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Haematology week 2 > Pancytopenia > Flashcards

Pancytopenia Flashcards

1
Q

Define pancytopenia

A

A reduction in all the major cell lines; RBC’s, platelets and WBC’s

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2
Q

Is pancytopenia a diagnosis ?

A

No

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3
Q

Does pancytopenia always mean bone marrow failure ?

A

No

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4
Q

For a steady state of haematopoiesis what needs to be balanced ?

A

The rate of cell production to the rate of cell destruction (breakdown/loss)

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5
Q

Recall the lifespan for RBC’s, neutrophils and platelets

A
  1. RBC’s - 120days
  2. Neutrophils - 7-8hrs
  3. Platelets - 7-10days
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6
Q

What are the 2 main processes which result in a pancytopenia ?

A

Reduced production or increased destruction

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7
Q

What are the causes for reduced production which can result in a pancytopenia ? (bone marrow failure causes)

A
  • Can be inherited or Acquired
  • Acquired can be primary or secondary in cause
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8
Q

What is aplastic anaemia and how does it present?

A
  • It is a rare stem cell disorder leading to pancytopenia and hypocellular bone marrow with no abnormal cells seen
  • Presents with features of bone marrow failure - anaemia (often normocytic), infections and bleeding
  • Hypocellularity in the bone marrow without evidence of significant dysplasia, blasts, fibrosis, or other abnormal infiltrate.
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9
Q

What test is needed for diagnosis for aplasitc anaemia ?

A

Bone marrow biopsy showing:

A hypocellular marrow is a definitive diagnostic finding for AA. Moreover, there should be an absence of abnormal cell population (such as blasts) and no fibrosis.

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10
Q

What is the pathogenesis behind aplastic anaemia ?

A

Autoimmune attack against haemopoietic stem cell(s) – preventing any of the linegaes

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11
Q

What are the causes of aplastic anaemia ?

A
  • It can be caused by heredity e.g. fanconis anaemia,
  • Immune disease
  • Exposure to chemicals, drugs, or radiation.
  • However, in about half the cases, the cause is unknown.
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12
Q

What are the hallmarks of inherited marrow failure syndromes and what mutations do they arise from ?

A

Arise due to defects in DNA repair/ribosomes

Hallmarks are:

  • Impaired haematopoiesis
  • Cancer pre-disposition
  • Associated congenital abnormalities
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13
Q

What is the main inherited marrow failure syndrome we should know about and how does it arise?

A

Fanconis anaemia

Arises due to detective stem cell repair and chromsome fragility where they are Unable to correct inter-strand cross-links (DNA damage)

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14
Q

Describe the features of fanconis anaemia

A
  • Short stature
  • Skin pigment abnormalities
  • Radial ray abnormalities
  • Hypogenitilia
  • Endocrinopathies
  • GI defects
  • Cardiovascular
  • Renal
  • Haematological
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15
Q

How does fanconis anaemia progress and what are the risk factors associated with it ?

A
  1. Macrocytosis (macrocytic anaemia) followed by thrombocytopenia, then neutropenia
  2. Bone marrow failure (aplasia) risk: 84% by 20 years
  3. Leukaemia risk: 52% by 40 years

Increased risk of AML and breast cancer (think back to the fact that inherited marrow failure syndromes increase the risk of malignancy)

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16
Q

What are the 2 main types of aquired causes for bone marrow failure (pancytopenia)?

A
  1. Can be primary i.e. the cause arises in the bone marrow
  2. Or can be secondary i.e. the cause does not arise in the bone marrow but causes pancytopenia
17
Q

What are the main primary causes of bone marrow failure ?

A
  1. Idiopathic aplastic anaemia
  2. Myelodysplatic syndromes
  3. Acute leukaemias
18
Q

What are myelodysplastic syndromes and who are they common in ?

A
19
Q

What is the appearance of the bone marrow shown and what condition is it associated with ?

A

Shows a hypocellular bone marrow on the R sided pic - this is diagnostic of aplastic anaemia

20
Q

What are myelodysplatic syndromes ?

A

A group of disoders which manifest as marrow failure due to the bone marrow producing abnormal cells causing ineffective haematopoiesis as there is increased apoptosis of progenitor and mature cells

21
Q

What are the test results you would see when investigating myelodysplastic syndromes ?

A
  • Hypercellular bone marrow on biopsy due to increased production because of the ineffective haematopoiesis
  • May see ring sideroblasts in the marrow
22
Q

What can myelodysplastic syndromes progress into ?

A

AML

23
Q

How does acute leukaemia result in pancytopenia ?

A
  • Proliferation of ABNORMAL cells (blasts)
  • Prevent normal haemopoietic stem/progenitor (HSC) development by ‘hijacking’/altering the haemopoietic niche and marrow microenvironment
24
Q

What are some of the secondary causes of bone marrow failure (pancytopenia)?

A
  1. Drug induced [eg chemotherapy, chloramphenicol, alcohol] – causes aplasia
  2. B12/folate deficiency (nuclear maturation can affect all lineages) (remember hypercellular!)
  3. Infiltrative- non-haemopoietic malignant infiltration, lymphoma
  4. Viral (eg HIV)
  5. storage diseases
25
Q

Note bone marrow failure is the cause of pancytopenia due to reduced production

A

Haematology week 2 (17 decks)

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