Haem 3 Flashcards

(68 cards)

1
Q

malignant haemopiesis is what

A

increased number of dysfunctional cells that have lost the normal haemopoiteic reserve

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2
Q

one or more of what is in malignant haemopoiesis

A

increased proliferation
lack of differentiation
lack of maturation
loss of apoptosis

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3
Q

acute leukaemia

A

abnormal proliferation and lack of maturation

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4
Q

ix for acute leukaemia

A

excess of blasts >20% in either peripheral blood or bone marrow
decreased/ loss of normal haemopoietic reserve

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5
Q

ALL is a malig disease of what
most common what
symptoms

A

malig disease of lymphocytes
most common childhood cancer
very severe bone pain, BM failure : infections, bleeding, anaemia
increased WCC, venous obstruction, CNS involvement

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6
Q
AML in who 
may be what of two 
similar to what 
subgroups 
occurs after treatment of what 
ix
A
>60s
may be denovo or decondary 
similar to ALL
DIC or gum infiltration 
alkylating agents 
auer rods
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7
Q

what can be done as ALL and AML look the same sometimes

A

BM aspirate immunophenytyping

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8
Q

myeloproliferative disorders is what

A

clonal haemopoietic stem cell disorders with an increase production of one or more haemopoitetic cells - in contrast to acute leukaemia maturation is relatively preserved

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9
Q

common features of myeloproliferative disorders

A
asymp
gout
weight loss
fatigue
sweats 
splenomegaly
thrombus in unusual place - no reactive explanation
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10
Q

CML is what

A

over production of grnaulocutes

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11
Q

pattern of CML

A

prolif of myeloid cells, chronic phase with intact maturation 3-5y, accelerated phase, blast crisis (acute leukaemia)

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12
Q

when is fatal of CML

A

fatal without stem cell/BM transplant in chronic phase

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13
Q

ix for CML

A

normal or low Hb
leucocytes/neurtrophilia
myeldoi precursors
eosinophilia, basophilia, thrombocytosis

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14
Q

philapdelphia

A

9+22 -> tyrosine kinase which causes abnormal phosphorylation - signalling leading to haematological changes in CML

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15
Q

treatment of CML

A

imatinib

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16
Q

PRV is overproduction of what

A

overproduction of RBCs

high Hb/hc accompanied by erythrocytes

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17
Q

ddx of secondary polycythaemia

A

chronic hypoxia, smoking, erytho secreting tumour

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18
Q

ddx of pseudopolycythaemia

A

dehydration, diuretics, obesity

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19
Q

aquagenic pruritus

A

polycythaemia ruba vera

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20
Q

ix for PRV

A

FBC
film
JAK2 mutation
px with secondary won’t have splenomegaly

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21
Q

JAK2

A

kinase

activation of erythropoiesis in absence of ligand

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22
Q

treatment of PRV

A

venesection to haemocrit <0.45
aspirin to reduce thromb risk
hydroxycarbomide - oral chemo

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23
Q

essential thrombocytopenia

high can lead to what

A

uncontrolled production of abnormal platelets

at high levels can lead to VW dx

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24
Q

symp of ET

A

MPD symp and bleeding (unpredictable risk of surgery)

microvascular occlusion

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25
ix for ET
exclude reactive thrombosis JAK2 in 50% CALR in rest MPL mutation characteristic bone marrow appearance
26
treatment for ET
aspirin hydoxycarbamide anagrelide interferon alpha last line
27
myelofibrous idiopathic
marrow failure, marrow fibrosis, extra medullary haemopoesis, leukthrombin film
28
tear dropped shaped RBC
myelofibrosis
29
when else can you get myelofibrosis
post polycythaemia or ET
30
BF in ET
tear dropped shaped RBC and leucoerythroblastic dry aspirate fibrate on trophine biopsy JAK2 or CALR mutation
31
what are the causes of a leukoerythroblastic film
reactive - sepsis marrow infiltration - cancer myelofibrosis
32
treatment of myelofibrosis
transfusion, AB, allogenic SC tranplant, splenectomy | JAK2 inhibitors
33
CLL is the commonest what
leukaemia
34
CLL who
65-75
35
what is CLL
clonal expansion os small lymphocytes
36
symp of CLL
``` asymp recurrent infection anaemia splenomegaly lymphocytes >5x109/L ```
37
what is pancytopenia
anaemia thrombocytopenia neutropenia
38
decreased production
inherited bone marrow failure arise due to to defects in DNA repair/ribosome falcon;s
39
fanconi's symp | what happens in it
short, skin abnormalities, radial ray abnormalities, hypogenitilia, endocrine, GI, haem, renal unable to repair inter stand cross links
40
fanconi's 97% | 52%
BM failure by 20yo | leukaemia by 40yo
41
acquired primary BMF
aplastic anaemia myelodysplastic dysplasia acute leukaemia
42
aplastic anaemia
AI attack against haemopoetic stem cells | hypocellular
43
myelodysplastic dyaslsoa
hypercellular mass ineffective haemopoiesis 20-30% risk of AML
44
acute leukaemia
prevention of normal haemopoiesis
45
secondary BMF
drug - chemo, chloramphenicol alcohol, B12 and folate infiltrative - malignancy, lymphoma, viral (HIV), storage disease
46
increased destruction
hypersplenism | portal htn, CCF, fieltys, splenic lymphoma
47
clinical features of pan
anaemia - fatigue, SOB CDV compromise, neutropenia infections - bleeding symp depending on cause
48
ix for for pan
FBC, film, B12/folate LFTs - hyersplenism HIV/virology AB tests (SLE)
49
test for fanconis
chromosome fragility test
50
hypocellular pan | hypercellular pan
aplastic | MDS, B12/folate, hypersplenism
51
rx for pan
``` malig - chemo congenital - transplan idiopathic aplastic anaemia - immunosuppression supportive transfusions ABs ```
52
normla splenic RC mass RC transit platelets pool
5% fast 20-40%
53
hyposplenic RC mass RC transit platelets pool
40% slow 90%
54
hodgkins symptoms
``` painless cervical LD mediastinum spread - cough hepatospleno B symp pruritus and alcohol related pain ```
55
burkitts translocation
8 and 14
56
burrkitts lymphoma
very high grade
57
mantle cell
similar tp low grade hodgkins but very aggressive and incurable with chemo
58
marginal zone NHL
low grade
59
other types of B cell NHL
follicular diffuse large b cell - commonest world wide lymphplasmocytic lymphoma
60
major haemorrhage protocol what does the blood bank send
4 units of RBC 4 frozen plasma 1 platelets
61
``` ongoing bleeding after transfusion management ix what should be given RBC how cryprecipitate when consider further what ```
repeat blood samples transfuse further RBC:FFP 2:1 (1:1 in trauma) cryoprecipitate if fibrinogen <1 (<2 in obstetric haem) consider further platelets
62
``` management if bleeding stopped after transfusion Hb ATTP and PT platelets fibrinogen use what ```
``` Hb>80 AtPP and PT ration <1.5 platelets >50 fibrinogen >1.5 (>2 in obstetrics) use blood warmer ```
63
when should you assume neutropenic sepsis
if received chemo within past 3 weeks, temp >=38 or clinical evidence of significant sepsis SIRS>=2
64
standard risk px
neutropenia and sepsis and SEWS score <6
65
standard risk px treatment
piperacillin/tazobactam PA teioplanin and aztreonam if anaphylaxis or angiooedema Teicoplanin and ciprofloxacin
66
high risk px
neutropenic and severe septic/septic shock or SEWS >=6
67
high risk px treatment
pipercillin/tazobactem and gentamicin PA - teicoplanin and aztreonam and gent if anaphylaxis or angiooedema teicplanin and ciprofloxacin and gentamicin
68
when should AB be started in neutropenic sepsis
within 1hr | don't wait for blood culture results