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Haematology > Pancytopenia > Flashcards

Pancytopenia Flashcards

1
Q

What is pancytopeenia?

A

A deficiency of blood cells of all lineages (generally excludes lymphocytes)

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2
Q

For steady state haemostasis, what must occur?

A

Cell production= cell destruction

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3
Q

What are the fundamental causes behind pancytopenia?

A

Reduced production or increased destruction

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4
Q

What are the causes of reduced production?

A

Bone marrow failure- Inherited syndromes, acquired: primary/secondary

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5
Q

What are the characteristics of inherited marrow failure syndromes?

A

Cancer pre-disposition
Impaired haemopoiesis
Congenital anomalies

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6
Q

Due to what do inherited marrow failure syndromes arise?

A

Defects in DNA repair/ribosomes

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7
Q

What are the clinical features of inherited marrow failure syndromes (very rare, e.g. Fanconi’s anaemia)?

A 
Short stature
Skin pigment abnormalities
Radial ray abnormalities
Hypogenitilia
Endocrinopathies
GI defects
Cardiovascular
Renal
Haematological
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8
Q

What skeletal and skin abnormalities occur in inherited marrow failure syndromes?

A

Oligodactyly

Café au lait spots

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9
Q

What haematological abnormalities occur in inherited marrow failure syndromes?

A

Median age onset:7yo
Unable to correct inter-strand cross-links (DNA damage)
Macrocytosis followed by thrombocytopenia, then neutropenia
Bone marrow failure (aplasia) risk: 84% by 20 years
Leukaemia risk: 52% by 40 years

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10
Q

What are the causes of acquired primary bone marrow failure?

A

Aplastic anaemia: AI attack against haemopoietic stem cell
Myelodysplastic syndrome (MDS)
Acute leukaemia

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11
Q

What cytokines are involved in aplastic anaemia?

A

IFN gamma

TNF alpha

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12
Q

What occurs in myelodysplastic syndrome?

A

Dysplasia
Hypercellular marrow
Increased apopotosis of progenitor and mature cells (inefffective haemopoiesis)
Propensity for evolution into AML

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13
Q

Why can acute leukaemia cause pancytopenia?

A

Proliferation of ABNORMAL cells (blasts) from leukaemic stem cells (LSC)
Failure to differentiate or mature into normal cells
Prevent normal haemopoietic stem/progenitor (HSC) development by ‘hijacking’/altering the haemopoietic niche and marrow microenvironment

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14
Q

What can cause secondary bone marrow failure?

A

Drug induced [eg chemotherapy, chloramphenicol, alcohol] – causes aplasia
B12/folate deficiency (nuclear maturation can affect all lineages) (remember hypercellular)
Infiltrative- non-haemopoietic malignant infiltration, lymphoma
Misc.: Viral (eg HIV)/storage diseases

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15
Q

What does hypersplenism cause?

A

Increased splenic pool

Increased destruction that exceeds bone marrow capacity, usually associated with significantly enlarged spleen

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16
Q

What changes occur with the splenic pool in hypersplenism?

A

Increased splenic red cell mass (from 5% to 40%)
Red cell transit slowed
Splenic platelet pool increased (20-40% to 90%)

17
Q

What are the causes of hypersplenism?

A

Splenic Congestion- Portal Hypertension, Congestive cardiac failure
Systemic diseases- Rheumatoid Arthritis (Felty’s)
Haematological diseases- Splenic lymphoma

18
Q

What is the triad of features in pancytopenia?

A

Anaemia
Neutropenia
Thrombocytopenia

19
Q

Signs and symptoms of pancytopenia can reflect what?

A

Lack of circulating blood cells

Cause of pancytopenia

20
Q

What are the signs and symptoms of anaemia?

A

Fatigue
SOB
CV compromise

21
Q

How is the cause of pancytopenia established?

A

History, including family history
Clinical findings
FBC, Blood film
Additional routine tests guided by above (B12/folate, LFT’s, virology, autoantibody tests)
Bone marrow examination (trephine biopsy)
Specialised tests guided by above (cytogenetics, eg chromosome fragility testing in Fanconi’s syndrome)

22
Q

How is a core marrow biopsy obtained?

A

Jamshidi needle

23
Q

What is the marrow cellularity in pancytopenia?

A

Variable
Hypocellular in aplastic anaemia
Hypercellular in MDS, B12/Folate deficiency, hypersplenism

24
Q

What is the treatment of pancytopenia?

A

Supportive

Specific- dependent on cause

25
Q

What is supportive treatment in pancytopenia?

A

Red cell transfusions
Platelet transfusions
Antibiotics and prophylaxis
Treat neutropenic fever promptly based on local policy without waiting for results

26
Q

What is the specific treatment for pancytopenia caused by primary bone marrow disorder?

A

Malignancy – consider chemotherapy
Congenital – consider bone marrow transplantation
Idiopathic Aplastic Anaemia – Immunosuppression

27
Q

What is the specific treatment for pancytopenia caused by secondary bone marrow disorder?

A

Drug reaction – STOP
Viral – eg treat HIV
Replace B12/folate

28
Q

What is the specific treatment for pancytopenia caused by hypersplenism?

A 
Treat cause if possible
Consider splenectomy (not appropriate in all cases)

Haematology (23 decks)

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