Haematology Flashcards
(131 cards)
1
Q
What is DIC
A
coagulation in blood walls → organ ischaemia.
Other blood vessels bleed -> cosumption of clotting factors
2
Q
What organs are affected by DIC ischaemia?
A
-Brain
-Liver
-Lung
-Kidney
3
Q
Clinical signs of DIC
A
-Bleeding
-bruising
-Confusion
-drop in bp
4
Q
Investigations of DIC:
A
Bloods: high D Dimer, Long PT and APTT, low platlets & fibrinogen, schistocytes.
5
Q
How to manage DIC
A
treat cause + tranfuse FFP
6
Q
What is haemochromatosis?
A
Depositation of iron on organs.
7
Q
What is the precursor of haemachromatosis?
A
haemosiderosis- no organ involvement
8
Q
symptoms (organ-involvement)
A
skin->melatonin
hands-> arthritis
liver-> cirrohsis
heart-> cardiomyopathy
pancreas-> diabetes
9
Q
What is the worst organ involvement in haemochromatosis?
A
heart
10
Q
Causes of haemochromatosis?
A
primary-> HFE mutation
Secondary-> transfusion
11
Q
Investigations for haemochromatosis:
A
FBC: Transferrin^ (transporter) , ferritin^ (cells holding iron) ,iron^,low TIBC (binding capacity)
LFTs
Cell microbiology: iron seen in blood
genetic testing: HFE
full body MRI: organ involvement
12
Q
First line tx haemochromatosis
A
Venesection
13
Q
second line tx for haemochromatosis
A
desferrioxamine (iron cheltion therapy)
14
Q
What does heparin inhibit?
A
thrombin and antithrombin
15
Q
Why is LMWH easier? (3)
A
longer halflife
no monitroing
better bioavailability
16
Q
LMWH vs HMWH
A
LM: binds to just thrombin helping it inactivate factor 10
HM: binds to antithrombin and thrombin (potentiating antithrombin)
17
Q
What is heparin used for?
A
actue coag problems (pe/dvt/ surgery)
18
Q
APTT and PT time with heparin
A
increased (factor 10 is in both)
19
Q
Risks of heparin
A
bleeding
osteoporosis
HIT
20
Q
How to reverse heparin
A
Protamine sulfate
21
Q
What is HIT and when does it occur?
A
heparin induced thrombocytopenia?
5-14 days after
22
Q
What is warfarin and what is it given for?
A
anticoag inhibits glycoslyation of vit k so factors 2,7,9,10 inhibited.
chronic coag problems
23
Q
How is warfarin administered vs heparin
A
W: oral
H: IV
24
Q
APTT and PT in warfarin
A
prolonged in both
25
Monitor warfarin?
INR
26
Reverse Warfarin
INR 8+ and bleed: PTC and vit K and stop W
everything else: stop W and vit K
iNR 5-8 no Bleed: withhold W
27
Risks with Warfarin?
tetrogenicity
skin necrosis
interactions
bleeding
28
Protein C depletion with Warfarin
skin necrosis
29
drug interactions with Warfarin
p450 inducers: reduce INR: SCARS: (smoking,alcohol,anti epi, st johns wort)
p450 inhbiitors: increase INR: ASS-ZOLES ( antibiotics, SSRIs, sodium valoporate, -zoles)
30
What is the heparin bridge?
give heparin before warfarin to stop skin necrosis+ warfarin takes time
31
What anti coag should you NOT give to pregnant women?
Warfarin
32
What are DOACS? (stand for)
Direct oral anticoagulants
inhibit factor 2 or 10
33
When is it used?
home therapy
second line to heparin: DVT, arrythmias
33
APTT and PT time in DOACs
prolonged both
34
Risk
Bleeding, hypersenitivity
35
reverse DOACS?
antibodies that bind to them:
Idarucizumab → anibody binds to factor2DOAcs
andexanet alpha → anitbody factor 10 doacs
36
What is polycythaemia?
increased haemtocrit/blood volume (psuedo or true)
37
primary vs secondary polycythaemia?
primary: polycythaemia vera JAK2 mutation
Secondary: increased EPO secreted -> hypoxia, kidney disease, hormone meds(test,steroids)
pseudo: decreased plasma: dehydration
38
What hormones medications increase EPO
Testosterone, steroids
38
signs/symptoms of pv
- plethoric (red face esp nose)
- **itchy after shower**
- headache
- fatigue
- -splenomegly
39
Risks for polcythaemia
thrombosis!!!!
39
polcythaemia is a sign of....
malignancy, JAK2 mut, meds
40
Invest of pv
Bloods: RBC, Haemtocrit, Hb
EPO serum ^
JAK2 mutation screen
40
1st line treatment of polcy
Venesection + aspirin,
41
second line treatment
add hydroxycarbiamide if not working OR high risk of thrombosis
41
How to detect Haem ?
genectic testing
Bloods: APTT prolonged, PT normal, Bleeding time normal, VWF low
42
What is Haemophilia A?
deficiency in factor 8
43
How to treat Haem ?
infuse lost clotting factor, sometimes desmopressin to stimulate VWF (only in A)
43
Signs of Haem ?
sustained bleeding, intramuscular and in joint bleeding, epistaxis.
44
Is mucosal bleeding in Haemophilia?
No
45
What is more common Haem A or B
Haem A
46
How is haemophilia passed down?
x LINKED Recessive
47
What is sustained haemophilia?
clotting factor 8/9 reduced by soemthing else eg DIC, autoimmune
48
What is 50:50 mixing and how does it relate to haem
after 50:50 mix there is NO correction (corrects in VWF)
49
How to confirm Haem A or b
Factor 8 & 9 assay
50
Treat
Desmopresin
50
How is VWD passed down?
Autosomal dominant
50
What is VWF?
deficiency in VWD
50
What deficiency is Haem b?
FACTOR 9 DEFIC
51
Signs of VWD?
Mucosal bleeding + mennorhagia + intramsucualr bleeding
52
Signs of bloods?
Factor 8 activity low, APTT prolonged, BLEEDING TIME LONG, PT normal
53
Is mucosal bleeding seen in VWD, haem or both?
only VWD
54
What is a Coombes/DAT test?
+ve if there is autoantibodies
55
What is Sickle Cell disease?
Inherited condition causing a defect in the beta chain of HbS whereby red blood cells change shape and can occlude blood vessels.
56
Signs of SCD?
- Unprovoked persistent pain
- **Splenomegly**
- anaemia symp (lethargy, pallor)
- bone pain
- high temp
-
57
When does scd present?
Typically 6 months, when HbF starts to decrease
58
What can increase risk of scd crises?
-infection
-dehydration
-hypoxia
-acidosis
-**cold**
58
different types of scd crises (3+1)?
- Sequestration
- Vaso-occlusive -> acute chest syndrome
- Aplastic
-
59
How is SCD passed down?
Autosomal recessive
60
Investi
FBC -> haemolytic anaemia, reticulocytes
blood film-> sickle shape
Chest x ray - acute chest syndrome
confirm: haem electrophoresis (HbF ^ HbS)
61
What is squestration crises + management?
pooling in spleen , sudden drop in haem, signs of shock.
fluid resus, splenectomy?
61
What is acute chest syndrome?
Type of vaso occulsive in the pulmonary vasculature. chest pain, resp symtoms
62
What is aplastic syndrome?
sudden reduction in bone marrow production. Hb drops below 10, no pain + parovirus infection
63
4 types of transfusion reactions?
- Delayed Haemolytic tranfusion reactions
- Acute Haemolytic
- Transfusion-Related Acute Lung Injury (TRALI)
- Autoimmune haemolytic Anaemia (AIHA)
- Transfusion overload
-
63
management for acute + chronic SCD?
acute: IV opiate, fluid, oxygen, antibx IF infection, transfusion
chronic: hydroxycarbidamide/hydroxyurea, Vaccinates
63
What virus is associated with aplastic syndrome?
Parovirus B19
64
splenomegly and massive splenomegly measurements
11-20 cm
>20cm
65
When does delayed Haemolytic reactions occur?
non-prevenatble, 3-10 days after
65
When does acute Haemolytic reactions occur?managemnt?
ABO incompatibilty, IgM antibodies, 24 hours -> STOP transfusion + recus
66
What is TRALI?sign? manag? x ray? treat?
activation in pulmonary vasculature -> increased perm -> pul odemea, fever, hypo. White out on CXR. tx w furosemide
67
What can cuase pancytopenia? (2 need to know + ...)
-B12 or folate def
-methotrexate
-chemo
-cancer
-BM disease
67
What is transfusion associated circulatory overload?
Too much fluid in circulation -> HYPERtension
67
target INR: Valve replace,VTE, AF
INR 2-3: AF, aortic VR, following VTE
2.5-3.5: mitral VR
3.-4: recurrent VTE
68
What do schistocytes mean?
haemolytic anaemia
68
What are reed sternberg cells seen in?
only Hodgkins Lymphoma
69
When are Tear Drop poikilocytes seen?
Myelofibrosis
70
When are howell Jolly bodies seen?
hyposplenism
71
target cells seen in?
SCD, thalassemia, liver disease
72
When are ringed sideroblasts seen?
Myelodysplasia
73
megaloblastic vs non megaloblastic anaemia
meg: b12 or folate defi (diet, malabsorp, pregn, pernious anaemia)
non-meg: alcohol, leauk
73
What are two kinds of anti-platlet therapy?
-P2Y12 inhib
-Aspirin
74
How does aspirin work?
Blocks cox 1+2, no thromboxane A2, no platelet agregg
75
How does clopidogerol work?
PY212 inhib. blocks platelet agregg
76
What is Aplatic Anaemia?
76
What is myelodysplasia?
precancer to CML
77
Investigations +results:
Bloods: pancytopenia, macro/normocytic anaemia, CRP high
BM analysis: ringed sideroblasts, blast cells less than 10%
78
Why does myelodysplasia have blast cells of less than 10%?
BC 10+ = CML
79
Myelodysplasia typical patient
Old w/ infections
80
Mangement of myelodysplasia D+S
direct: chemo
supportive: transfusion + iron chelation therapy
81
What is myelofibrosis?
BM fibrosis -> failure
82
examination and blood signs of myelofibrosis (3)
splenomegaly, pancytopenia, **TEAR DROP POIKILOCYTES
82
BM analysis and aspirate for myelofibrosis
fibroblasts on analysis and dry tap on aspirate.
83
Who is MM most likely in?
Older patients
84
# 1.
signs of MM?
bone pain + FRACTURES
Anaemia
Hypercalcaemia
renal impairment
infections
85
Blood film finding for MM? (1 SPECIAL)
Roleaux formation, pancytopenia, urea and creatinine up
86
BM analysis for MM
Plasma cells 10+%
87
Other tests for MM
U&E
whole body MRI for staging
88
Main treatment for MM?
Stem cell transplant
89
Tumour lysis syndrome tx and biochemical chnages
PUKE CALCIUM
Allopurinol
90
Which leukaemia is seen in kids?
ALL
90
Which leukaemias have massive splenomegly?
CML, CLL
91
BM analysis of CLL?
Hypercellularity
91
Which cancers have lymphadenopathy?
CLL, Lymphomas
91
What is found on blood film in CLL?
Smudge cells
91
CML management?
Tyrosine kinase inhib -> Imitimab
92
Abnormal mutation in CML
BCR-ABL t(9:22)
93
Blood film difference in ALL and AML
AML had aurer rods
93
HL vs NHL
H: Reed-sternberg cells
94
What are cabot rings seen in?
lead poinsoning
95
What blood film is seen with hyposplenism
Howell-Jolly boides and sideroblasts
96
What do you give with methotrexate and why?
Folate - inhibit dihydroxide reductase
97
What nuero issues does vit b12 defi cause
hands and foot pins and needles
98
3 things to give with sickle cell crisis
-iv opiate
-oxygen
-fluids
99
lymphoma that has dysphagia with alcohol
Hodgkins lymphoma
100
1st line tx of VWD
Desmopressin
101
target INR for mitral valve
2.5-3.5
102
target INR for aortic valve
2-3
103
febrile transfusion recation tx?
1st: slow (with minor temp) stop (with major temp) + paracetemol
104
how to diagnose MM + staging
Protein electrophoresis
Whole body MRI
105
lymphoma Ann Arbour staging
(A) (B)- B symptoms
stage 1: one node
stage 2: 2 or more nodes on the sameside
stage 3:nodes on both sides of the diagphrm
stage 4: **organ** involvement far away
106
what blood changes can be seen when you have a transfusion?
Hypocalaemia or Hyperkalaemia
