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Haematology > Pancytopenia > Flashcards

Pancytopenia Flashcards

1
Q

What is pancytopenia?

A

a deficiency of blood cells of all lineages (generally excludes lymphocytes)
anaemia + neutropenia + thrombocytopenia

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2
Q

What are the causes of reduced production resulting in pancytopenia?

A

bone marrow failure

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3
Q

What are the characteristics of inherited marrow failure syndromes?

A

cancer predisposition; imapired haemopoiesis; congenital anomalies

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4
Q

Why do inherited marrow failure syndromes arise?

A

defects in DNA repair/ribosomes

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5
Q

give an example of an inherited marrow failure syndrome?

A

Fanconi’s anaemia

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6
Q

What are the features of fanconi’s anaemia?

A

short stature; skin pigment abnormalies; hypogenitalia; GI defects; CVS; renal; radial ray abnormalities

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7
Q

What is the dermatomogical feature of fanconi’s anaemia?

A

cafe au lait spots

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8
Q

What is the average age of onset of fanconis anaemia?

A

7 years

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9
Q

What is the underlying defect in Fanconi’s anaemia?

A

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

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10
Q

What are the blood changes seen in fanconi’s anaemia?

A

macrocytosis–thrombocyopenia–neutropenia

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11
Q

Waht are the causes of acquired primary bone marrow failure?

A

idiopathic aplastic anaemia; myelodysplastic syndromes; acute leukaemia

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12
Q

What is the pathogenesis of aplastic anaemia?

A

auto-reactive T cells which produce IFN-gamma and TNF-alpha which prevents development of stem cells and precursors

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13
Q

What are hte features of myelodysplastic syndrome?

A

dysplasia; hypercellular marrow; increased apoptosis of progenitor and mature cells

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14
Q

What cancer does myelodysplastic syndrome evolve into?

A

AML

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15
Q

Why can acute leukaemia cause pancytopenia?

A

proliferation of abnormal cells (blasts) from leukaemic stem cells, failure to differentiate or mature into normal cells; prevention of normal haemopoeitic stem development by hijacking the niche and marrow microenviornment

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16
Q

What are the causes of secondary bone marrow failure?

A

drugs; B12/folate deficinecy; infilitrative; infections; storage disease

17
Q

What drugs can cause marrow aplasia?

A

chemotherapy; chloramphenicol; alcohol

18
Q

What is the cellularity of the marrow with B12/folate deficinecy?

A

hypercellular

19
Q

What happens in hypersplenism?

A

increased splenic pool of RBCs and platelets; increased destruction; slower RBC transit

20
Q

What are the causes of hypersplenism?

A

portal hypertension; CCF; RA (Felty’s); splenic lymphoma

21
Q

What is Felty’s syndrome?

A

RA; splenomegaly; pancytopenia

22
Q

What infections are implicated in pancytopenia?

A

HIV; mycobacetria

23
Q

What are the signs of anaemia?

A

fatigue; SOB; CVS compromise

24
Q

What are the signs of thrombocytopenia?

A

purpura; petechiae; wet bleeds

Haematology (29 decks)

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