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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
Causes of hypersplenism
Splenic congestion - portal hypertension - congestive heart failure Systemic illness - rheumatoid disease Haematological diseases - splenic lymphomas
26
Pancytopenia - reduced production - overall cause
Primary bone marrow failure
27
Pancytopenia - reduced production - inherited cause
Primary bone marrow failure - Fanconi anaemia
28
Pancytopenia - reduced production - acquired causes PRIMARY (3)
Idiopathic aplastic anaemia Myelodysplastic syndromes Acute leukaemia
29
Pancytopenia - reduced production - acquired causes SECONDARY (5)
Drug induced aplasia Vitamin deficiency (B12, folate) Infections Metastatic cancer Storage disorders
30
What are the 3 constituent parts of pancytopenia?
Anaemia Neutropenia Thrombocytopenia
31
Pancytopenia - anaemia component - clinical features
Fatigue Shortness of breath CVS compromise
32
Pancytopenia - neutropenia component - clinical features
Infection signs
33
Pancytopenia - thrombocytopenia - clinical features
Bleeding - purpura - petichae
34
How do you establish the cause of pancytopenia
``` History Clinical examination FBC, blood film Additional routine tests Bone marrow examination Specialist tests - e.g. if ?Fanconi's anaemia ```
35
If ?Fanconi's anaemia, what specialised test do you do?
Chromosome fragility testing
36
Marrow cellularity is hypocellular/hypercellular in aplastic anaemia
Hypocellular
37
Marrow cellularity is hypocellular/hypercellular in myelodysplastic syndromes
Hypercellular
38
Marrow cellularity is hypocellular/hypercellular in B12/folate deficiency
Hypercellular
39
Marrow cellularity is hypocellular/hypercellular in hypersplenism
Hypercellular
40
Management of pancytopenia - supportive
Red cell transfusions | Platelet transfusions
41
Patient develops neutropenic fever. Do you wait for microbiology results before commencing antibiotics?
NO | - start antibiotics immediately
42
Management of pancytopenia - primary bone marrow disorder - idiopathic aplastic anaemia
Immunosuppression
43
Management of pancytopenia - primary bone marrow disorder -congenital
Bone marrow transplant
44
Management of pancytopenia - secondary bone marrow disorder - drug reaction
Stop the drug
45
Management of pancytopenia - hypersplenism
``` Treat cause (if possible) Consider splenectomy ```

Haematology SOH (22 decks)

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