hypersplenism

A

Hypercellular

40
Q

Management of pancytopenia - supportive

A

Red cell transfusions

Platelet transfusions

41
Q

Patient develops neutropenic fever. Do you wait for microbiology results before commencing antibiotics?

A

NO

- start antibiotics immediately

42
Q

Management of pancytopenia - primary bone marrow disorder - idiopathic aplastic anaemia

A

Immunosuppression

43
Q

Management of pancytopenia - primary bone marrow disorder -congenital

A

Bone marrow transplant

44
Q

Management of pancytopenia - secondary bone marrow disorder - drug reaction

A

Stop the drug

45
Q

Management of pancytopenia - hypersplenism

A 
Treat cause (if possible) 
Consider splenectomy

Haematology SOH (22 decks)

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