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Year 3 Clinical > Pancytopaenia > Flashcards

Pancytopaenia Flashcards

1
Q

Define pancytopaenia?

A

Deficiency of blood cells of ALL lineages (but generally excluding lymphocytes)

Patient has anaemia + neutropaenia + thrombocytopaenia

It is not a diagnosis and does not always mean bone marrow failure

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2
Q

Lifespan of rbcs?

A

~120 days (longest life span of any blood cell)

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3
Q

Lifespan of neutrophils?

A

~7-8 hours

Cell turnover is very high, which is why the myeloid : erythroid ratio favours the myeloid compartment

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4
Q

2 broad causes of pancytopaenia?

A

Reduced production

OR

Increased destruction

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5
Q

Causes of reduced production in pancytopaenia?

A

Bone marrow failure:
• Inherited syndromes - arise due to defects in DNA repair / ribosomes; very rare
• Acquired (primary or secondary) - more common

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6
Q

Occurrence of Fanconi’s anaemia?

A

Very rare; median age of onset if 7 years of age

Short stature, skin pigment abnormalities (cafe au lait spots), radial ray abnormalities, hypogenitalia, endocrinopathies

GI, CV, renal, haematological defects

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7
Q

Cause of haematological abnormalities in Fanconi’s anaemia?

A

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

Patients has a macrocytosis followed by a thrombocytopenia and then a neutropenia

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8
Q

Risk of bone marrow failure (aplasia) in Fanconi’s anaemia?

A

84% by the age of 20 years old (very high risk)

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9
Q

Risk of leukaemia in Fanconi’s anaemia?

A

52% by 40 years old (very high risk)

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10
Q

Evolution of Fanconi’s anaemia?

A

Patient starts with bone marrow failure, with no mutations

They sustain mutations, due to an inability to repair DNA, and eventually develop cancer

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11
Q

Reasons for acquired primary bone marrow failure (reduced production)?

A

No obvious cause; although there is usually a stem cell defect

There is an intrinsic marrow issue, i.e: problem with the cells in the bone marrow

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12
Q

Causes of an intrinsic marrow problem, i.e: acquired primary bone marrow failure?

A

Idiopathic aplastic anaemia - autoimmune attack against haemopoietic stem cell(s)

Myelodysplastic syndrome (MDS)

Acute leukaemia (WCC can be variable)

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13
Q

Pathogenesis of aplastic anaemia?

A

Auto-reactive T cells produce IFN-ᵞ and TNF-α, which result in death of the HSC, so there are no progenitors or mature cells

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14
Q

Bone marrow biopsy appearance of aplastic anaemia?

A

Normal cells are absent and replaced with fat

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15
Q

What is myelodysplastic syndrome?

A

Dysplastic cells create a HYPERCELLULAR marrow

There is increased apoptosis of progenitor and mature cells (ineffective haemopoiesis)

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16
Q

Risks assoc. with myelodysplastic syndrome?

A

Propensity for evolution into AML

17
Q

Occurrence of myelodysplastic syndrome?

A

Relatively common >60 years old

18
Q

Why can acute leukaemia cause pancytopaenia?

A
  1. Proliferation of ABNORMAL cells (blasts) from leukaemic stem cells (LSC)
  2. Failure to differentiate or mature into normal cells
  3. Prevent normal HSC development by altering the haemopoietic niche and marrow micro-environment
19
Q

Causes of acquired secondary bone marrow failure?

EXAMS

A

Drug-induced, e.g: chemotherapy, CHLORAMPHENICOL (antibiotic), alcohol - cause aplasia

B12 / folate deficiency (NUCLEAR MATURATION CAN AFFECT ALL LINEAGES) - causes HYPERCELLULAR MARROW

Infiltrative, e.g: non-haemopoietic malignancy infiltration, lymphoma

Others:
• Viral, e.g: HIV
• Storage disease

20
Q

Why does B12 / folate deficiency cause hypercellular marrow?

A

Hypoxia, due to the anaemia, triggers increased Epo production and the more primitive compartment of the bone marrow begins to expand

21
Q

Causes of increased destruction resulting in pancytopaenia?

A

Hypersplenism:
• Increased splenic pool
• Increased destruction that exceeds bone marrow capacity, usually assoc. with significantly enlarged spleen

Any cause of splenomegaly can potentially cause hypersplenism
NOTE - splenic size alone may not always correlate with hypersplenism

22
Q

Compare features of the normal spleen vs hypersplenism?

A

Normal spleen:
• Splenic red cell mass - 5%
• Red cell transit - fast
• Splenic platelet pool - 20-40%

Hypersplenism:
• Splenic red cell mass - 40%
• Red cell transit - slow
• Splenic platelet pool - 90%

23
Q

Causes of hypersplenism?

A

Splenic congestion:
• Portal hypertension
• CCF

Systemic disease:
• RA (Felty’s)

Haematological disease:
• Spenic lymphoma

24
Q

Summarise the causes of pancytopaenia?

A

ADD IMAGE

25
Q

What do the signs and symptoms of pancytopaenia reflect?

A

Lack of circulating blood cells, i.e: anaemia, neutropaenia and thrombocytopaenia

Cause of the pancytopenia

26
Q

Symptoms due to anaemia?

A

Fatigue

SoB

CV compromise

27
Q

Symptoms due to neutropaenia?

A

Infections (severity, duration)

28
Q

Symptoms due to thrombocytopaenia?

A

Bleeding:
• Purpura
• Petechiae
• ‘Wet’ bleeds, inc. visceral bleeds

29
Q

How to establish the cause of pancytopaenia?

A

History, inc. DH and FH

Clinical findings

FBC and blood film

Additional routine tests  guided by above:
• B12/folate
• LFT’s
• Virology
• Auto-antibody tests

Bone marrow examination:
• Bone marrow aspirate
• Core biopsy (retrieved using Jamshidi needle)

Specialised tests guided by above:
• Cytogenetics, e.g: chromosome fragility testing in Fanconi’s syndrome (expose chromosome to DNA damaging agent; cells cannot repair themselves so abnormalities occur)

30
Q

Classes of marrow cellularity in pancytopaenia?

A

Hypocellular in aplastic anaemia

Hypercellular:
• Myelodysplastic syndromes (due to proliferation and apoptosis)
• B12 / folate deficiency (late maturation failure + early proliferation + apoptosis)

Hypersplenism

31
Q

Treatment of pancytopaenia?

A
  1. Supportive

2. Specific - dependent on cause, e.g: pancytopaenia due to B12 / folate deficiency

32
Q

Supportive treatments for pancytopaenia?

A

Red cell transfusions

Platelet transfusions

Antibiotics treatment and prophylactic use:
• Anti-bacterials - treat neutropaenic fever empirically and promptly, with broad-spectrum antibiotics
• Anti-fungals
• Anti-virals

33
Q

Specific treatment of primary bone marrow disorders?

A

Malignancy – consider chemotherapy

Congenital – consider bone marrow transplantation

Idiopathic Aplastic Anaemia – IMMUNOSUPPRESSION

34
Q

Specific treatment of secondary bone marrow disorders?

A

Drug reaction – STOP

Viral, e.g: treat HIV

Replace B12/folate

35
Q

Specific treatment of hypersplenism?

A

Treat cause if possible

Consider splenectomy (not appropriate in all cases)

Year 3 Clinical (100 decks)

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