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Haematology > Pancytopenia > Flashcards

Pancytopenia Flashcards

1
Q

what is pancytopenia

A

a defic of blood cells of all lineages (but generally exclude lymphocytes)

anaemia, thrombocytopenia, neutropenia

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2
Q

does pancytopenia mean bone marrow failure

A

not always

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3
Q

lifespan of red cells
neutrophils
platelets

A

120 days
7-8 hours
7-10 days

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4
Q

turnover for red cells
neutrophils
platelets

A

2.5 mill per second are lost and produced
1-2 million
1 million

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5
Q

causes of pan

A

reduced production or increased destruction

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6
Q

reduced production causes

A

inherited
primary acquired - stem cell defect
secondary acquired - absence of b12 and folate

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7
Q

why do inherited marrow failure syndromes arise

what do they lead to

A

due to defects in DNA repair/ribosomes

cancer pre dispostion
congenital anomalies
impaired haemopoiesis

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8
Q

fanconi’s anaemia is what

signs /symo

A

inherited marrow failure synd

short, skin pigment abnormalities, radial ray abnormalities, hypogenitilia, endocrinopathies, GI defects, CDV, renal, haem

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9
Q 
median age onset for fanconis
what happens 
progress
prognosis 
risk
A

7 years
unable to correct inter strand cross links (DNA damage)

macrocytosis followed by thrombocytopenia then neutropenia

bone marrow failure (aphasia) risk is 84% by 20 years

leuk risk 52% by 40 years

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10
Q

primary acquired bone marrow failure

A

plastic anaemia
myelodysplastic syndromes
acute leuk

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11
Q

aplastic anaemia

A

autoimmune attack against haemopoietic stem cells
autoreactice T cells (esp CD* and sometimes CD4) produce IFN-gamma and TNF-alpha
this leads to lack of production of cells

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12
Q

myelodysplastic syndromes

A

dysplasia

hypercellular marrow

increased apoptosis of progenitor and mature cells (ineffective haemopoiesis)

propensity for evolution into AML 20-30% px

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13
Q

why can acute leuk cause pan

A

prolif of abnormal cells (blasts ) from leukaemia stem cells

fail to differentiate or mature into normal cells

prevents normal haemopoietic sten/proginator development by hijacking/altering haemopietic niche and marrow microenviroment

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14
Q

causes of secondary bone marrow failure

A

drug induced -> aplasia

B12 and folate defic - affects all lineages (hypercellular)

infiltrative - non haemopoietic malig infiltration, lymphoma

viral (e.g. HIV)/storage diseases

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15
Q

causes of increased destruction of cells

A

hypersplenism

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16
Q

hypersplenism

A

increased splenic pool

-increased destruction that exceeds bone marrow production - assoc w enlarged spleen

17
Q

splenic red cell mass

A

normal spleen 5%

hypersplenism 40%

18
Q

red cell transit

A

normal spleen fast

hypersplenism slow

19
Q

splenic platelet pool

A

normal 20-40% in the spleen

hypersplenism 90% can sit in the spleen

20
Q

is the splenic size linked to the hypersplenism

A

no

21
Q

causes of hyperpslenism

A

splenic congestion - portal htn, congestive HD

systemis dx - rheumatoid (felty’s)

haem dx - splenic lymphoma

22
Q

what are the clinical features of pan divided into

A

lack of circulating blood cells

the cause of the pancytopaenia

23
Q

symptoms- for lack of cells

A

anaemia - fatigue, SOB, cardiovascular compromise
neutropenia - infections
thrombocytopenia - bleeding in purport, petechiae

24
Q

clinical features depending on cause

A

Fanconi – skeleteal, genital, renal

Acquired – aplastic – history of other AI conditions, B12 and folate may have neuro problems, Infections

25
Q

ix for pan

A

FBC, blood film
B12.folate, LFTS [hypersplenism], virology, AI tests
bone marrow exam next
cryogenics - chromosome fragility testing in Fanconis syndrome

26
Q

bone marrow testing

A

from post iliac spine

bone marrow trephine biopsy with jamshidi needle

27
Q

mallular cellularity in pan

A

hypo cellular in aplastic anaemia

hypercellular in myelodysplastic, B12/folate defic, hyperplsenism

28
Q

treatment of pan

A

supportive

specific depending on cause

29
Q

supportive treatment

A

red cell transfusion
platelet transfusions
antibiotics treatment and prophylactic use - antibacterials, anti fungals, antivirals

30
Q

how should neutropenic fever be treated

A

promptly without waiting for microbiology results

31
Q

primary bone marrow disorder treatments

A

malig - chemo
congenital - bone marrow transplantation
idiopathic aplastic anaemia - immunosuppression

32
Q

secondary bone marrow disorder treatment

A

drug reaction - stop the drug
viral - treat
replace b12/folate

33
Q

hypersplenism treatment

A

treat cause if possible

consider splenectomy - not in all causes

Haematology (26 decks)

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