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3 HAEM > pancytopenia > Flashcards

pancytopenia Flashcards

1
Q

what is pancytopaenia

A

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

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2
Q

which cells are most affected in pancytopenia

A

RBCs
platelets
granulocytes

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3
Q

what cells are present the pathway of the maturation of an RBC

A 
pronormoblast 
early normoblast 
intermediate normoblast 
late normoblast 
reticulocyte
RBC
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4
Q

what cells are present in the pathway of neutrophil maturation

A 
myeloblast 
promyelocyte 
myelocyte 
metamyelocyte 
band neutrophil
segmented neutrophil
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5
Q

what are the average lifespans for RBCs, neutrophils and platelets

A

RBCs: 120 days

neutrophils: 7-8 hours
platelets: 7-10 days

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6
Q

causes of reduced production of all cell types

A

inherited syndromes causing bone marrow failure

acquired bone marrow failure (primary or secondary)

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7
Q

what are the clinical features of Fanconi’s anaemia

A 
short stature
skin pigment abnormalities 
hypogenitalia 
endocrinopathies 
GI/CV/renal defects 
haematological symptoms
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8
Q

haematological abnormalities in Fanconi’s anaemia

A

macrocytosis followed by thrombocytopenia, then neutropenia
bone marrow failure by 20 years
increased leukaemia risk

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9
Q

examples of primary bone marrow failure

A

idiopathic aplastic anaemia (autoimmune attack of haemopoietic stem cells)
myelodysplastic syndromes
acute leukaemia

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10
Q

which cells are responsible for autoimmune attack in aplastic anaemia

A

auto-reactive T cells

release IFN-gamma and TNF-alpha

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11
Q

what are features of myelodysplastic syndromes

A

dysplasia (disordered development)
hypercellular marrow
increased apoptosis of progenitor and mature cells (ineffective haemopoiesis)

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12
Q

myelodysplastic syndromes carry a risk of evolving into…

A

acute myeloid leukaemia

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13
Q

why can leukaemia cause pancytopenia

A

proliferation of abnormal cells (blasts) from leukaemia stem cells results in failure to differentiate or mature into normal cells

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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: abnormal nuclear maturation

infiltrative (non-haemopoieotic malignant infiltration, lymphoma)

viral/storage diseases

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15
Q

what can cause increased destruction of all cell types

A

hypersplenism

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16
Q

vascular supply of the spleen

A 
splenic artery (coeliac axis)
splenic vein (with SMV forms portal vein)
17
Q

how does hypersplenism cause pancytopaenia

A

increased splenic pool results in increased destruction of cells
that exceeds bone marrow capacity for production

18
Q

what can cause hypersplenism

A

splenic congestion: portal HTN, congestive cardiac failure

system disease: RA

haematological diseases: splenic lymphoma

19
Q

clinical features of pancytopaenia

A

anaemia: fatigue, SOB, CV compromise
neutropenia: infections (severity/duration)
thrombocytopenia: bleeding (purport, petechiae, visceral bleeds)

20
Q

what investigations should be done following FBC and blood film

A 
depending on results:
B12/folate 
LFTs
virology 
autoantibody tests 
bone marrow examination
21
Q

what is bone marrow trephine biopsy

A

take a little bit of bone away

22
Q

how does marrow cellularity reflect the cause of pancytopenia

A

hypocellular: aplastic anaemia
hypercellular: myelodysplastic syndromes (proliferation and apoptosis), B12/folate deficiency, hypersplenism

23
Q

examples of supportive treatment in pancytopenia

A

red cell/platelet transfusions
(neutrophil transfusions not routine)

antibiotics prophylaxis/treatment

24
Q

how should neutropaenic fever be managed

A

treat promptly with empiric antibiotics without waiting for microbiology results

25
Q

specific management of primary bone marrow disorders

A

malignancy: chemo
congenital: bone marrow transplant

idiopathic aplastic anaemia: immunosuppression

26
Q

specific management of secondary bone marrow disorder

A

drug reaction: stop drug

viral: treat virus

B12/folate: supplements

27
Q

specific management of hypersplenism

A

treat cause if possible

consider splenectomy

3 HAEM (20 decks)

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