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Haematology > Pancytopenia > Flashcards

Pancytopenia Flashcards

1
Q

Define pancytopenia

A

A deficiency of blood cells of all lineages

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2
Q

Name the two key causes of pancytopenia

A

Reduced production

Increased destruction

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3
Q

What are the causes of reduced production?

A

Bone marrow failure - inherited or acquired

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4
Q

What are the features of inherited bone marrow failure?

A

Impaired haemopoiesis
Congenital anomalies
Cancer pre-disposition

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5
Q

Why do inherited conditions cause marrow failure?

A

Defects in DNA repair/ribososmes/telomeres

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6
Q

Name an example of an inherited bone marrow failure disease

A

Faconi’s Anaemia

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7
Q

What are the clinical features of Faconi’s?

A

Short stature, skin pigment abnormalities (cafe au lait) radial ray abnormality, hypogenitalia, endocrinopathies, GI, cardio, renal defects

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8
Q

Describe Faconi’s haematological problems

A

Onset 7 years old
Unable to correct cross links in strands of DNA
Macrocytosis and thrombocytopenia

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9
Q

What does Faconi’s anaemia increase your risk of?

A

Marrow failure - 84% by 20 years old

Leukaemia - 52% by 40 years old

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10
Q

State the causes of primary acquired bone marrow failure

A

Idiopathic aplastic anaemia
Myelodysplastic syndrome
Acute leukaemia

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11
Q

Describe idiopathic aplastic anaemia

A

Auto-reaction T cells against stem cells and progenitors

INF gamma and TNF alpha produced

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12
Q

What will trephine biopsy of idiopathic aplastic anaemia show?

A

Loss of all haemopoietic cells and excess of fat spaces

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13
Q

What is myelodysplastic syndrome?

A

Disordered development and increased apoptosis of progenitor and mature cells leads to hyper cellular bone marrow

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14
Q

What can myelodysplastic syndrome progress to?

A

AML

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15
Q

What can help predict risk of AML in myelodysplastic syndrome?

A

Cytogenetic/chromosome abnormalities

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16
Q

How does acute leukaemia cause pancytopenia?

A

Proliferation of leukaemia stem cells that fail to mature/differentiate prevents normal haemopoietic development by altering the niche

17
Q

State the causes of secondary acquired marrow failure

A 
Drug induced - hypo cellular 
B12/folate deficiency - hyper cellular 
Infiltrative 
Viral 
Storage disease
18
Q

What drugs can cause marrow failure?

A 
Chemotherapy 
Azathioprine 
Chloramphenicol 
Alcohol 
Methotrexate
19
Q

What causes increased marrow destruction?

A

Hypersplenism

20
Q

What are the features of hypersplenism?

A

Splenomegaly, reduced circulation of blood cells and compensatory increase in cellularity of bone marrow

21
Q

What causes hypersplenism?

A

Congestion - portal hypertension
Systemic - rheumatoid arthritis
Haematological - splenic lymphoma

22
Q

What is meant by splenic pooling?

A

Spleen red cell mass increases
Red cells transit is much slower
Platelet pool increases

23
Q

What are the clinical features of pancytopenia?

A

Anaemia
Neutropenia
Thrombocytopenia

24
Q

How is pancytopenia investigated?

A

FBC, blood film
Additional - B12/folate, LFT, virology, autoantibodies
Bone marrow examination
Specialised - cytogenetics

25
Q

How is pancytopenia treated?

A

Supportive - transfusions/antibiotics

Specific to cause

26
Q

How is primary pancytopenia treated?

A

Malignancy - chemo
Congenital - bone marrow transplant
Aplastic anaemia - immunosuppression or transplant

27
Q

How is secondary pancytopenia treated?

A

Drugs - stop and consider antidote
Treat infection
Replace B12/folate

28
Q

What is the antidote to methotrexate?

A

Folic acid

29
Q

How is hypersplenism treated?

A

Treat cause

Consider splenectomy

Haematology (18 decks)

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