Pancytopenia Flashcards

1
Q

What is the definition of pancytopenia?

A

Deficiency in blood cells in the blood (usually excludes lymphocytes)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the life span of neutrophils and platelets?

A
N = 7-8 hours
P = 7-10 days
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What problems involving reduced production can cause pancytopenia?

A

Bone marrow failure - primary or secondary

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is Fanconi’s syndrome?

A

An inherited marow failure syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are features of Fanconi’s?

A
Short stature
Skin pigmented abnormalities
Radial ray abnormalities
Hypogenitalia
Endocrinopathies
GI defects
Cardiovascular
Renal
Haematological
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What skin changes can be seen in Fanconi’s?

A

Cafe au lait spots

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are the haematological issues in Fanconi’s?

A

Unable to correct inter-strand cross-links (DNA damage)

Macrocytosis followed by thrombocytopenia, then neutropenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

In Fanconi’s, what is the risk of bone marrow failure and leukaemia?

A
BM = 84% by 20 years
L = 52% by 40 years
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are acquired primary bone marrow failure disorders?

A

An intrinsic marrow problem

  • Aplastic anaemia (autoimmune attack against own haemopoietic stem cell
  • Myelodysplastic syndromes (MDS)
  • Acute leukaemia (white cell count can be variable)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Describe Myelodysplastic syndrome?

A

Dysplasia
Hypercellular marrow
increased apoptosis of progenitor and mature cells (ineffective haemopoiesis)
Propensity for evolution into AML

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Why can acute leukaemia cause pancytopenia?

A

Proliferation of abnormal cells (blasts) from leukaemia stem cells
Failure to differentiate or mature into normal cells
Prevent normal haemopoietic stem/progenitor development

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Give examples of secondary bone marrow failure?

A

Drug induced (chemo/alcohol)
B12/folate deficiency (nuclear maturation)
Infiltrative - non-haemopoietic malignant infiltration, lymphoma
Viral/storage diseases (HIV)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What can cause increased destruction of blood cells?

A

Hyperslenism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the normal spleen values for a) splenic red cell mass, b) red cell transit and c) splenic platelet pool?

A

a) 5%
b) Fast
c) 20-40%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are the hyperslenism values for a) splenic red cell mass, b) red cell transit and c) splenic platelet pool?

A

a) 40%
b) Slow
c) 90%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are causes of hyperslenism?

A

Splenic congestion - Portal hypertension, Congestive cardiac failure
Systemic distress - RA (Felty’s)
Haematological - Splenic lymphoma

17
Q

What 3 things predominantly make up pancytopenia?

A

Anaemia
Neutropenia
Thrombocytopenia

18
Q

What are signs/symptoms of anaemia?

A

Fatigue
SOB
Cardiovascular compromise

19
Q

What can occur in neutropenia?

A

Infections

20
Q

What can occur in thrombocytopenia?

A

Bleeding
Purpura
Petechiae

21
Q

What type of biopsy is used to examine bone marrow, and what needle is used?

A

Trephine biospy

Jamshidi needle

22
Q

In pancytopenia, when is the marrow hypocellular?

A

In aplastic anaemia

23
Q

In pancytopenia, when is the marrow hypercellular?

A

In myelodysplastic syndromes
B12/folate deficiency (maturation failure)
Hypersplenism

24
Q

What supportive treatment can be offered for pancytopenia?

A

Red cell/platelet transfusion

ABx and prophylactic use of antibacterials, antifungals and antivirals

25
Q

What should you treat based on local ABx policy ASAP without waiting on microbiology results?

A

Neutropenic fever

26
Q

How do you treat primary bone marrow disorder?

A

Malignancy - chemo
Congenital - bone marrow transplant
Idiopathic aplastic anaemia - Immunosuppression

27
Q

How do you treat secondary bone marrow disorder?

A

Drug reaction - STOP
Viral - treat the virus
Replace B12/folate

28
Q

How do you treat hyperslenism?

A

Treat cause if possible

Consider splenectomy