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haem Flashcards

1
Q

which mutation is present in patients with polycythaemia

A

JAK2 mutation

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2
Q

what is the emperical antibiotic of choice for neutropenic sepsis

A

piperacillin with tazobactam

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3
Q

what treatment is is neutropenic sepsis generally a consequence of

A

chemotherapy

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4
Q

which bacteria generally causes neutropenic sepsis

A

gram positive - staph epidermis

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5
Q

definition of neutropenic sepsis

A

neutrophil count of < 0.5* 10^9 in patient having chemo and -
temp > 38
other signs + symptoms consistent with clinically significant sepsis

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6
Q

classical symptom of polycythaemia vera

A

intense itching when exposed to hot water / hot + humid weather

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7
Q

what are the features of Multiple Myeloma ?

A

CRABBI
Calcium : hypercalcaemia
Renal : renal damage presenting as dehydration and thirst
Anaemia
Bleeding
Bones
Infection

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8
Q

what is the gold standard investigation for Multiple Myeloma ?

A

bone marrow aspiration : confirms diagnosis if the number of plasma cells is raised

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9
Q

what sign is seen on peripheral blood film in multiple myeloma ?

A

Rouleaux formation

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10
Q

name 4 signs of transfusion associated circulatory overload

A

HTN
raised jugular venous pulse
afebrile
S3

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11
Q

give 3 signs of transfusion related acute lung injury

A

hypotension
pyrexia
normal // unchanged JVP

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12
Q

What is the management of DVT in pregnancy

A

subcutaneous low molecular weight heparin

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13
Q

What is the management of DVT in pregnancy

A
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14
Q

why do you treat vit b12 deficiency before starting treatment for folic acid ?

A

to avoid precipitate sub-acute combined degeneration of the cord.

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15
Q

An aplastic crisis may be triggered by ________ in persons with hereditary spherocytosis

A

parvovirus

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16
Q

how does a macrocytic anaemia present on blood film?

A

Hypersegmented neutrophil polymorphs

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17
Q

what is the bacteria that most commonly causes neutropenic sepsis ? How does it present under microscope ?

A

Staph. Epidermis, coagulase negative, gram positive bacteria

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18
Q

which cells of the body does myeloma affect

A

plasma cells

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19
Q

what are plasma cells ?

A

Plasma cells are b lymphocytes that produce antibodies

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20
Q

what is multiple myeloma

A

when myeloma affects multiple bone marrow areas in the body

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21
Q

what is monoclonal gammopathy of undetermined significance

A

it involves the production of a specific para protein ( abnormal antibody) without any other features of myeloma or cancer

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22
Q

what is smouldering myeloma

A

involves both abnormal plasma cells and paraproteins but no organ damage or symptoms

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23
Q

what is a plasmacytoma

A

tumour formed by cancerous plasma cells

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24
Q

what is the gold standard investigation for the diagnosis of sickle cell disease

A

haemoglobin electrophoresis

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25
Q

what is the most common cause of vitamin B12 deficiency ?

A

Pernicious anaemia

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26
Q

what is the typical picture of DIC on blood tests

A

low platelets
increased PT
increased APTT
bleeding time prolonged

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27
Q

what is the management of beta thalassaemia major

A

lifelong iron transfusion

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28
Q

when would you use prothrombin complex concentrate ?

A

emergency reversal of Anti-coagulation in patients with either severe bleeding or head injury with suspected intracerebral haemorrhage.

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29
Q

what is the first line investigation for patients with suspected AML

A

very urgent FBC within 48 hours

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30
Q

what is the management of folate deficiency

A

1 mg of IM hydroxocobalamin 3 times a week for 2 weeks then once every 3 months

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31
Q

how is acute chest syndrome in sickle cell disease managed ?

A

PATO
Pain relief
Abx
Transfusion
O2

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32
Q

what are the positive prognostic factors for ALL

A

age 3-7
female

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33
Q

what are the poor prognostic factors for ALL

A

age <1 or >10
wcc > 100 x 10^9
translocation of chromosomes 9:22 ( Philadelphia)
male

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34
Q

how does acute chest syndrome present and how is it managed?

A

Vaso-occlussion within pulmonary microvasculature causing lung parenchyma infarction.

presents with : dyspnoea, chest pain, pulmonary infiltrates on CXR and low pO2.

management includes pain relief , respiratory support and antibiotics

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35
Q

how does an aplastic crisis present ?

A

Parvovirus B19 Infection
sudden fall in haemoglobin
bone marrow suppression leading to reduced reticulocyte count

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36
Q

how does a sequestration crisis present

A

sickling within spleen or lung causes pooling, worsening the anaemia.

associated with increased reticulocyte count

presenting with abdominal pain, haemodynamic instability and hepato/ splenomegaly

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37
Q

how does a vaso-occlusive crisis present ?

A

painful / vasooculsive crisis is precipitated by infection, dehydration, deoxygenation

clinical diagnosis presenting with acute pain,

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38
Q

what is the key presentation of chronic myeloid leukaemia and how is it managed?

A

increase in granulocytes at different stages of maturation.

managed first line with tyrosine kinase inhibitors such as imanitib

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39
Q

when is a V/Q scan more appropriate to use than CTPA

A

renal impairment

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40
Q

what reversal agent can be used for rivaroxaban and apixaban ?

A

andexanet alfa

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41
Q

what is a non-haemolytic febrile reaction and how does it present ?

A

fevers + chills - manage with slow, stop the transfusion and paracetamol.

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42
Q

how does a minor allergic reaction to blood transfusion present and how is it managed ?

A

pruritis and urticaria

manage by temporarily stopping and giving anti-histamine and monitoring

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43
Q

why can anaphylaxis due to blood transfusion occur?

A

in patients with IgA deficiency who have anti IgA antibodies

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44
Q

why and how does an acute haemolytic reaction present and how it managed ?

A

ABO-incompatible blood e.g. secondary to human error

presenting with -
Fever, abdominal pain, hypotension

management -

stop transfusion
confirm diagnosis and patient
supportive

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45
Q

why and how does TACO present ? how is it managed?

A

excessive transfusion rate causing pulmonary oedema and HTN, managed with stopping transfusion and considering IV Diuretics

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46
Q

why and how does TRALI present ? how is it managed?

A

hypoxia, pulmonary infiltrates on CXR , hypotension, managed by stopping transfusion, O2 and supportive care

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47
Q

what are the risk factors for Burkitt’s lymphoma ? how does it present on microscopy ? how is it managed? what is a risk factor of the management?

A

RF’s - HIV, EBV - v big rf

starry sky on microscopy

chemotherapy - can causes tumour lysis syndrome

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48
Q

what is the mechanism of action of unfractionated heparin ?

A

activates anti thrombin III

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49
Q

how are standard heparin and LMWH monitored

A

standard : APTT
LMWH : anti factor Xa- not recquired routinely

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50
Q

what is the reversal agent for heparin

A

protamine suphate

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51
Q

what is the pathophysiology of pernicious anaemia ?

A

antibodies to intrinsic factor and gastric parietal cells

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52
Q

what are the features of pernicious anaemia

A

anaemia - lethargy, pallor, dyspnoea

neurological features - peripheral neuropathy ( pins and needles)

jaundice- lemon tinge
glossitis

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53
Q

what is the most specific antibody for pernicious anaemia ?

A

anti- intrinsic factor antibodies

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54
Q

what is the management of pernicious anaemia

A

vitamin B12 replacement given IM , 3 injections per week for 2 weeks followed by 3 monthly treatment of vitamin B12 injections.

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55
Q

what is a key malignancy linked with pernicious anaemia

A

gastric cancer

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56
Q

what is the most common of lymphoma in the UK

A

diffuse large b cell

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57
Q

what is heparin induced thrombocytopenia ?

A

Prothrombotic state occurring due to antibodies forming against complexes of platelet 4 and heparin causing a drop in platelets.

managed with direct thrombin inhibitor such as argatroban

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58
Q

what are the key points in the investigation of myeloma?

A

Bloods: FBC and peripheral blood film - rouleaux
U+E showing renal failure

protein electrophoresis : showing raised IgA and IgG , known as Bence Jones proteins in the urine.

bone marrow biopsy - shows raised plasma cells

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59
Q

what imaging is used for mm

A

whole body MRI

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60
Q

what are some complications of blood transfusions

A

febrile reaction
ARDS
iron overload
clotting abnormalities
hyperkalaemia

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61
Q

how do you manage suspected DVT if D-dimer is positive but scan is negative ?

A

stop interim therapeutic anticoagulation and repeat proximal lung vein ultrasound scan 6-8 days later

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62
Q

what is myelofibrosis? what are it’s features? what lab findings are associated with it ?

A

myeloproliferative disorders due to hyperplasia of abnormal megakaryocytes.

elderly person with anaemia and fatigue
massive splenomegaly

lab findings include - high white cell count, tear drop poikilocytes

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63
Q

what anti-platelet regime is used in patients post stroke

A

aspirin 300 mg for 2 weeks then clopidogrel lifelong

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64
Q

what is the mechanism of action of aspirin

A

inhibits production of thromboxane A2

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65
Q

which haemophilia is most common? what is the nature of inheritance?

A

Haemophilia A
X linked

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66
Q

what is the pathophysiology of an acute haemolytic reaction

A

Binding of IgM type antibodies to RBC’s causing haemolysis

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67
Q

what is the mechanism action of fondaparinaux

A

activates antithrombin 3

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68
Q

what is the best test to screen for haemachromatosis ? how are family members tested

A

transferrin solutions, genetic testing for HFE mutation

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69
Q

what is the diagnostic test for polycythaemia vera

A

JAK2 mutations screen

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70
Q

what are the laboratory features of beta-thalassaemia trait

A

mild hypochromic, microcytic anaemia ( disproportionate microcytosis to anaemia)

Hb2 raised

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71
Q

what other investigations are recommended in a patient with suspected PE - other than CTPA

A

chest xray to exclude other pathology
ECG which may show S1Q3R3 and sinus tachycardia

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72
Q

what combination of blood results are most likely to be seen in a patient with sickle cell disease

A

Low Hb
normal MCV
raised reticulocytes

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73
Q

what is the mechanism of action of aspirin ?

A

Non reversible COX 1 and 2 inhibitor

74
Q

what will the ABG most likely show in PE

A

respiratory alkalosis due to hyperventilation

75
Q

do anti-platelets need to be stopped before a dental procedure ?

A

take aspirin as normal

76
Q

what is the diagnostic test for sickle cell disease ?

A

haemoglobin electrophoresis

77
Q

which medication reduces risks of complications and acute crisis in sickle cell patients

A

Hydroxycarbamide

78
Q

what is the vitamin B12 replacement regime?

A

vitamin B12 deficiency IM B12 1mg three times weekly then 1 mg IM every 3 months

79
Q

which gene translocation is burkitt’s lymphoma associated with ?

A

C- myc gene translocation

80
Q

what findings are seen on bone marrow aspirate in multiple myeloma?

A

increased number of plasma cells

81
Q

how long before surgery is warfarin stopped

A

5 days

82
Q

ileo-caecal valve resection can cause what type of anaemia

A

vitamin b12 deficiency

83
Q

what would you be expected to see on blood film in DIC

A

schistocytes

84
Q

what chromosome does beta-thalassaemia major affect ? what are it’s features and management ?

A

affects chromosome 11, presenting in 1st year of life with failure to thrive and hepatosplenomegaly

HbA2 and HbF are raised
HbA is absent

management is with repeated transfusions and iron chelation therapy with deferoxamine

85
Q

what is essential thrombocytosis ? What are it’s features and management ?

A

myeloproliferative disorder, with overproduction of platelets, JAK2 mutation and characteristically a burning sensation in the hands.

managed with hydroxyurea ( hydroxycarbamide) and low dose aspirin

86
Q

what clotting does warfarin cause

A

raised PT, normal APTT

87
Q

what is seen on blood film in autoimmune haemolytic anaemia ?

A

spherocytes and reticulocytes

88
Q

what test can be used to check for auto-immune haemolytic anaemia ?

A

positive direct anti-globulin test - Coomb’s test

89
Q

what are the 2 types of AIHA? what are they caused by ?

A

Warm AIHA - IgG ( ur hot, a real G)
idiopathic, AI, lymphoma, CLL

Cold AIHA - IgM
neoplasia and infections

mx of warm is generally management of the underlying cause, steroids, rituximab

90
Q

how often do patients with SCD require the pneumococcal vaccine ?

A

5 years

91
Q

what is the mechanism of dabigatran

A

direct thrombin inhibitor

92
Q

what biochemical pattern is seen in myeloma on blood film ?

A

High calcium , normal phosphate, normal ALP

93
Q

what is the INR target post PE

A

3.5

94
Q

what is low haptoglobin associated with

A

haemolysis

95
Q

which organisms cause post splenectomy sepsis

A

streptococcus pneumoniae
haemophilus influenzae
meningococci

96
Q

what is the mechanism of action of -

rivaroxaban
dabigatran
warfarin
heparin

A

direct factor Xa inhibitor - rivaroxaban
direct thrombin inhibitor - dabigatran
antithrombin III activator - heparin
inhibition of clotting factor II,VII, IX and X - warfarin

97
Q

what are the guidelines regarding warfarin in patients undergoing emergency surgery ?

A

if surgery can wait 6-8 hours : 5 mg Vitamin K IV
surgery cannot wait : 25-50 units/ kg of four factor PT complex

98
Q

give 4 complications of CLL

A

anaemia
hypogammaglobulinaemia
warm autoimmune haemolytic anaemia
transformation to high grade lymphoma - richter’s transformation

99
Q

what are the features of Richter’s transformation

A

suddenly unwell patient
lymph node swelling
weight loss
night sweats
nausea
abdo pain

100
Q

what type of infection is most likely to occur after platelet transfusion

A

bacterial

101
Q

what are the enzyme inducers? what action do they have on warfarin?

A

SCRAP GP
smokers
carbamazepine
rifampicin
alcohol
phenytoin

griseofulvin
phenobarbital

reduce the action of warfarin

102
Q

what are the enzyme inhibitors? what action do they have on warfarin?

A

SICKFACES.COM
sodium valproate
isoniazid
cimetidine
ketoconazole
fluconazole
alcohol
chloramphenicol
erythromycin
sulphonamides
ciprofloxacin
omeprazole
metronidazole

increase action of warfarin

103
Q

the following cell types are seen in what condition ?

smear/ smudge cells
reed Sternberg cells
Auer rods
howell jolly bodies
heinz bodies
spherocytes

A

smear/ smudge cells - CLL
reed Sternberg cells - Hodgkins lymphoma
Auer rods- AML
howell jolly bodies - decreased spleen function
heinz bodies - G6PD, Alpha thalassaemia
spherocytes - hereditary spherocytosis

104
Q

what medications cause aplastic anaemia ?

A

cytotoxic drugs
chloramphenicol
sulphonamides
phenytoin
gold

105
Q

what are the secondary causes of polycythaemia?

A

COPD
Altitude
obstructive sleep apnoea
excessive erythropoietin

106
Q

which condition can cause hypogonadotrophic hypogonadism

A

haemachromatosis

107
Q

which patients can be managed as an outpatient if they are having a PE ?

A

PESI score class 1, 2

108
Q

management of anti-phospholipid syndrome in pregnancy

A

Low dose aspirin
Low molecular weight heparin once a foetal heart is seen on ultrasound

109
Q

which is the most common inherited bleeding disorder?

A

Von Willebrands disease

110
Q

feature’s of von willebrands disease

A

prolonged bleeding time
APTT can be prolonged

111
Q

haemophilia vs VW

A

von willebrands : increased PT, increased APTT and bleeding

haemophilia : increased APTT ( by a lot more), normal bleeding time

112
Q

Tear drop poikilocytes are present in which condition

A

myelofibrosis

113
Q

picture of CML on bloods

A

significantly raised WCC
predominance of neutrophils, eosinophils and basophils

114
Q

picture of AML on blood film

A

thrombocytopenia - low platelets
neutropenia = decrease in neutrophils

115
Q

general management of a sickle cell crisis

A

analgesia
rehydration
oxygen
antibiotics if infection
blood transfusion/ exchange transfusion

exchange transfusions rapidly reduce the number of HbS containing cells

116
Q

what should be prescribed alongside red cells during transfusion

A

furosemide

117
Q

why are packed red cells transfused with furosemide

A

chronic anaemia and large amounts of fluid will cause cardiovascular compromise

118
Q

when are platelet rich plasma transfused

A

thrombocytopenia and bleeding

119
Q

FFP

A

contains clotting, albumin and immunoglobulin - used in correcting clotting deficiencies

120
Q

cryoprecipitate

A

factor 8 and fibrinogen

121
Q

major indication for cryoprecipitate

A

low fibrinogen

122
Q

indication for FPP

A

high PT - showing deficiencies in factor II, V, VII , X

123
Q

when should the following be given
FFP
Cryoprecipitate
packed red cells
prothrombin complex
platelets

A

FFP : Raised PT indicating issue with clotting
cryoprecipitate : low fibrinogen
Packed red cells : low Hb
PTT : emergency reversal of anticoagulation
platelets : thrombocytopenia

124
Q

first line management of ITP

A

oral prednisolone and pooled normal Human immunoglobulin

125
Q

ITP

A

immune mediated reaction against platelet count with antibodies directed against glycoprotein IIB/IIa

126
Q

hyper segmented neutrophils indicate :

A

megaloblastic anaemia

127
Q

schistocytes are seen in

A

intravascular haemolysis

128
Q

features of thrombotic thrombocytopenic purpura

A

fever
neurological signs
thrombocytopenia
haemolytic anaemia
renal failure

129
Q

pathogenesis of TTP

A

clumping of von willebrands factor causing platelets to clump

130
Q

causes of ttp

A

post infection
pregnancy
drugs - cocp, penicillin, clopidogrel, aciclovir
tumours
SLE
HIV

131
Q

interpretation of well’s score for DVT

A

2 or more - DVT likely - carry out proximal leg vein ultrasound within 4 hours

cannot be carried out in 4 h - interim therapeutic anticoagulation within 4h and D dimer

dvt unlikely - do d dimer in 4 hours, if not intermediate anticoagulation

132
Q

when is a platelet transfusion offered to a patient

A

platelet count < 30 and clinically significant bleeding

platelet count < 10 and no active bleeding / planned invasive procedure

133
Q

ivg for ITP

A

antiplatelet autoantibodies ( IgG)
bone marrow aspiration shows megakaryocytes

134
Q

Mx for ITP

A

oral prednisolone
IV immunoglobulins

135
Q

G6PD inheritance pattern

A

X-linked

136
Q

features of G6PD deficiency

A

neonatal jaundice
intravascular haemolysis
gallstones
Heinz bodies, bite and blister cells

137
Q

drugs causing haemolysis

A

anti-malarials
ciprofloxacin
sulphonamides
sulphasalazine
sulfonylureas

138
Q

difference between hereditary spherocytosis and G6PD

A

HS = extravascular haemolysis and spherocytosis
G6PD = heinz bodies, blister and bite cells, intravascular haemolysis

139
Q

The most common cause of an isolated thrombocytopenia

A

ITP

140
Q

benign ethnic neutropenia

A

common in black/ afro carribean

141
Q

what are the pre surgery/ procedure platelet thresholds

A

> 50 - normal
50-75 - high risk
100 - surgery at critical sites

142
Q

presentation of hereditary spherocytosis

A

failure to thrive
jaundice
gallstones
aplastic crisis

143
Q

signs of lead poisoning

A

abdo pain
peripheral neuropathy
fatigue
constipation
blue lines on gum margin

144
Q

what does FBC show in lead poisoning

A

basophilic stippling

145
Q

mx of lead poisoning

A

dimercaptosuccinic acid

146
Q

medications that increase the risk of VTE

A

COCP
HRT
Raloxifene , tamoxifen
antipsychotics - olanzapine

147
Q

test to differentiate b/w hereditary spherocytosis and G6PD

A

EMA binding test

148
Q

myelodysplastic syndromes

A

ineffective haematopoiesis
peripheral blood cytopenia’s
risk of progression to AML
radiation and chemo increase this risk

149
Q

clinical features of myelodysplastic syndromes

A

fatigue
weakness
pallor due to anaemia
recurrent infections due to neutropenia, easy bruising or bleeding due to thrombocytopenia

150
Q

myelofibrosis

A

anaemia, thrombocytopenia and leukopenia with b symptoms

151
Q

how quickly are red blood cells transfused

A

90-120 mins in a non urgent scenario

152
Q

why are irradiated blood products used

A

to avoid transfusion associated graft vs host disease caused by viable t lymphocytes

153
Q

clinical tumour lysis syndrome

A

laboratory tumour lysis syndrome plus-
increased serum creatinine
cardiac arrythmias
seizures

154
Q

graft vs host disease

A

acute
painful maculopapular rash
jaundice
water or bloody diarrhoea
nausea and vomiting

chronic
skin, eye, GI, and lung manifestations

155
Q

acute vs chronic graft vs host disease

A

acute - within 100 days of transplantation , affecting skin > 80%
chronic - after 100 days

156
Q

transfusion threshold for patients with ACS

A

80 g/l

157
Q

sideroblastic anaemia

A

hypochromic microcytic anaemia, basophilic stippling

158
Q

all REFERRALS

A

A 48 hour blood test is recommended for children and young people with general symptoms or signs of leukaemia, however, when either hepatosplenomegaly or unexplained petichiae are present, the patient should be referred for immediate assessment

159
Q

which lymph nodes does cervical cancer first spread to

A

internal and external iliac lymph nodes

160
Q

deep inguinal lymph node cancers

A

anal and vulval cancer

161
Q

thymoma

A

most common tumour of the anterior mediastinum and associated with myasthenia gravis

162
Q

how does factor V leiden deficiency increase the risk of VTE

A

activated much more slowly by activated protein C

163
Q

blood film in alcoholics

A

macrocytosis and thrombocytopenia

164
Q

platelet transfusion threshold for severe bleeding

A

100

165
Q

most common inherited thrombophilia

A

factor v leiden

166
Q

transfusion most at risk of infection

A

platelet transfusion

167
Q

which drugs can cause a methemoglobinemia

A

sulphonamides, nitrates

168
Q

management of VWF

A

tranexamic acid
desmopressin
factor VIII concentrate

169
Q

what is post thrombotic syndrome

A

painful and heavy calves
pruritis
swelling
venous ulceration
varicose veins

  • it is a complication of DVT
170
Q

mx of prothrombotic state

A

compression stockings

171
Q

how is tranexamic acid given in cases of major haemorrhage

A

IV bolus followed by an infusion

172
Q

diagnosis of G6PD

A

G6PD enzyme assays should be repeated around 3 months after acute haemolytic episodes to avoid false negatives

173
Q

universal donor of FFP

A

AB RhD

174
Q

how to differentiate haemolytic anaemia and hereditary spherocytosis

A

only mild spherocytosis in haemolytic anaemia

175
Q

causes of neutropenia

A

HIV, EBV
Autoimmune
severe sepsis
haemodialysis

176
Q

prevention of VTE in high risk patients

A

Rasburicase

177
Q

what anaemia can prosthetic heart valves cause

A

haemolytic anaemia

178
Q

how to differentiate essential thrombocytopenia and polycythaemia

A

essential thrombocytopenia = isolated thrombocytosis

178
Q

diagnosis of acute intermittent porphyria

A

raised urine porphobilinogen
urine turns deep red on standing

179
Q

management of flares of acute intermittent porphyria

A

IV haem arginate

180
Q

typical DIC picture on bloods

A

low platelets, low fibrinogen, raised APTT , PT and raised D dimer

181
Q
A

SBA passmed Flashcards (30 decks)

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