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Phase 4 > Haematology > Flashcards

Haematology Flashcards

1
Q

What is acute lymphoblastic leukaemia?

A

malignant clonal proliferation of lymphoblastic cells (most commonly B cells)

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2
Q

What is the most common childhood cancer?

A

ALL

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3
Q

what age range is ALL most common in ?

A

<20 => children

bimodal age peaks - 4-5 year and 50+

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4
Q

What are 4 risk factors for ALL?

A

Age (child, 50+)
genetics
Hx of malignancy
Down’s (AML more common)

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5
Q

what is the pathophysiology of ALL?

A

Genetic mutations => uncontrolled proliferation and clonal expansion of lymphoid progenitor cells => leukemic blasts infiltrate the bone marrow and other organs, which disrupts their normal function.
CNS involvement common

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6
Q

What are 6 manifestations of ALL?

A 
lymphadenopathy 
hepatosplenomegally
fever, fatigue, pallor
dizziness, palpitations, SOB
bleeding - anaemia, infection, epistaxis, bruising, petechia
bone/abdo pain
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7
Q

what are 3 investigations for ALL?

A

FBC with differential - anaemia, leukocytosis, neutropenia, thrombocytopenia

Peripheral blood smear - leukaemia lymphoblasts

immunophenotyping

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8
Q

what is the gold standard investigation for ALL/AML?

A

bone marrow aspiration and biopsy

>20% lymphoblasts/myeloblasts in bone marrow is diagnostic

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9
Q

what are 3 differentials for Leukaemias?

A

meningococcal septicaemia
idiopathic thrombocytopenia purpura
vasculitis

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10
Q

what is the treatment for ALL?

A

Chemo - vincristine + corticosteroids
imatinib - if Philadelphia +ve
mercaptopurine + methotrexate => 2 years maintenance

bone marrow transplant

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11
Q

What are 3 complications of ALL?

A

stunted growth and development in children
tumour lysis syndrome
infertility

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12
Q

What is acute myeloid leukaemia?

A

the clonal expansion of myeloid blasts in the bone marrow, peripheral blood, or extramedullary tissues.

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13
Q

what age group is AML more common in?

A

anywhere about 45 - most common 65+

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14
Q

what are 5 risk factors for AML?

A 
65+
previous chemo/radiation
Down's syndrome
benzene - painters, petroleum, rubber
myelodysplastic/proliferative syndromes
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15
Q

what is the pathophysiology of AML?

A

Accumulation of myeloid blasts unable to differentiate into mature neutrophils, RBCs or platelets resulting in bone marrow failure

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16
Q

what are 6 presentations of AML?

A 
pallor 
bruising (ecchymoses + petechia) and bleeding
infections 
lymphadenopathy 
hepatosplenomegaly 
fatigue, dizziness, palpitations
bone/abdo pain
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17
Q

what are 3 investigations of AML?

A

FBC with differential - anaemia, macrocytosis, leukocytosis, neutropenia, thrombocytopenia

peripheral blood smear - blasts, Auer rods

coagulation pannel - may be normal, if abnormal suspect DIC

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18
Q

what is the treatment of AML?

A

Chemo - cytarabine and an anthracycline (tretinoin)
All-trans retinoid acid

stem cell transplant

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19
Q

what are 3 complications of AML?

A

tumour lysis syndrome
Disseminated intravascular coagulation (DIC)
infection

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20
Q

what is the prognosis for AML?

A

high incidence of relapse

5 year survival - 25%

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21
Q

what is reticulocyte count?

A

measure of immature RBCs

shows if production or removal is cause of anaemia

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22
Q

What are 5 causes of microcytic anaemia?

A

TAILS

Thalassaemia 
Anaemia of chronic disease
Iron deficiency
Lead Poisoning 
Sideroblastic anaemia
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23
Q

what are 5 causes of normocytic anaemia?

A

AAAHH

Acute blood loss
Anaemia of chronic disease
Aplastic anaemia - CKD, bone marrow suppression
Haemolytic anaemia
Hypothyroidism
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24
Q

what are 7 causes of macrocytic anaemias?

A

FAT RBC

Foetus - pregnancy
Alcohol - normoblastic
Thyroid disease - hypo

Reticulocytosis - haemolytic anaemia/blood loss
B12 and Folate deficiency - megaloblastic
Cirrhosis and liver disease

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25
Q

what are 5 symptoms of anaemia?

A

often asymptomatic

fatigue/faintness
headache, confusion 
SOB
angina/palpitations/claudication
Pica - abnormal cravings
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26
Q

where is B12 found?

A 
Meat 
fish 
dairy
eggs 
NO PLANTS
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27
Q

how long do B12 stores last?

A

4 years

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28
Q

what are 3 causes of B12 deficiency anaemia?

A

diet
malabsorption
pernicious anaemia (most common)

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29
Q

How is B12 absorbed?

A

combines with intrinsic factor secreted from parietal cells in stomach and absorbed in terminal ileum

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30
Q

why is B12 necessary?

A

needed for DNA and thymine synthesis

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31
Q

what is pernicious anaemia?

A

autoimmune destruction of parietal cells leading to B12 deficiency anaemia

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32
Q

what are 3 risk factors for B12 anaemia?

A

vegan
history of GI surgery
H.pylori infection

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33
Q

What are 4 manifestations of B12 anaemia?

A

insidious onset of anaemia symptoms
decreased vibration sense
glossitis
psychiatric problems/cognitive impairment

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34
Q

what are 3 differentials for B12 anaemia?

A

B9 deficiency
myelodysplatic syndrome
alcoholic liver disease

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35
Q

what is the treatment for B12 anaemia?

A

oral cyanocobalamin
IV hydroxocobalamin

FOLIC ACID

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36
Q

what are 3 complications of B12 anaemia?

A

neurological deficits
haematological deficits
gastric cancer

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37
Q

What is chronic lymphocytic leukaemia?

A

a malignant monoclonal proliferation of B lymphocytes

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38
Q

what is the most common leukaemia?

A

CLL

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39
Q

what are 3 risk factors for CLL?

A

70+
male
FHx

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40
Q

what are 6 manifestations of CLL?

A

often asymptomatic

SOB + fatigue 
lymphadenopathy
hepatosplenomegally 
B symptoms 
recurrent infections
easy bruising and bleeding
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41
Q

what are B symptoms?

A 
fever
chills
night sweats
weight loss
fatigue
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42
Q

What are 4 investigations for CLL?

A

FBC - lymphocytosis
Blood film - smudge cells, spherocytes and polychromasia
immunophenotyping

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43
Q

what are 3 treatment options for CLL?

A

monitoring - early stage
chemo - FCR, chlorambucil and rituxibam

stem cell transplant

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44
Q

what are 3 complications of CLL?

A 
Richter transformation (to non-hodgkins lymphoma)
tumour lysis syndrome
autoimmune haemolytic anaemia
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45
Q

what is the rule of 3s in CLL?

A

1/3 don’t progress
1/3 progress slowly
1/3 progress actively

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46
Q

what is chronic myeloid leukaemia?

A

the malignant proliferation of partially mature myeloid cells (especially granulocytes) in bone marrow and blood

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47
Q

what gene is related to CML??

A

the Philadelphia gene

BCR-ABL fusion gene

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48
Q

what leukaemia is the Philadelphia gene related to? and what percentage have it?

A

CML

80%

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49
Q

when is the peak incidence of CML?

A

65-75

almost exclusively in adults

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50
Q

what is a known risk factor for CML?

A

ionising radiation

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51
Q

what are 7 presentations of CML?

A 
hepatosplenomegaly
SOB 
easy bruising and bleeding - epistaxis 
bone pain - sternum 
pallor and pyrexia 
recurrent infections 
B symptoms

also may be symptoms of gout due to purine breakdown

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52
Q

what are 3 investigations for CML?

A

FBC - elevates WBCs, anaemia, normal platelets, thrombocytosis/thrombocytopenia

peripheral blood smear - mature/maturing myeloid cells, elevated basophils and eosinophils

bone marrow biopsy - granulocytic hyperplasia

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53
Q

what is the gold standard for CML?

A

presence of Philadelphia chromosome - cytogenetics/FISH

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54
Q

what is the treatment for CML?

A

tyrosine kinase inhibitors - imatinib!!

chemo + stem cell transplant if refractory

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55
Q

what are 3 complications of CML?

A

Gout
Tumour lysis syndrome
blast crisis

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56
Q

what is the prognosis for CML?

A

75% 5 year survival

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57
Q

what is Virchow’s triad?

A

blood constituents
blood flow
endothelial injury

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58
Q

What are 5 risk factors for DVT?

A 
bedridden
airplane 
active cancer
pregnancy 
trauma/surgery
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59
Q

where are DVTs most likely to embolism to lungs from?

A

ABOVE knee

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60
Q

what are 5 manifestations of DVT?

A 
calf swelling (unilateral)
localised deep leg pain
redness and warmth/coolness
asymmetrical oedema 
cyanosis
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61
Q

what are 2 blood tests for DVT?

A

D dimmer - elevated (tests for protein fragments in blood)

FBC/U+E/LFTs

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62
Q

what is the gold standard for DVT?

A

venous ultrasound - duplex ultrasonography

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63
Q

what are 3 differentials for DVT?

A

cellulitis
calf muscle/achillies tear
calf muscle haematoma

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64
Q

what is he treatment for DVT?

A

1st - apixiban (no renal probs)
OR LMWH and warfarin

physical activity
compression stocking

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65
Q

what are 3 complications of DVT?

A

pulmonary embolism
anticoagulant induced bleeding
embolic stroke

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66
Q

what is folate (B9) present in?

A 
green vegetables
legumes
some fruits
yeast
liver 
nuts
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67
Q

how long do folate stores last?

A

4 months

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68
Q

where in folate absorbed?

A

proximal jejunum/duodenum

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69
Q

what are 4 risk factors for folate deficiency?

A

elderly/young
poverty
chron’s/coeliac
pregnant

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70
Q

what is folate needed for?

A

DNA synthesis and repair

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71
Q

what are 5 manifestations of folate deficiency?

A

may be asymptomatic

anaemia symptoms 
tachycardia 
signs of heart failure
glossitis/painful swallow
prolonged diarrhoea
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72
Q

What is the management of folate deficiency anaemia?

A

oral folic acid + B12 ALWAYS

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73
Q

what are 3 differentials of folate deficiency?

A

B12 deficiency
hypothyroidism
alcoholic liver disease

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74
Q

what are 2 complications of folate deficiency?

A 
pregnancy complications (neural tube defects, spina bifida)
heart conditions
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75
Q

what is G6PD?

A

X-linked condition causing haemolytic anaemia due to susceptibility to free radicals
common in Kurdish jews and sub-saharan Africa

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76
Q

what are 5 causes of haemolytic anaemia?

A 
RBC membrane defects (spherocytosis)
enzyme defects (G6PD)
haemoglobinopathies (thalassaemia, sickle cell)
autoimmune 
infection/trauma
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77
Q

what are 4 risk factors for haemolytic anaemia?

A

autoimmune disorders
FHx
drugs
prosthetic heart valves

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78
Q

what are 4 manifestations of haemolytic anaemia?

A

pallor, fatigue, dizzy, SOB
jaundice
dark urine
splenomegaly

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79
Q

what is the gold standard for haemolytic anaemia?

A 
Coombs test (direct antiglobulin test - for autoimmune 
peripheral smear
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80
Q

what are 3 differentials for haemolytic anaemia?

A

blood loss anaemia
underproduction anaemia
transfusion reaction

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81
Q

what is the treatment for haemolytic anaemia?

A 
corticosteroids 
stop causative drugs 
splenectomy 
plasma exchange
anticoagulation 
avoidance of triggers
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82
Q

What is Hodgkin’s lymphoma?

A

Uncommon haematological malignancy arising from mature B cells (lymphocytes)
Characterised by the presence of Hodgkin’s cells and Reed-Sternberg cells.

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83
Q

what are 4 risk factors for Hodgkin’s lymphoma?

A

EBV infection
FHx
Bimodal age - 15-35 and 60+
autoimmune conditions

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84
Q

what are 7 manifestations of Hodgkin’s lymphoma?

A 
lymphadenopathy
hepatosplenomegally (less common than in NHL)
B symptoms 
dyspnoea 
Pel-Ebstein fever - intermittent
pruritus
Alcohol induced lymph node pain
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85
Q

what are 3 investigations for Hodgkin’s lymphoma?

A

FBC with differential - low Hb, high/low WBCs
Lactate dehydrogenase - elevated
immunophenotyping

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86
Q

what is the gold standard for Hodgkin’s lymphoma?

A

exicisional lymph node biopsy

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87
Q

what are 3 differentials for Hodgkin’s lymphoma?

A

non-hodgkin’s
lymphadenopathy from other malignancy
infectious mononucleosis

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88
Q

what is the treatment for Hodgkin’s lymphoma?

A

chemo - ABVD (Adriamyacin, Bleomycin, Vinblastine, Dacarbazine)
radiotherapy
Rituximab

marrow transplant

89
Q

what are 3 complications for Hodgkin’s lymphoma?

A

radiotherapy related thyroid abnormalities
chemo related cardio disease
pulmonary toxicity

90
Q

what staging is used in Hodgkin’s lymphoma?

A

Ann Arbor classification

91
Q

what are the Ann Arbor classification stages?

A 
I - single lymph node
II - 2+ lymph nodes one side of diaphragm
III - nodes on both sides of diaphragm 
IV - spread beyond lymph nodes 
A/B => presence of B symptoms or not
92
Q

what is the most common type of anaemia?

A

iron deficiency

93
Q

what percentage of menstruating women have iron deficiency anaemia?

A

14%

94
Q

what are 4 causes of iron deficiency?

A

low dietary iron
impaired absorption (coeliac)
increased iron loss (bleeding)
increased iron required (children, pregnant, lactating)

95
Q

what are 6 risk factors for Iron deficiency anaemia?

A 
pregnancy
veggie/vegan diet
mennhoragia
hook worms 
CKD
gasterectomy/NSAIDs
96
Q

what are 6 manifestations of iron deficiency anaemia?

A 
anaemia symtoms 
restless leg syndrome
nail changes - thin, flat, spoon
dysphagia/dyspepsia
glossitis 
heart failure
97
Q

where is iron absorbed?

A

duodenum

98
Q

what are 5 causes of microcytic anaemia?

A

TAILS

Thallasaemia 
Anaemia of chronic disease
Iron deficiency 
Lead poisoning 
Sideroblastic anaemia
99
Q

what is the treatment of iron deficiency anaemia?

A

oral iron replacement - ferrous sulphate

absorbic acid
IV iron
RBC replacement

100
Q

what are 3 complications of iron deficiency anaemia?

A

cognitive impairment
impaired muscular performance
high output heart failure

101
Q

what is the top cause of iron deficiency worldwide?

A

hook worms

102
Q

what is malaria?

A

a parasitic infection caused by protozoa of the genus Plasmodium which is transmitted to humans by the bite of an anopheles mosquito.

103
Q

what plasmid of malaria causes the most deaths?

A

Plasmodium falciparum

104
Q

what are 6 manifestations of malaria infection?

A 
fever and headache
myalgia/weakness
seizures
jaundice/pallor/anaemia
hepatosplenomegaly
influenza like resp symptoms
105
Q

what are 4 investigations for malaria?

A

Giemsa stain blood film - gold
FBC, U+E, LFTs
rapid diagnostic test
thin and thick blood smear

106
Q

what are 3 differentials for malaria?

A

Dengue fever (headache, retrobulbar pain worsening with eye movement)
Zika virus
Yellow fever

107
Q

what is the management of uncomplicated malaria?

A

arthemether + lumefantrine
malarone
quinine sulphate
doxycycline

108
Q

what are 3 complications of malaria?

A

AKI
cerebral malaria
severe haemolytic anaemia

109
Q

what is multiple myeloma?

A

cancer of differentiated B lymphocytes (plasma cells)

110
Q

what are 5 risk factors for multiple myeloma?

A 
MGUS - monoclonal gamopathy of undetermined significance 
Increasing age
FHx
radiation/petroleum exposure
Black African
111
Q

what is the most common Ig produced in multiple myeloma?

A

IgG - 55%

112
Q

what are 6 manifestation of multiple myeloma?

A

Old CRAB

  • Age 65+
  • Calcium - high
  • Renal failure
  • Anaemia
  • Bone pain

infections
fatigue

113
Q

what are 3 investigations for multiple myeloma?

A

urine electrophoresis
serum electrophoresis
bone marrow aspirate and biopsy >10%

114
Q

what is the gold standard for multiple myeloma?

A

serum and urine protein electrophoresis

bone marrow examination and imaging

115
Q

what are 3 differentials for multiple myeloma?

A

MGUS
smoldering myeloma
amyloidosis

116
Q

what is the treatment for multiple myeloma?

A

chemo - bortezomib + dexamethasone/prednisolone (70+)

bone marrow transplant
bisphosphonates - zoledronate

117
Q

what are 3 complications of multiple myeloma?

A

recurrent infections
Al amyloidosis
pathological fractures

118
Q

is multiple myeloma curable?

A

NO

- relapse in 2-5 years

119
Q

what is non-Hodgkin’s lymphoma?

A

Malignant proliferations of lymphocytes without reed-Sternberg cells

120
Q

What are 5 risk factors for non-Hodgkin’s lymphoma?

A 
FHx
50+
male 
EBV/HHV-8
autoimmune disease
121
Q

are non-hodgkin’s lymphomas more commonly of T or B cell origin?

A

B cell - 80%

T cell - 20%

122
Q

what are 6 presentations of non-hodgkin’s?

A 
B symtoms 
lymphadenopathy 
hepato/splenomegaly 
chest/bone/back pain 
SOB/cough 
neuronal abnormalities - weakness in legs
123
Q

what are 3 investigations of nom-hodgkin’s lymphoma?

A

FBC with differential - thrombocytopenia, pancytopenia, lymphocytosis
blood smear
excision lymph node biopsy

124
Q

what are 3 differentials for non-hodgkin’s lymphoma?

A

Hodgkin’s lymphoma
ALL
infectious mononucleosis

125
Q

what is the management of non-hodgkin’s lymphoma?

A

chemo
radiotherapy
rituximab
stem cell transplant

126
Q

what are 3 complications of non-hodgkin’s lymphoma?

A

impaired immunity
myelosuppresion and neutropenic sepsis
tumour lysis syndrome

127
Q

what is Polycythaemia vera?

A

a malignant clonal proliferation of erythrocytes

128
Q

what genetic mutation if associated with Polycythaemia vera?

A

JAK2 V617F

129
Q

what are 6 presentations of Polycythaemia vera?

A 
splenomegaly
conjunctival prethora
hypertension
headache, fatigue, dizziness
pruritus 
erythromelalgia (red painful palms and soles)
130
Q

what are 4 investigations of Polycythaemia vera?

A

FBC - elevated Hb + haematocrit
U+E +LFTS
EPO - distinguish primary from secondary
JAK2 mutation

131
Q

what are 3 differentials for Polycythaemia vera?

A

CML
essential thrombocythaemia
congenital Polycythaemia

132
Q

what are 4 treatments of Polycythaemia vera?

A

venesection
hydroxycarbamide
aspirin
ruxolitinib

133
Q

what is pulmonary embolism?

A

a life-threatening condition due to dislodged thrombi occluding the pulmonary vasculature

134
Q

what percentage of DVTs embolize?

A

51%

135
Q

what are 5 risk factors for PE?

A 
age 
DVT
obesity 
surgery
malignancy
136
Q

what are 6 presentations of PE?

A 
dyspnoea 
pleuritic chest pain 
hyperaemia 
signs of DVT
fever and cough
haemoptysis
137
Q

what are 3 investigations for PE?

A

CT pulmonary angiography - gold
ECHO
d-dimmer

138
Q

what are 3 differentials for PE?

A

unstable angina
MI
pneumonia

139
Q

what is the management of PE?

A

anticoagulation - apixiban (1) or LMWH (2)

thrombolysis

140
Q

what are 3 complications of PE?

A

acute bleeding during treatment
pulmonary infarction
cardiac arrest/death

141
Q

what is sickle cell anaemia?

A

a disease of red blood cells caused by an autosomal recessive single gene defect in the beta chain of haemoglobin, which results in production of sickle cell haemoglobin (HbS) which can obstruct blood flow and break down prematurely

142
Q

what triggers sickling in sickle cell disease?

A

hypoxia, acidosis, dehydration, cold temperatures, extreme exercise, stress, and infections can precipitate sickling

143
Q

when does sickle cell disease manifest and why?

A

6 months

before that point there is foetal haemoglobin

144
Q

what is foetal haemoglobin made up of?

A

2 alpha chains and 2 gamma chains

145
Q

what are 6 presentations of sickle cell?

A 
parent with sickle cell/trait 
persistent skeletal pain (chest, abdomen)
dactylitis 
high temperature 
pneumonia like syndrome 
failure to thrive
146
Q

what are 3 investigations for sickle cell?

A

blood film
Hb isoelectric focusing
peripheral blood smear
Newborn Guthrie heel prick test (5 days)

147
Q

what are 3 differentials of sickle cell?

A

gout
septic arthritis
connective tissue disorders

148
Q

what is the management of sickle cell?

A 
analgesia 
oral hydroxycarbamide (increase foetal haem)
blood transfusion
stem cell transplant 
antibiotics and antihistamine
149
Q

what is acute chest syndrome?

A

complication of sickle cell - chest pain, cough, SOB, fever, hypoxia (low oxygen level) and lung infiltrates

150
Q

what are 3 complications of sickle cell?

A

anaemia
opioid dependance
iron overload (chronic transfusion)

151
Q

what is deltaparin and how does it work?

A

anticoagulant

inhibits factor Xa and thrombin by antithrombin

152
Q

what is riveroxiban and how does it work?

A

DOAC anticoagulant

factor Xa inhibitor

153
Q

what is warfarin’s MOA?

A

competitively inhibits vitamin K epoxide reductase

154
Q

what are the vitamin K dependant clotting factors?

A

X, IX, VII, II - 1972

155
Q

what is heparin’s MOA?

A

binds to antithrombin III which then inactivates factors Xa and IIa (thrombin)

156
Q

can warfarin be used in pregnancy?

A

no it’s teratogenic

157
Q

what is the name of the histological feature that appears like stacked coins?

A

Rouleaux formation

158
Q

what disease do you see rouleaux formation in?

A

multiple myeloma

stacked coin appearance of RBCs due to high levels of immunoglobulins causing cells to stick together

159
Q

what are 5 generic signs of anaemia?

A 
pale skin and conjunctiva
tachycardia
bounding pulse
raised resp rate 
postural hypotension
160
Q

what are 4 signs of iron deficiency anaemia?

A

koilonychia - spoon shaped nails
angular chelitis
atrophic glossitis
Brittle hair and nails

161
Q

what is a sign of haemolytic anaemias?

A

jaundice

162
Q

what is a sign of thalassaemia?

A

bone deformities

163
Q

what are 3 signs of CKD anaemia?

A

oedema
hypertension
excoriations on skin

164
Q

what are 8 investigations for anaemia?

A 
FBC - Hb and MCV
Blood film 
Reticulocyte count 
Ferritin
B12 and folate
Bilirubin - up in haemolysis
Direct Coombs test - autoimmune haemolytic 
Haemoglobin electrophoresis
165
Q

what are the 4 iron studies?

A

serum iron
serum ferritin
total iron binding capacity (increased in anaemia)
transferrin saturation

166
Q

what is the inheritance pattern of hereditary spherocytosis?

A

majority autosomal dominant

167
Q

what is the management of hereditary spherocytosis?

A

phototherapy or exchange transfusion - neonates with jaundice

folic acid supplementation

Splenectomy

phenoxymethylpenicillin

168
Q

what are 3 complications of hereditary spherocytosis?

A

gallstones
aplastic crisis
bone marrow expansion

169
Q

what are 4 precipitators of G6PD deficiency?

A

Fava beans
soy
red wine
infections

170
Q

what does the urine look like in G6PD deficiency?

A

tea coloured

171
Q

what is aplastic anaemia?

A

stem cell disorder characterised by pancytopenia

172
Q

what are 4 causes of aplastic anaemia?

A

radiation and toxins
drugs
infections
fanconi’s anaemia

173
Q

what condition are Howell-jolly bodies seen in?

A

Sickle cell

174
Q

what is a histological sign go sickle cell disease?

A

Howell-jolly bodies

175
Q

what is the most common cause of osteomyelitis in sickle cell crisis?

A

salmonella

176
Q

what are the components of the wells score?

A 
active cancer
bedridden/major surgery
calf welling >3cm
superficial veins present
entire leg swollen
tenderness along veins
pitting oedema of one leg
immobility of affected leg
previous DVT

alternative diagnosis likely? => -2

177
Q

what do Reed-Sternberg cells look like?

A

owl eye nuclei

178
Q

what is found in the urine in multiple myeloma?

A

bench-jones proteins - Ig light chains

179
Q

what is found on the serum electrophoresis in multiple myeloma?

A

monoclonal paraprotein band

180
Q

what is the treatment of severe malaria?

A

Artemisinin combination therapy (ACT)

quinine

181
Q

what is the inheritance pattern for haemophilia?

A

X-linked recessive

182
Q

which factor is there a deficiency in in haemophilia A?

A

VIII

183
Q

which factor is there a deficiency in in haemophilia B?

A

IX

184
Q

what are 3 aquired causes of haemophilia?

A

liver failure
vitamin K deficiency
autoimmunity against clotting factors

185
Q

what are 6 clinical manifestations of haemophilia?

A 
abnormal bleeding 
excessive bleeding 
easy bruising
spontaneous haemorrhage
haematomas
joint pain
186
Q

what are 2 differentials for haemophilia?

A

Von Willebrand disease

platelet dysfunctions

187
Q

what is the management for haemophilia?

A

avoid contact sport
IV infusion of clotting factor
desmopressin
antifibronolytics

188
Q

what are 2 complications of haemophilia?

A

bleeds into brain

haemarthrosis

189
Q

what chromosome codes for von willebrand factor?

A

chromosome 12

190
Q

what does von willebrand factor do?

A

attaches to collagen fibres allowing platelets to adhere

191
Q

what is the most common bleeding disorder?

A

Von Willebrand disease

192
Q

what is disseminated intravascular coagulation?

A

an acquired syndrome characterised by activation of coagulation pathways resulting in intravascular thrombi and depletion of platelets and coagulation factors

193
Q

what are 5 manifestations of DIC?

A 
shock 
bleeding 
confusion
bruising
thrombotic events
194
Q

what is the treatment of DIC?

A

platelet transfusion
fresh frozen plasma
RBC transfusion

195
Q

what is thalassaemia

A

autosomal recessive haemoglobinopathy which causes microcytic anaemia

196
Q

what demographic is alpha thalassaemia most common in?

A

asian and African descent

197
Q

what demographic is beta thalassaemia most common in?

A

SE asian, Mediterranean and Middle Eastern descent

198
Q

what can be given to thalassaemia patients to prevent iron overload?

A

deferoxamine

199
Q

what can reverse heparin overdose?

A

protamine sulphate

200
Q

what is the antidote to warfarin?

A

vitamin K1

201
Q

What is the Philadelphia chromosome?

A

Translocation of a part of chromosome 9 to chromosome 22

202
Q

what is immune thrombocytopenia purpura?

A

a blood disorder characterized by a decrease in the number of platelets in the blood.

bleeding, recent Hx of infection/immunisation, autoimmune disorders

203
Q

what is the management of immune thrombocytopenia purpura?

A

1 - corticosteroids (prednisolone) and IV IgG

2 - splenectomy, immunosuppreion (azathioprine)

204
Q

is primaquine safe in pregnancy?

A

NO

205
Q

what is the most common chromosome abnormality in myeloma?

A

t(11;14)

206
Q

what is given as tumour lysis prophylaxis in AML?

A

allopurinol

207
Q

what electrolyte imbalanced does tumour lysis syndrome cause?

A

hyperkalemia, hypocalcemia, hyperphosphatemia, and hyperuricemia.

208
Q

what are the histological signs of G6PD?

A

bite cells
Heinz bodies
reticulocytes

209
Q

what antibiotic is contraindicated in G6PD?

A

trimethoprim

210
Q

what are the 6 Ps of critical ischaemia?

A 
pale
pulseless
perishingly cold
painful 
parasethesis 
paralysis
211
Q

what is Disseminated intravascular coagulation?

A

n acquired syndrome characterised by activation of coagulation pathways, resulting in formation of intravascular thrombi and depletion of platelets and coagulation factors.

212
Q

what is the sepsis 6?

A

Give: Fluids, Broad spectrum Abx, Administer O2 if required Take: Bloods, Urine Output, Lactate levels

213
Q

What leukaemia is tumour lysis syndrome most common in?

A

AML

214
Q

what cells are seen in CLL?

A

smudge cells

215
Q

what cells are seen in AML?

A

Auer rods

216
Q

what cells are seen in ALL?

A

blast cells

217
Q

what is antiphospholipid syndrome?

A

autoimmune disease that causes blood to be hypercoagulable. increases risk of PE, DVT, Stroke, MI and MISCARRIAGE

218
Q

what antibodies cause antiphospholipid syndrome?

A

antiphospholipid antibodies

Phase 4 (19 decks)

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