Vascular and Haematology Flashcards
(135 cards)
1
Q
What are the symptoms of chronic limb ischaemia?
A
Most patients are asymptomatic
Intermittent claudication - pain on exercise, relieved by rest
Diminished or absent pulse
Can progress to critical limb ischaemia and pain on rest
Symptoms worsened when lying down
2
Q
What are the signs of acute limb ischaemia?
A
Pallor
Perishingly cold
Pulselessness
Pain
Paraesthesia
Paralysis
3
Q
What are the features of critical limb ischaemia?
A
ischaemic rest pain >2 weeks, requiring opiate analgesia
presence of ischaemic lesions/ulcers/gangrene
ABPI <0.5
4
Q
what are the differentials for chronic limb ischaemia?
A
spinal stenosis: symptoms relieved by sitting, pain from back radiating down leg
acute limb ischaemia: rapid onset
5
Q
What are the risk factors for PAD?
A
Smoking
Diabetes
HTN
Hyperlipidaemia
Age >40 years
6
Q
How is PAD diagnosed?
A
Ankle-brachial index (0.9 for diagnosis, claudication: 0.4-0.9, rest pain: 0.2-0.4, tissue loss and gangrene: 0-0.4)
Handheld arterial Doppler examination
Assess whether ischaemia is due to thrombus (atherosclerotic plaque) or embolus (secondary to AF)
7
Q
How is acute limb ischaemia treated?
A
high flow oxygen and IV heparin immediately
surgery: embolectomy, local intra-arterial thrombolysis, bypass surgery
irreversible limb ischaemia requires amputation
8
Q
How is claudication treated?
A
80mg atorvastatin OD
75mg clopidogrel OD
smoking cessation and supervised exercise programme
naftidrofuryl oxalate (vasodilator)
treat comorbidities (HTN, diabetes, obesity)
9
Q
What are the symptoms of varicose veins?
A
Dilated tortuous veins
Leg fatigue or aching with prolonged standing (improves with elevation)
Leg cramps, usually nocturnal
Venous ulcers
Oedema
10
Q
What are the risk factors for varicose veins?
A
Increasing age
Female gender
Pregnancy - the uterus causes compression of the pelvic veins
Obesity
11
Q
How are varicose veins investigated?
A
Duplex ultrasound (retrograde venous flow)
12
Q
How are varicose veins treated?
A
MOSTLY CONSERVATIVE TREATMENT - leg elevation, weight loss, exercise, compression stockings
Endovenous thermal ablation (radiofrequency or laser)
Phlebectomy or foam sclerotherapy
13
Q
How is leukaemia investigated?
A
Pancytopenia (anaemia, leukopenia, thrombocytopenia)
Blood film
LDH (raised, non-specific)
Bone marrow biopsy
CT, MRI or PET (staging)
CXR (lymph node involvement, infection)
Lymph node biopsy
14
Q
What is AML?
A
Rapid proliferation of myeloblasts
15
Q
What are the risk factors for acute myeloid leukaemia?
A
Increasing age (most common in adults)
Previous chemotherapy
Down’s syndrome
Irradiation
Myeloproliferative disorder, e.g. polycythaemia ruby vera or myelofibrosis
16
Q
What are the symptoms of acute myeloid leukaemia?
A
Anaemia: pallor, lethargy, weakness
Neutropenia: frequent infections, fever
Thrombocytopenia: bleeding, petechiae, abnormal bruising
Splenomegaly
Bone pain
17
Q
What is characteristic of the blood film of AML?
A
High proportion of blast cells with Auer rods in their cytoplasm
18
Q
What is ALL?
A
Rapid proliferation of lymphoblasts
19
Q
What are the risk factors for acute lymphoblastic leukaemia?
A
Most common childhood cancer
Genetics and FHx
Down’s syndrome
Influenza
Radiation
20
Q
What are the symptoms of ALL?
A
Bone pain
Hepatosplenomegaly
Painless unilateral testicular swelling
Pancytopenia symptoms (fever, bleeding, lethargy)
Focal neurological symptoms, papilloedema, nuchal rigidity
Lymphadenopathy (unlike in AML)
Fever, weight loss, night sweats
21
Q
What is characteristic of the blood film of ALL?
A
> 20% lymphoblasts on bone marrow biopsy
22
Q
What is CML?
A
Hyperproliferation of granulocyte precursors
23
Q
What are the risk factors for chronic myeloid leukaemia?
A
Philadelphia chromosome
Age >65
24
Q
What are the phases of CML?
A
Chronic phase (lasts 5 years, asymptomatic, diagnosed incidentally with raised WCC)
Accelerated phase (abnormal blast cells take high proportion of cells in bone marrow - patients become more symptomatic and immunocompromised)
Blast crisis (like an acute leukaemia, severe, fatal)
25
What are the symptoms of CML?
Up to 50% are asymptomatic - more likely to be asymptomatic during chronic phase
Hyperviscosity symptoms (due to more RBCs) - thrombotic events, headaches
Hypermetabolic symptoms: weight loss, night sweats, malaise
Bone marrow failure: pallor, bleeding, infections/fever
Splenomegaly (more than in acute as time for spleen to enlarge)
Decreased leukocyte ALP
26
What is chronic lymphocytic leukaemia?
Progressive accumulation of functionally incompetent lymphocytes (failure of apoptosis of incompetent cells)
27
What are the symptoms of CLL?
Often asymptomatic - can present with infections, anaemia, bleeding, weight loss
Non-tender lymphadenopathy
Warm autoimmune haemolytic anaemia
Can transform into high-grade lymphoma - Richter's transformation (non-Hodgkin lymphoma)
28
What does a blood film show with CLL?
'Smear' or smudge' blood cells - from rupture of aged or fragile WBCs
29
What are the risk factors for Hodgkin's lymphoma?
HIV
Epstein-Barr virus
Autoimmune conditions, e.g. RA and sarcoidosis
FHx
30
What are the symptoms of Hodgkin's lymphoma?
Painless enlarging neck mass, which may become painful after alcohol consumption
Non-tender, 'rubbery' lymph nodes with hepatosplenomegaly
Fever, weight loss, night sweats
Neutrophilia
Skin excoriations
31
How is Hodgkin's lymphoma investigated?
LDH (raised)
FBC (normocytic anaemia, eosinophilia)
Lymph node biopsy (Reed-Sternberg cells - bi-nucleate, large B cells)
CT, MRI, PET for staging
32
What are the risk factors for non-Hodgkin's lymphoma?
More common than Hodgkin's lymphoma
EBV
FHx
History of chemo- or radiotherapy
Immunodeficiency (transplant, HIV, diabetes mellitus)
Autoimmune disease (SLE, Sjogren's, coeliac)
33
What are the symptoms of non-Hodgkin's lymphoma?
Painless enlarging mass in neck, axilla or groin
Fever, weight loss, night sweats (less common than HL)
Skin rashes (e.g. mycosis fungoides), headache, hepatosplenomegaly (more common than HL)
Neutropenia
34
How is non-Hodgkin's lymphoma diagnosed?
Lymph node biopsy - no Reed-Sternberg cells
35
What is Burkitt's lymphoma?
Subtype of non-Hodgkin's lymphoma
Strong association with EBV infection, malaria, HIV
Gives starry sky appearance under microscopy
36
What is multiple myeloma?
Proliferation of plasma cells (B-lymphocytes) - production of a monoclonal immunoglobulin (IgA or IgG)
37
What are the risk factors for multiple myeloma?
Male
Black African ethnicity
Older age
FHx
Obesity
Ionising radiation
38
What are the features of multiple myeloma?
Hypercalcaemia (constipation, nausea, anorexia, confusion) - increased osteoclastic bone resporption
Renal impairment - dehydration and increasing thirst
Normocytic anaemia - due to bone marrow crowding suppressing EPO production
Bone lesions - increased osteoclast activity (bony pain)
Infection
Hyperviscosity (bruising, bleeding, visual loss, palmar erythema)
39
How is multiple myeloma investigated?
Urine protein electrophoresis (Bence-Jones protein)
Serum protein electrophoresis
Blood film (Rouleaux formation)
Bone profile (hypercalcaemia, normal ALP - in bone cancer, ALP is raised)
U&Es (raised urea, creatinine)
FBC (normocytic anaemia)
Bone marrow aspirate (increased plasma cells)
X-ray ('raindrop skull', lytic lesions, punched out lesions)
40
How is multiple myeloma treated?
Chemotherapy with bortezomid, thalidomide, dexamethasone
Stem cell transplantation
Aspirin/low molecular weight heparin for thromboembolism prophylaxis
41
What are the risk factors for haemophilia?
FHx
Male sex
42
What are the signs and symptoms of haemophilia?
Haemarthrosis (bleeding into joints) - can lead to joint damage and deformity
Haematoma (bleeding into muscles)
Excessive bruising
Haematuria and GI bleeding
Prolonged bleeding after surgery or trauma
43
How is haemophilia investigated?
APTT (prolonged)
Coagulation factor assay (decreased factor VIII or IX levels)
44
How is haemophilia managed?
IV infusions of factor VIII or IX - can lead to antibodies against the clotting factor
45
What are the myeloproliferative disorders?
Primary myelofibrosis (proliferation of haematopoietic stem cells)
Polycythaemia vera (proliferation of erythroid cell line)
Essential thrombocythaemia (megakaryocytic cell line)
They have the potential to transform into acute myeloid leukaemia
46
What are the features of polycythaemia vera?
pruritus, typically after a hot bath
splenomegaly
hypertension
hyperviscosity (arterial and venous thrombosis)
haemorrhage (secondary to abnormal platelet function)
low ESR
JAK-2 mutation
47
How is polycythaemia vera investigated?
FBC (raised haematocrit)
JAK2 mutation
Serum ferritin
Renal and liver function tests
48
What is myelofibrosis?
Proliferation of the cell line leads to fibrosis of the bone marrow - can lead to anaemia and leukopenia, as production of blood cells is disrupted
Haematopoiesis begins in other areas, e.g. the liver and the spleen - hepatosplenomegaly, portal hypertension, spinal cord compression
49
What are the features of myelofibrosis?
Elderly person with symptoms of anaemia (e.g. fatigue)
massive splenomegaly
hypermetabolic symptoms: weight loss, night sweats etc
50
How is myelofibrosis investigated?
Bone marrow biopsy (dry tap)
Blood film: 'tear drop' poikilocytes
Anaemia
High urate and LDH
51
What are the symptoms of DIC?
Oliguria, hypotension, tachycardia (signs of circulatory collapse)
Delirium or coma
Bleeding
Bruising
SOB
52
What are the risk factors for DIC?
Sepsis
Trauma
Obstetric complications
Malignancy
53
How is DIC diagnosed?
Prolonged PT and APTT
Low platelets and fibrinogen
Low haemoglobin
High D-dimer
54
How is DIC treated?
Treat underlying conditions
FFP (replace clotting factors)
Cryoprecipitate (replaces fibrinogen)
Anticoagulation (heparin)
55
What are the risk factors for sickle cell disease?
Genetic - autosomal recessive
More common in countries with high rates of malaria
56
What are the symptoms of sickle cell disease?
Dactylitis (swollen hands and feet)
Vaso-occlusive crisis - pain in skeleton, chest and/or abdomen
Splenic sequestration (splenomegaly, severe anaemia, hypovolaemic crisis)
Aplastic crisis (loss of creation of new blood cells - severe anaemia)
Acute chest syndrome
Haemolytic anaemia
57
How is sickle cell disease diagnosed?
Haemoglobin electrophoresis
Newborn screening heel prick test
Also high reticulocyte count
58
What are the complications of sickle cell disease?
Anaemia
Increased risk of infection
Stroke
Avascular necrosis in large joints such as the hip
Pulmonary hypertension
Painful and persistent penile erection (priapism)
Chronic kidney disease
Sickle cell crises
Acute chest syndrome
59
what are the features of thrombotic crises in sickle cell disease?
precipitated by infection, dehydration, deoxygenation
infarcts occur in various organs
60
What does sickle cell disease look like on blood film?
Howell-Jolly bodies
Sickle cells
61
How is sickle cell disease treated?
Pneumococcal immunisation
Avoid dehydration and other triggers
Antibiotic prophylaxis (penicillin V)
Hydroxycarbamide can be used to stimulate production of fetal haemoglobin (HbF)
Blood transfusion for severe anaemia
Bone marrow transplant can be curative
62
How is acute chest syndrome treated?
pain relief
oxygen therapy
antibiotics
exchange transfusion
63
What are the symptoms of acute chest syndrome?
Dyspnoea
Chest pain
Fever
New infiltrates seen on CXR
Low O2
64
what are the features of an aplastic crisis?
caused by infection with parvovirus
sudden drop in haemoglobin
bone marrow suppression causes decreased reticulocyte count
65
what are the features of a sequestration crisis?
increased reticulocyte count
sickling within organs causes pooling of blood
66
What are the features of iron deficiency anaemia on a blood film?
Aniso-poikilocytosis (RBCs with different sizes and shapes)
Pencil cells/elliptocytes
Hypochromic and microcytic
67
What are the causes of iron deficiency anaemia?
Reduced uptake: malnutrition, coeliac, IBD
Increased loss: GI malignancy, peptic ulcer, IBD, menstruation
Increased requirement: pregnancy, breastfeeding
68
What are the risk factors for haemolytic uraemic syndrome?
Exposure to contaminated food/water (E. coli)
Typically seen in children
69
What are the symptoms of haemolytic uraemic syndrome?
AKI
Bloody diarrhoea
Microangiopathic haemolytic anaemia (jaundice, conjunctival pallor)
Thrombocytopenia
Schistocytes and helmet films on blood film
70
What are the risk factors for DVT?
Age >60
Active cancer
Dehydration
Recent orthopaedic or pelvic surgery
Long-distance travel
Obesity
Thrombophilias
Combined oral contraceptives and hormone replacement therapy
Pregnancy
71
What are the signs of DVT?
Unilateral warm, swollen calf or thigh
Pain on palpation of deep veins
Distention of superficial veins
Pitting oedema
72
How is DVT assessed?
The Wells score - if score is >2, DVT is likely
73
How is DVT managed if Wells score >2?
Doppler ultrasound within 4 hours - if not possible, do D-dimer and give interim DOACs
D-dimer if ultrasound is negative
If scan is negative and D-dimer is positive, stop interim anticoagulation and repeat ultrasound 6-8 days later
74
How is DVT managed if Wells score <2?
D-dimer - high sensitivity, low specificity so will only exclude DVT
Ultrasound within 4 hours if D-dimer is positive
75
How long should patients with DVT be anticoagulated for?
Provoked DVT: 3 months
Unprovoked DVT: 6 months
76
What are the risk factors for venous ulcers?
Increasing age
Pre-existing venous incompetence (inc. varicose veins) or history of VTE
Pregnancy
Obesity
Severe leg injury or trauma
77
How do venous ulcers present?
Seen on median malleolus
Shallow, sloughy ulcers
Haemosiderin deposition of the lower leg
Oedema
Skin thickening
Eczema
78
How are venous ulcers managed?
Leg elevation and increased exercise
Multicomponent compression bandaging - do ABPI first to exclude arterial involvement
Emollient treatment
Oral pentoxyfilline
Surgery if concurrent varicose veins
79
What are the risk factors for arterial ulcers?
Smoking
DM
HTN
Hyperlipidaemia
FHx
Increasing age
80
How do arterial ulcers present?
Preceding history of intermittent claudication or critical limb ischaemia
Occur on toes or heels
'Deep, punched out' appearance
Painful
There may be areas of gangrene
Cold with no palpable pulses
Low ABPI measurements
81
How are arterial ulcers treated?
Lifestyle changes (weight loss, diet, exercise)
Statin, aspirin/clopidogrel, ACEi
Surgery: angioplasty or bypass grafting
82
What are the risk factors for neuropathic ulcers?
Any disease causing peripheral neuropathy - most commonly B12 deficiency or diabetes
Foot deformity
Concurrent peripheral vascular disease
83
How do neuropathic ulcers present?
Painful neuropathy
Peripheral neuropathy in 'glove and stocking' distribution
Warm feet and good pulses
84
What is pernicious anaemia?
Auto-antibodies against intrinsic factor or parietal cells, leading to vitamin B12 deficiency
It can predispose to gastric carcinoma
85
What are the causes of anaemia of chronic disease?
Malignancy
Chronic infections such as TB
Connective tissues disease such as rheumatoid arthritis
86
What is the difference between anaemia of chronic disease and iron deficiency anaemia?
ACD: normal or raised ferritin, low total iron binding capacity, low iron
IDA: low ferritin, high total iron binding capacity, low iron
87
What are the causes of microcytic anaemia?
Thalassaemia
Anaemia
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemia
88
what are the features of sideroblastic anaemia?
hypochromic microcytic anaemia
high ferritin, high iron, high transferritin saturation
basophilic stippling of red blood cells
89
What are the causes of normocytic anaemia?
Acute blood loss
Anaemia of Chronic Disease
Aplastic Anaemia
Haemolytic Anaemia
Hypothyroidism
90
What are the causes of hereditary haemolytic anaemia?
Hereditary spherocytosis (autosomal dominant)
G6PD deficiency (X-linked recesive)
Sickle cell disease, thalassaemia
COOMBS TEST NEGATIVE
91
What does the blood film of G6PD deficiency show?
Heinz bodies
Bite cells
92
What are the features of haemolytic anaemia?
Jaundice
Pallor
Low haemoglobin
Low haptoglobin
93
What are the features of acute haemolytic transfusion reaction?
Fever, abdominal pain and hypotension minutes after transfusion is started
94
How is acute haemolytic transfusion reaction managed?
Stop transfusion
IV fluids
95
What are the complications of acute haemolytic transfusion reaction?
DIC
Renal failure
96
What are the features of non-haemolytic febrile reaction? How is it managed?
Fever, chills - stop transfusion, paracetamol
97
What are the features of transfusion-related acute lung injury?
Hypoxia
Pulmonary infiltrates on CXR
Fever
Hypotension
98
How is transfusion-related acute lung injury treated?
Stop transfusion
Oxygen and supportive care
99
How is transfusion-associated circulatory overload treated?
Stop transfusion
Consider IV furosemide
100
What are the features of transfusion-associated circulatory overload?
Pulmonary oedema
Hypertension
101
What are the causes of hyposplenism?
splenectomy
sickle-cell
coeliac disease, dermatitis herpetiformis
Graves' disease
systemic lupus erythematosus
amyloid
102
What are the features of hyposplenism on blood film?
Howell Jolly bodies
Pappenheimer bodies (siderocytes)
Target cells
103
What are the indications for splenectomy?
Trauma
Rupture (e.g. in EBV infection)
Haemolytic anaemia
Hypersplenism: hereditary spherocytosis, immune thrombocytopenia
Neoplasia (lymphoma or leukocytic infiltration)
104
what are the causes of metabolic acidosis with normal anion gap?
GI bicarbonate loss
renal tubular acidosis
ammonium chloride injection
Addison's disease
105
what are the causes of metabolic acidosis with raised anion gap?
lactic acidosis: sepsis, shocks, burns, hypoxia, metformin
ketones: diabetic ketoacidosis, alcohol
urate: renal failure
acid poisoning: salicylates, methanol
106
what are the causes of metabolic alkalosis?
vomiting/aspiration - vomiting can also lead to hypokalaemia
diuretics
hypokalaemia
primary hyperaldosteronism
Cushing's syndrome
107
what are the causes of respiratory acidosis?
COPD
decompensation in other respiratory conditions, e.g. life-threatening asthma, pulmonary oedema
neuromuscular disease
obesity hypoventilation syndrome
sedative drugs: benzodiazepines, opiate overdose
108
what are the causes of respiratory alkalosis?
anxiety leading to hyperventilation
pulmonary embolism
salicylate poisoning - mixed respiratory alkalosis and metabolic acidosis
CNS disorders: stroke, encephalitis
altitude
pregnancy
109
what are the features of beta-thalassaemia trait?
mild hypochromic, normocytic anaemia
HbA2 raised (>3.5%)
usually asymptomatic
110
what are the features of thalassaemia?
presents in first year of life with failure to thrive
hepatosplenomegaly
microcytic anaemia
bone deformities - pronounced forehead and malar eminences
fatigue, pallor
jaundice
gallstones
111
how is thalassaemia treated?
repeated transfusions with iron chelation therapy (desferrioxamine)
112
how is thalassaemia investigated?
FBC: normocytic anaemia
haemoglobin electrophoresis
DNA testing
serum ferritin is checked for iron overload
113
what are the features of iron overload in thalassamia?
Fatigue
Liver cirrhosis
Infertility and impotence
Heart failure
Arthritis
Diabetes
Osteoporosis and joint pain
114
what is relative polycythamia?
falsely elevated haemoglobin secondary to low plasma volume - caused by dehydration and stress
115
what is primary polycythaemia?
excess erythrocytosis independent of erythropoetin (EPO) - caused by polycythaemia rubra vera
116
what are the causes of secondary polycythaemia?
COPD
altitude
obstructive sleep apnoea
117
how is polycythaemia vera treated?
aspirin - reduces risk of thrombotic events
venesection - first line treatment
chemotherapy - hydroxyurea, phosphorus-32
118
what are the investigation findings of autoimmune haemolytic anaemia?
normocytic anaemia
reticulocytosis
low haptoglobin
raised LDH and indirect bilirubin
blood film: spherocytes and reticulocytes
positive Coomb's test
119
how is warm autoimmune haemolytic anaemia treated?
steroids +/- rituximab
treatment of underlying disorder
120
what are the causes of warm autoimmune haemolytic anaemia?
idiopathic
autoimmune disease, e.g. SLE
drugs, e.g. methyldopa
neoplasia - lymphoma, CLL
121
what are the causes of cold autoimmune haemolytic anaemia?
neoplasia: e.g. lymphoma
infections: e.g. mycoplasma, EBV
122
what is basophilic stippling and what are the causes?
blue staining of ribosomal precipitates within the cytoplasm of RBCs
megaloblastic anaemia
thalassaemias (particularly alpha)
sideroblastic anaemia
alcohol abuse
123
what are howell jolly bodies and what causes them?
remnants of rbc nucleus - typically removed by the spleen
feature of hyposplenism
124
what are schistocytes?
fragments of RBCs seen in microangiopathic haemolytic anaemia - haemolytic uraemic syndrome, thrombotic thrombocytopaenia purpura, DIC
125
when are target cells seen?
obstructive liver disease
haemoglobinopathies (sickle cell disease, thalassaemia)
post-splenectomy
small number may be seen in iron deficiency anaemia
126
what is immune thrombocytopaenia?
an autoimmune condition of unknown cause where the number of circulating platelets is reduced
127
what are the features of immune thrombocytopaenia in children?
more acute - follows infection or vaccination
bruising
petechial or purpuric rash
bleeding is less common and typically presents as epistaxis or gingival bleeding
128
what are the features of immune thrombocytopaenia in adults?
may be detected incidentally following routine bloods
symptomatic patients may present with petechiae, purpura, bleeding (e.g. epistaxis)
129
how is immune thrombocytopaenia managed?
oral prednisolone
iV immunoglobulins may also be used
130
what are the features of hyperviscosity?
blurred vision
headache
vertigo
seizures
hearing loss
ataxia
increased bleeding frequency
131
what are the features of Henoch-Schonlein purpura?
IgA mediated small vessel vasculitis
usually seen in children following infection
palpable purpuric rash over buttocks and extensor surfaces of arms and legs
abdominal pain
polyarthritis
features of IgA nephropathy - haematuria, renal failure
132
how is Henoch-Schonlein purpura managed?
usually self-limiting
monitor blood pressure and renal involvement
133
what are the features of granulomatosis with polyangiiitis?
upper respiratory tract: epistaxis, sinusitis, nasal crusting
lower respiratory tract: dyspnoea, haemoptysis
saddle-shaped nose deformity (perforated nasal septum)
rapidly progressive glomerulonephritis (haematuria, proteinuria - frothy urine)
134
how is granulomatosis with polyangiiitis investigated?
cANCA positive, sometimes pANCA positive
CXR: cavitating lesions
renal biopsy: epithelial crescents in Bowman's capsule
135
what are the features of eosinophilic granulomatosis with polyangiiitis?
asthma
blood eosinophilia
paranasal sinusitis
pulmonary infiltrates
