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Y3 - Haem > Pancytopenia > Flashcards

Pancytopenia Flashcards

1
Q

What is pancytopenia?

A

Deficiency in all blood cell lineages - RBCs, WBC, platelets

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2
Q

What are the causes of pancytopenia?

A 
Idiopathic aplastic anaemia
Panconi anaemia
Vit B12 and folate deficiency
Drugs: chemotherapy, chloramphenicol, NSAIDs
Infections: B19, TB, HIV
Myelodysplastic syndromes
Acute leukaemia
Myeloma
Hypersplenism
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3
Q

What is aplastic anaemia?

A

Anaemia due to marrow failure defined by pancytopenia and hypocellular bone marrow

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4
Q

In aplastic anaemia are abnormal cells found in the peripheral blood or bone marrow?

A

No

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5
Q

What is the pathophysiology of aplastic anaemia?

A

Reduction in the number of stem cells with a fault in those remaining or immune reaction against them resulting in them being unable to repopulate the bone marrow
Can progress to myelodysplasia, paroxysmal nocturnal haemoglobinuria or acute myeloid leukaemia

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6
Q

What are causes of aplastic anaemia?

A

Idiopathic aplastic anaemia
Fanconi anaemia
Chemicals - benzene, glue sniffing
Drugs - cytotoxic, chloramphenicol, sulphonamides, phenytoin, gold, NSAIDs
Infections - parvovirus (B19), hepatitis, EBV, HIV, TB
Radiation

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7
Q

What is the most common cause of aplastic anaemia

A

Idiopathic aplastic anaemia

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8
Q

What is idiopathic aplastic anaemia?

A

Autoimmune cause of bone marrow failure

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9
Q

What is Fanconi anaemia?

A

Inherited autosomal recessive cause of aplastic anaemia

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10
Q

What is the pathophysiology of Fanconi anaemia?

A

Failure to repair DNA crosslinks

Defective DNA repair in stem cells, predisposing to DNA fragility and mutations

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11
Q

What is the presentation of Fanconi anaemia?

A 
Short stature
Abnormal skin pigment
Missing thumb, radius
Hypogenitalia
Endocrinopathies
GI, cardiovascular, renal defects
Reduced IQ
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12
Q

Which conditions does Fanconi anaemia have a much higher risk of?

A

Bone marrow failure

Acute myeloid leukaemia

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13
Q

What is the management for Fanconi anaemia?

A

Stem cell transplant

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14
Q

What do investigations show in aplastic anaemia?

A

Bloods - pancytopenia, absence of reticulocytes

Bone marrow trephine biopsy - hypo cellular marrow with increased fat spaces

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15
Q

What is myelodysplasia?

A

Dysplasia (disordered development) of the bone marrow

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16
Q

What is the pathophysiology of myelodysplasia?

A

Acquired DNA mutations in haemopoietic stem cells result in hypercellular bone marrow and ineffective haemopoiesis
This causes increased apoptosis of progenitor and mature cells

17
Q

What are some management options for myelodysplasia?

A 
Regular blood transfusions
Immunosuppression
Erythropoietin
Chemotherapy
Lenalidomide
Allogenic SCT
18
Q

How does acute leukaemia cause pancytopenia?

A

Proliferation of abnormal cells from leukaemia stem cells produces cells that fail to differentiate or mature into normal cells

19
Q

What is hypersplenism?

A

Increased destruction of blood cells, exceeding bone marrow capacity

20
Q

What are causes of hypersplenism?

A 
Splenic congestion
- Portal hypertension
- Chronic liver disease
Rheumatoid arthritis
Splenic lymphoma
21
Q

What are the clinical features of pancytopenia?

A 
Anaemia 
- fatigue
- shortness of breath
- cardiovascular compromise
Neutropenia
- infections
- increased severity and duration of infections
Thrombocytopenia
- bleeding
- purpura
- petechiae
22
Q

What investigations can be done to determine the cause of pancytopenia?

A 
FBC
Blood film
B12/folate, LFTs, virology, autoantibody tests
Bone marrow examination
Genetic tests
23
Q

What are the hypo and hyper cellular causes of pancytopenia?

A 
Hypocellular:
- aplastic anaemia
Hypercellular
- myelodysplastic syndromes
- B12/folate deficiency
Hypersplenism
24
Q

What are the options for supportive management of pancytopenia?

A

Red cell transfusions
Platelet syndromes
Antibiotic prophylaxis/treatment
Treat neutropenic fever promptly

25
Q

What is the management for congenital causes of pancytopenia?

A

Bone marrow transplant

26
Q

What is the management for idiopathic aplastic anaemia?

A

Immunosuppression

Y3 - Haem (9 decks)

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