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MedEd Path > MedEd Haem 2 > Flashcards

MedEd Haem 2 Flashcards

1
Q

preg lady in first trimester has low Hb and high MCV. not taken any multivits / supplements. Dx?

A

folate deficiency

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2
Q

5M has fatigue, SoB, jaundice. father had splenectomy when younger. blood film shows spherocytes. most sensitive Ix?

A

eosin-5-maleimide

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3
Q

18M holiday to malawi where he turned yellow, had brown urine and felt tired. no PMH. took antimalarial prophylaxis.
BT - normocytic anaemia with unconjugated BR. DAT negative.
Dx?

A

G6PD deficiency

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4
Q

which lab finding would you not expect to see in AIHA:
reticulocytes
raised conjugate BR
positive DAT
low Hb
raised LDH

A

raised conjugated BR

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5
Q

confused pt has extreme lethargy, oliguric.
blood film shows schiostocytes
BT shows thrombocytopaenia, rapid rise in urea and creatinine
Dx?

A

TTP

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6
Q

how do you manage TTP

A

plasma exchange

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7
Q

define anaemia

A

reduced ability to deliver oxygen due to lower number of RBCs / decreased Hb

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8
Q

anaemia cut off for men and women

A

men <130
women <120

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9
Q

3 mechanisms for anaemia

A

blood loss
decreased RBC production
increased RBC destruction

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10
Q

DDx of microcytic anaemia

A

iron def
thalassaemia
sideroblastic anaemia

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11
Q

key Ix for microcytic anaemia

A

peripheral blood smear
iron studies

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12
Q

commonest cause of iron def anaemia

A

blood loss

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13
Q

buzzword of iron def anaemia on blood film

A

pencil cells

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14
Q

iron study results in iron def anaemia

A

low iron
low ferritin
high transferrin
high TIBC

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15
Q

buzzword of FBC for iron deficiency

A

reactive thrombocytosis

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16
Q

what is transferrin

A

molecule that binds iron

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17
Q

what is TIBC

A

directly proportional to transferrin

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18
Q

mx of iron def anaemia

A

investigate underlying cause
iron supplements

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19
Q

blood film buzzword of thalassaemia

A

basophilic stippling (purple spots - ribosomal material)
target cells

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20
Q

iron studies of thalassaemia

A

all normal

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21
Q

causes of target cells

A

thalassaemia
hyposplenism
hepatic failure
haemaglobinopathies

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22
Q

mx of thalassaemia

A

iron supplementation
regular transfusions
iron chelation

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23
Q

iron studies of anaemia of chronic disease

A

low Hb
low iron
normal or low TIBC / transferrin
normal transferrin saturation
normal or high ferritin

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24
Q

why can ferritin be high in anaemia of chronic disease

A

its an acute phase protein so high in the acute phases of chronic disease

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25
Q

what is ferritin

A

iron storage molecule

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26
Q

what is sideroblastic anaemia

A

have lots of iron in the body but you can’t incorporate it into haemaglobin - end up with iron ringed around cells

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27
Q

blood film buzzword of sideroblastic anaemia

A

basophilic stippling (purple dots)

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28
Q

iron studies of sideroblastic anaemia

A

high iron ** key one
high / normal ferritin (differentiates between iron def and this)
low transferrin / TIBC

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29
Q

BM biopsy of sideroblastic anaemia (buzzword)

A

ringed sideroblasts

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30
Q

mx of sideroblastic anaemia

A

treat underlying cause
regular transfusions

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31
Q

causes of sideroblastic anaemia

A

congenital
lead poisoning
high alcohol intake

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32
Q

ddx of macrocytic anaemia

A

megaloblastic anaemia - vit b12 / folate def
alcohol
hypothyroidism
pregnancy

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33
Q

key Ixs for macrocytic anaemia

A

peripheral blood film
LFTs
TFTs

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34
Q

how does alcohol cause macrocytic anaemia

A

too much deposition of cholesterol around RBCs causes increased size of cells

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35
Q

how does hypothyroidism cause macrocytic anaemia

A

low T4
T4 also stimulates production of erythropoietin, so less erythropoeitin = less RBC production and bigger

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36
Q

what foods have high b12 vs folate

A

b12 = eggs, meat, fish
folate = leafy green veg

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37
Q

key test to differentiate b12 from folate deficiency and result

A

serum methylmalonic acid (high in b12)

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38
Q

how does the duration of sx vary between b12 and folate def

A

folate def = months
b12 def = years

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39
Q

key sx difference between b12 and folate def

A

b12 def associated with neurological changes

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40
Q

what test can be used to determine cause of b12 def and why

A

schilling test - positive if b12 def due to pernicious anaemia
(outdated test)

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41
Q

what drug in the histoy would indicate folate def and why

A

phenytoin - inhibits folate absorption

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42
Q

mx of b12 / folate def

A

vitamin supplementation

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43
Q

LFT results of alcoholism causing macrocytic anaemia

A

AST more than double ALT
raised AST, ALT and GGT

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44
Q

key sx to identify hypothyroidism

A

thinning of outer 1/3rd of eyebrows

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45
Q

ddx of normocytic anaemia

A

haemolytic
- inherited
- acquired: immune / non immune mediated
non haemolytic
- anaemia of chronic disease
- failure of erythropoeisis

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46
Q

Ix for normocytic anaemia

A

peripheral blood smear
DAT
CRP, ESR

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47
Q

causes of anaemia of chronic disease

A

infection
inflammation
cancer

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48
Q

why does chronic disease cause anaemia

A

lots of cytokine and free radical production
molecule called hepcidin produced
hepcidin inhibits erythropoiesis
less RBCs produced –> anaemia

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49
Q

iron studies of anaemia of chronic disease

A

high iron
high ferritin **key one
low transferrin and TIBC

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50
Q

mx of anaemia of chronic disease

A

tx underlying cause

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51
Q

causes of inherited haemolytic anaemia

A

membrane: hereditary spherocytosis
cytoplasm / enzymes: G6PD def
haemoglobin

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52
Q

causes of acquired haemolytic anaemia

A

immune
- AI: warm vs cold HA
- alloimmune: ABO / RhD incompatibility
non immune
- microangiopathic vs macroangiopathic
- infection

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53
Q

causes of intravascular haemolysis

A

alloimmune - ABO / RhD
autoimmune
hereditary spherocytosis

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54
Q

causes of extravascular haemolysis

A

malaria
G6PD def
drugs
PNH - paroxysmal noctural haemaglobinuria

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55
Q

PC of paroxysmal nocturnal haemaglobinuria

A

haemaglobinuria in the morning
low haptoglobin
high LDH

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56
Q

what causes hereditary spherocytosis

A

defect in vertical interaction of RBC membrane

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57
Q

most sensitive test for hereditary spherocytosis

A

eosin-5-maleimide

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58
Q

what other test would be + in hereditary spherocytosis

A

osmotic fragility test

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59
Q

blood smear of hereditary spherocytosis

A

spherocytes
polychromasia

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60
Q

mx of hereditary spherocytosis

A

folate supplement
splenectomy

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61
Q

inheritance pattern of hereditary spherocytosis

A

AD

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62
Q

inheritance of G6PD

A

X linked recessive

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63
Q

PX of g6pd def

A

episodes of acute haemolysis after an exposure to oxidative stress

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64
Q

triggers of g6pd flares

A

fava beans
mothballs
drugs - anti malarials (primiquine), doxycycline, ciprafloxacin

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65
Q

buzzwords of blood film of g6pd def

A

heinz bodies
bite cells

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66
Q

BR and haptoglobin levels in g6pd def

A

high unconjugated BR
low haptoglobin

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67
Q

mx of g6pd def

A

avoidance of triggers
supportive care

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68
Q

pathophysiology of g6pd def causing haemolysis

A

g6pd is used in pentose phosphate pathway
generates NADPH
used to make glutathione
glutathione prevents RBCs being damaged by oxidative stress
–> therefore g6pd def = vulnerable to damage by oxidative stress

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69
Q

what test is positive in AIHA

A

DAT

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70
Q

difference in mediator in warm / cold AIHA

A

warm = IgG
cold = IgM

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71
Q

association of warm AIHA

A

CLL (** key), SLE, methyldopa

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72
Q

association of cold AIHA

A

mycoplasma, EBV, hep C

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73
Q

how is the haemolysis different in warm and cold AIHA

A

warm = extravascular
cold = intravascular

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74
Q

mx of AIHA

A

treat underlying cause
steroids
rituximab ** key one

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75
Q

which type of AIHA is more severe

A

warm

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76
Q

DAT result of non AIHA

A

negative - no ABs produced

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77
Q

causes of non AIHA

A

infection eg malaria
structural issue –> MAHA

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78
Q

what is mechanism of MAHA

A

damage to endothelial surface –> fibrin deposition –> platelet aggregation –> fragmentation of RBCs

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79
Q

buzzword blood film features of MAHA

A

schistocytes
thrombocytopaenia

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80
Q

3 conditions that can cause MAHA

A

HUS
TTP
DIC

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81
Q

how can you distinguish DIC from HUS/TTP

A

HUS / TTP = normal APTT, PT, fibrinogen
DIC would have really abnormal levels

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82
Q

bacterial cause of HUS

A

e.coli O157:H7

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83
Q

who gets HUS

A

children
adults can get it but its less common and more severe

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84
Q

PC of HUS

A

sx after diarrhoeal illness
triad: MAHA, thrombocytopaenia, AKI

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85
Q

mx of HUS

A

supportive - self limiting in kids
do not give ABx to treat the diarrhoeal illness

86
Q

cause of TTP

A

deficiency of ADAMTS13 (inherited or acquired)
**buzzword

87
Q

PC of TTP

A

pentad
- MAHA
- thrombocytopaenia
- AKI
- neuro sx
- fever

88
Q

mx of TTP

A

supportive care
plasma exchange

89
Q

what is DIC

A

MEDICAL EMERGENCY
activation of coagulation and fibrinolysis

90
Q

triggers of DIC

A

sepsis (most common)
trauma - head injury, fat embolism
obs - amniotic fluid emboli, placental abruption
malignancy - APML

91
Q

BT results of DIC

A

low platelets
low fibrinogen
high PT and APTT
high D dimer

92
Q

PC of DIC

A

bleeding from mouth / lines
massive bleeding from anywhere

93
Q

clotting studies for DIC

A

prolonged APTT, PT, TT
decreased fibrinogen & platelets
increased FDP
film - schistocytes

94
Q

what does the DAT test show

A

AI mediated haemolysis

95
Q

what does urinary haemosiderin / Hb show

A

intravascular haemolysis

96
Q

what does + osmotic fragility test show

A

hereditary spherocytosis

97
Q

what does PK deficiency cause

A

chronic haemolytic anaemia (in contrast to G6PD which is episodic)

98
Q

what does heinz bodies stain suggest

A

oxidative haemolysis

99
Q

what is hams test for

A

paroxysmal nocturnal haemaglobinuria

100
Q

which is true about alpha thalassaemia:
caused by defect on chr 11
increased destruction of beta globin chains
decreased gamma globin chains
4 mutated genes can lead to hydrops fetalis
increased prod of alpha globin chains

A

4 mutated genes can lead to hydrops fatalis

101
Q

XR features of beta thal major

A

hair on end - looks like spikey hair on top of bone, rathe than smooth

102
Q

best Ix to diagnose beta thal

A

high performance liquid chromatography

103
Q

3 types of hb with make up of each and who has each

A

HbF (a2g2) - foetus, infant
HbA (a2b2) - late foetus, infant, child, adult [default one]
HbA2 (a2d2) - infant, child, adult

104
Q

dx of haemglobinopathies

A

hb electrophoresis (high performance liquid chromatography)

105
Q

who gets more HbA2 and why

A

beta thalassaemia pts - they can’t make the beta chains seen in HbA

106
Q

what chromosome is affected in beta thal

A

11

107
Q

key features of the 3 types of beta thal

A

major - severe anaemia needinf regular blood transfusions
intermedia - mod reduction in beta globin chain prod
minor - benign but genetic implications

108
Q

dx of beta thal

A

high performance liquid chromatography

109
Q

mx of beta thal

A

regular blood transfusions
iron chelation
folate supplementation

110
Q

what chromosome is affected in alpha thal

A

16

111
Q

how many genes code for beta vs alpha thal

A

beta = 2
alpha = 4

112
Q

give the name for 4 / 3 / 2 / 1 alpha genes affected in alpha thalassaemia

A

4 = Hb Barts
3 = HbH
2 = trait
1 = silent

113
Q

features of the 4 types of alpha thalassaemia

A

Hb Barts = fatal in utero, hyrops fetalis
HbH = severe anaemia in childhood, hepatosplenomegaly
trait = mild anaemia
silent = aSx

114
Q

mx of alpha thalassaemia

A

regular blood transfusions
iron chelation
folate supplementation

115
Q

inheritance of sickle cell

A

AR

116
Q

pathophysiology of sickle cell (mutation, location, outcome)

A

glutamine –> valine mutation at codon 6 on beta globin chain
produces HbS

117
Q

forms of sickle cell Hb produced

A

HbSS
HbAS
HbSC
HbSbeta

118
Q

PC of sickle cell disease

A

haemolytic crisis
sequestration crisis
aplastic crisis
infection

119
Q

2 key buzzword infections that occur in sickle cell disease pts

A

strep pneumo sepsis
salmonella osteomyelitis

120
Q

what ix is used in sickle cell disease and in which types is it +

A

sickle solubuility test
HbSS
HbAS

121
Q

mx of sickle cell disease

A

vaccination
folate supplementation
hydroxyurea
supportive in acute crisis

122
Q

19M with acutely swollen knee. PMH bleeding after dental extraction. what factor is most likely to be deficient?

A

factor 8

123
Q

what is the most common inherited thrombophilia

A

hereditary activated protein c resistance (factor V leiden)

124
Q

list disorders of primary and secondary haemostasis

A

primary - platelets adhesion / aggregation
secondary - coagulation cascade

125
Q

inherited disorders of thrombosis

A

factor V leiden (most common)
anti thrombin deficiency
protein C / S deficiency

126
Q

acquired disorders of thrombosis

A

HIT
malignancy
immobilisation

127
Q

what do dysfunction of primary vs secondary haemostasis cause

A

primary = bleeding disorders (superficial bleeding)
secondary = coagulation disorders (deep bleeding)

128
Q

causes of dysfunctional primary haemostasis

A

vWF disease
ITP / HIT

129
Q

causes of secondary haemostasis dysfunction

A

inherited - haemophilia A / B
acquired - liver disease, vit K def

130
Q

defect in haemophilia A vs B

A

defect in factor 8 (A) vs 9 (B)

131
Q

what is the problem with platelets in ITP

A

decreased survival of platelets

132
Q

what is the problem with platelets in DIC

A

increased consumption

133
Q

what can cause acquired defective platelet function

A

aspirin
CKD

134
Q

what can cause inherited defective platelet function

A

thrombasthenia

135
Q

3 subtypes of vWF disease and what they are

A

type 1 = quantitative defect
type 2 = qualitative defect
type 3 = mixed defect

136
Q

inheritance of the types of vWF disease

A

type 1 and 2 = AD
type 3 = AR

137
Q

PC of vWF disease

A

mucocutaenous bleeding
usually female

138
Q

bloods of vWF disease

A

low platelets
increased bleeding time
increased APTT
normal PT
low factor 8

139
Q

2 DDx of vWF and how you can distinguish them

A

bernard-soulier disease (large platelets)
glanzmann’s throbasthenia (normal ristocetin)
[low yield]

140
Q

what protein is abnormal in vWF

A

ristocetin

141
Q

mx of vWF disease

A

desmopressin
vWF and factor 8 concentrates

142
Q

who gets acute ITP

A

children, sex even

143
Q

who gets chronic ITP

A

adults, females >males

144
Q

trigger of acute vs chronic ITP

A

acute = infection
chronic = no trigger

145
Q

mx of acute ITP

A

self limiting
tx steroids, IVIG if platelets count really low

146
Q

mx of chronic ITP

A

steroids
IVIG
splenectomy

147
Q

progression of chronic ITP

A

long term relapsing remitting

148
Q

inheritance of haem A

A

x linked recessive

149
Q

pc of ham A

A

haemarthrosis (bleeding into joints)
spontaneous, deep bleeding

150
Q

blood results of haem A

A

normal platelet count, bleeding time, PT
prolonged APTT

151
Q

which haemophilia is more common

A

A

152
Q

mx of haem A

A

factor 8 concentrate - can become resistant to this eventually though

153
Q

how can you tell haem A from B clinically

A

you can’t

154
Q

what is vit k used for

A

synthesis of factors 2, 9, 10 and protein C/S

155
Q

causes of vit k def

A

malabsorption
warfarin
ABx

156
Q

which factor is first to be depleted in vit k def

A

factor 7
** key exam Q

157
Q

blood results of vit k def

A

normal platelets, bleeding time
prolonged APTT and PT

158
Q

ddx of vit k def and how to tell them apart

A

liver disease (low platelets)
scurvy (corkscrew hair) **buzzword

159
Q

mx of vit k def

A

vit k replacement
PCC
FFP

160
Q

inheritance of factor V leiden

A

AD

161
Q

pathophysiology of factor V leiden

A

resistance to protein C –> failure to degrade factor V –> hypercoagulable state

162
Q

what are factor V leiden pts predisposed to

A

VTE (arterial rare)

163
Q

mx of factor v leiden

A

long term anticoagulation

164
Q

inheritance of anti thrombin def

A

AD

165
Q

2 key features of anti thrombin def
**buzzwords

A

highest risk of thrombosis
develop thrombi in unusual places eg splenic / mesenteric arteries

166
Q

dx of anti thrombin def

A

anti thrombin assay

167
Q

ddx of anti thrombin def

A

protein C/S def

168
Q

mx of anti thrombin def

A

long term anti coag - warfarin / argatroban

169
Q

inheritance of protein c / s def

A

AD

170
Q

what are you predisposed to with protein c/s def

A

VTE (arterial rare)

171
Q

buzzword association of protein c/s def

A

warfarin induced skin necrosis

172
Q

dx of protein c /s def

A

protein c / s assay

173
Q

mx of protein c/s def

A

long term anti coag - argatroban

174
Q

35F 34 weeks preg has swollen L calf/ preferred anticoag?

A

LMWH

175
Q

which of the following in low in preg
- fibrinogen
- factor 7
- protein S
- plasminogen activator inhibitor 1

A

protein S

176
Q

pathophysiology of anaemia in preg

A

volume expansion –> increased cardiac output and dilutional anaemia

177
Q

what happens to platelet count in preg

A

thrombocytopaenia

178
Q

what is HDN

A

prior sensitisation of Rh- women from prev preg

179
Q

mediation of HDN

A

IgG

180
Q

complications of HDN

A

foetal anaemia
hydrops fetalis
neonatal jaundice –> kernicterus

181
Q

how is HDN monitored for

A

MCA doppler USS

182
Q

mx of HDN

A

prevent sensitisation with anti D
IU transfusion

183
Q

features of HELLP syndrome

A

anaemia
elevated LFTs
low platelets

184
Q

blood test features of HELLP

A

MAHA
raised AST / ALT
low platelets
normal APPT, PT

185
Q

mx of HELLP

A

supportive
delivery of foetus

186
Q

15M with beta thal major has blood transfusion. feels feverish and gets chills shortly after starting. normal obs inc temp. Dx?

A

non haemolytic transfusion reaction

187
Q

40M has blood transfusion. within 10 mins, gets fevers, rigors, abdo pain. tachycardia, low BP, fever. Dx?

A

acute haemolytic transfusion reaction

188
Q

63M SoB 2 hrs post transfusion. high RR, low BP, low o2 sats, high temp.
O/E bilateral inspiratory creps. Dx?

A

transfusion related acute lung injury

189
Q

give the timeline of transfusion reactions

A

immediate - anaphylaxis
mins to hours - bacterial contamination, ABO incompatability, febrile non haemolytic TR
hours to days - TRALI, transfusion assoicated circulatory overload
days - GvHD, delayed hamolytic transfusion reaction

190
Q

who is more likely to have anaphylaxis after blood transfusion
**buzzword

A

IgA deficient people

191
Q

what mediates ABO incompatibility **buzzword

A

IgM

192
Q

with what type of transfusion does bacterial contamination occur most commonly **buzzword

A

platelet transfusion

193
Q

what is febrile non haemolytic TR

A

rise in temp <1 degree without circulatory collapse

194
Q

cause of febrile non haemolytic TR

A

release of cytokines by leucocytes

195
Q

how can febrile non haemolytic TR be prevented

A

leucodepletion

196
Q

sx of transfusion assoicated circulatory overload

A

pulmonary oedema or fluid overload sx over hours

197
Q

3 signs seen in transfusion associated circulatory overload (TACO)

A

raised JVP
high PCWP
high BP
(heart failure signs)

198
Q

sx of TRALI

A

similar to TACO - fluid overload / pulm oedema sx

199
Q

cause of TRALI

A

interaction with HLA ABs in donor blood with recipient

200
Q

when does TRALI occur

A

<6 hours post transfusion

201
Q

key buzzword feature of TRALI

A

absence of heart failure

202
Q

TRALI / TACO CXR

A

bilateral pulmonary infiltrates

203
Q

mx of TACO

A

diuretics - furosemide

204
Q

mediator of delayed haemolytic transfusion reaction

A

IgG

205
Q

when does delayed haemolytic TR occur

A

within 1 week

206
Q

pc of delayed haemolytic TR

A

jaundice / splenomegaly few days after transfusion

207
Q

cause of sx in delayed haemolytic TR

A

extravascular haemolysis

208
Q

sx of GvHD

A

diarrhoea, liver failure
** key one: skin desquamation
BM failure

209
Q

pathophysiology of GvHD

A

donor lymphocytes recognsie recipient HLA as foreign
attack gut / liver / skin / BM

210
Q

prevention of GvHD

A

irradiating blood components for immunosuppressed recipients

MedEd Path (10 decks)

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