Haematology Flashcards
(217 cards)
1
Q
What is anaemia defined as in men and women?
A
Men - Hb <130g/L
Women - Hb <120g/L
2
Q
What is the underlying cause of a microcytic anaemia?
A
Cytoplasmic problem resulting in a low MCV
3
Q
`What are causes of a microcytic anaemia?
A
Iron deficiency
Thalassaemia
Sideroblastic
4
Q
What are causes of a normocytic anaemia?
A
Blood loss
Chronic disease
Renal failure
Pregnancy
5
Q
What are the two categories of macrocytic anaemia?
A
Megaloblastic and non-megaloblastic
6
Q
What is the underlying cause of a megaloblastic anaemia?
A
A nuclear (DNA) problem
7
Q
What are some causes of a megaloblastic anaemia?
A
Vitamin B12 deficiency
Folate deficiency
Drugs
Pernicious anaemia
8
Q
What are some causes of non-megaloblastic anaemia?
A
Alcohol/liver disease
Hypothyroidism
Marrow failure - myelodysplasia, aplastic anaemia
9
Q
What are some signs of iron deficient anaemia?
A
Pallor, tiredness, koilonychia, glossitis, angular chelitis
10
Q
What test should you do to check the bodies iron levels?
A
Serum ferritin
11
Q
How is iron deficient anaemia treated?
A
Treat the cause first
Ferrous sulphate orally
12
Q
What is sideroblastic anaemia?
A
X-linked condition of ineffective erythropoeisis. Results in a microcytic anaemia that does not respond to iron
13
Q
What is the dietary source of vitamin B12?
A
Animal products
14
Q
What is the dietary source of folate?
A
Green leafy vegetables
15
Q
How long do body stores of B12 last?
A
2-4 years
16
Q
How long do body stores of folate last?
A
4 months
17
Q
Where is vitamin B12 absorbed in the gut?
A
Terminal ileum
18
Q
Where is folate absorbed in the gut?
A
Jejunum
19
Q
What are some causes of B12 deficiency?
A
Veganism, decreased stomach acid, pancreatitis, small bowel issues
20
Q
What are some causes of folate deficiency?
A
Inadequate intake, coeliac, crohns. pregnancy, anti-convulsants
21
Q
What are some general symptoms of megaloblastic anaemia?
A
Anaemia, weight loss, diarrhoea, beefy tongue
22
Q
What are some symptoms specific to B12 deficiency?
A
Neurological problems - neuropathy, dementia, subacute combined degeneration of the cord
23
Q
How is vitamin B12 deficiency treated?
A
initially, 3 IM injections of hydroxycobalamine per week for 2 weeks
If diet related -> biyearly injections
If not diet related -> injection every 3 months for life
24
Q
How is folate deficiency treated?
A
Folic acid tablets 5mg/day
25
If a patient has both B12 and folate deficiency which one should be treated first and why?
B12 as folate can precipitate subactube combined degeneration of the cord
26
What is pernicious anaemia?
Autoimmune destruction of gastric parietal cells leading to macrocytosis and pancytopenia
27
What antibodies will be positive in pernicious anaemia?
Anti-GPC, Anti-IF
28
How is pernicious anaemia treated?
B12 injections and folate tablets for life
29
What is a normal haemoglobin molecule made of?
Haem & Globin - 2 alpha chains and 2 beta chains
30
On what chromosome is the gene for alpha globin chains?
Chromosome 16
31
On what chromosome is the gene for beta globin chains?
Chromosome 11
32
How does alpha trait present?
Clinically asymptomatic. Microcytic haemochromatic cells. Normal ferritin. No treatment needed.
33
What is HbH disease?
3 faulty alpha chains (--l-a)
34
What is seen in bloods of HbH disease?
Anaemia, very low MCV and MCH
35
How does HbH present?
Variable - spelnomegaly, jaundice, growth retardation, gallstones, iron
36
What is the inheritance of HbH disease?
Autosomal recessive
37
What is Hb Barts Hydrops Fetalis?
Most severe form of alpha thalassamia - --l--
38
What haemoglobin types are commonly seen in Hb Barts hydrops fetalis?
Hb Barts and HbH
39
Why is Hb Barts so bad?
It has a high affinity for oxygen and so does not give it up readily to tissues
40
How does Hb Barts present?
Pallor, oedema, cardiac failure. Most babies die in utero or shortly after birth
41
What is seen on alpha thalassaemia blood films?
Anisopoikilocytosis
| Target Cells
42
What is the inheritance of B-thalassaemia?
Autosomal recessive
43
How will beta-thalassaemia trait present?
Asymptomatic. Low MCV/MCH.
| Raised HbA2 is diagnostic.
44
What is beta thalassaemia intermedia?
Two defective copies of B gene or one absent B gene and one defective B gene
45
How is beta thalassaemia intermedia treated?
Occasional blood transfusion
46
What is beta thalassaemia major also known as?
Cooley's anaemia
47
What is seen in FBC of beta thalassaemia major patients?
Moderate to severe anaemia
| Very low MCV/MCH
48
What is seen on blood film in beta thalassaemia major?
Reticulocytosis
Anisopoikilocytosis
Target cells
49
What does liquid chromatography (HPLC) show in beta thalassaemia major?
Mainly HbF
Small HbA
Increased HbA2`
50
When does beta-thalassaemia major present?
Aged 6-24 months
51
How does beta-thalassaemia major present?
Failure to thrive, pallor, extramedullary haematopoesis, hepatosplenomegaly, skeletal changes, organ damage
52
How is beta-thalassaemia major managed?
Regular transfusions to maintain Hb 95-105g g/L
53
What are some complications of beta-thalassaemia major?
Viral infections, alloantibodies, iron overload, iron overload, transfusion reactions
54
What is sickle cell disease?
Point mutations change the structure of B globin chains to make HbS
55
How is sick cell disease inherited?
Autosomal recessive
56
What happens to HbS in periods of low oxygen?
Polymerises and damages RBC, leading to sickling where cells are fragile, haemolyse and block small vessels
57
What is sickle cell trait?
One abnormal copy of the gene, asymptomatic carrier state. HbS levels too low to polymerise
58
What are some precipitants of sickle cell crisis?
Hypoxia, dehydration, infection, cold, stress, fatigue
59
What is the management of sickle cell crisis?
Opiate analgesia
| Hydration, rest, oxygen, antibiotics if infection
60
How could a severe sickle cell crisis be managed?
Blood transfusion
61
What causes an aplastic crisis in sickle cell disease?
Parvovirus B19
62
What is a sequestration crisis?
Pooling of blood in the spleen - requires an urgent transfusion
63
What temperature should red blood cells be stored at?
4 degrees C - in fridge
64
What temperature should platelets be stored at?
22 degrees C - room temperature
65
What temperature should FFP be stored at?
-30 degrees C - freezer
66
How long do you have to transfuse red cells and FFP?
Must be transfused within 4 hours
67
How long do you have to transfuse platelets?
Must be transfused within 1 hour
68
On what chromosome is ABO determined?
Chromosome 9
69
On what chromosome is Rhesus status determined?
Chromosome 1
70
What test should you use to screen patients plasma for irregular red cell antibodies?
Indirect antiglobulin test
71
What blood type is the universal donor?
O negative
72
What blood type is the universal acceptor?
AB positive
73
What antibody mediates an immediate transfusion reaction?
IgM - attack on RBCs
74
How does an immediate transfusion reaction present?
May occur after only 1ml has been transfused - fever, dizziness, sweating, tachycardia, hypotension, DIC, AKI, shock
75
How should you manage an immediate transfusion reaction?
STOP TRANSFUSION
Start IV fluids to maintain BP and urine output
Check bag of blood to see patient identity
Take bloods (FBC, U&Es, coag, cultures, LDH, blood film)
Contact haematology and return blood to the lab
76
What antibody mediates a delayed haemolytic transfusion reaction?
IgG - attack on antibodies in transferred blood
77
When does a delayed haemolytic transfusion reaction occur?
5 to 10 days following transfusion
78
How does a delayed haemolytic transfusion reaction present?
Jaundice, unexpected fall in Hb, AKI,
| Lab results show anaemia, spherocytic red cells, raised bilirubin and raised LDH
79
How do you manage a delayed haemolytic transfusion reaction?
Supportive care for any AKI/clotting issues
| Screen for irregular antibodies and give an antibody reference card for future transfusions
80
What is a febrile non-haemolytic reaction?
Reaction to white blood cells within the transfusion
81
When and how does a febrile non-haemolytic reaction present?
About 30 mins into transfusion - fever, rigors etc
82
How is a febrile non-haemolytic reaction managed?
Slow/stop transfusion. Paracetamol. Close monitoring. Next transfusion - give leucodepleted blood products
83
What mediates an urticarial transfusion reaction?
IgE - response to infused plasma proteins
84
How does an urticarial transfusion reaction present?
Rash/wheals within first few minutes
85
How is an urticarial transfusion reaction managed?
Slow/stop transfusion
Supportive care
Antihistamines
86
What is associated with an anaphylaxis transfusion reaction?
IgA deficiency
87
How does an anaphylactic transfusion reaction present?
Hypotension, mucosal swelling, stridor
88
How should an anaphylactic transfusion reaction be treated?
```
STOP TRANSFUSION
ABCDE and fluid challenge
Adrenaline 0.5ml 1:1000 IM
Hydrocortisone 200mg IV
Chloramphenimine 10mg IV
```
Give IgA depleted bloods next transfusion
89
What is the commonest childhood cancer?
ALL
90
How does ALL present?
Anaemia, infection, bleeding, bone pain, hepatosplenomegaly, orchidomegaly, lymphadenopathy
91
What sort of anaemia does ALL cause?
Normocytic normochromic anaemia, neutropenia and high WCC
92
How is ALL managed?
Multi-agent chemotherapy
Targeted therapies
Allogenic stem cell transplant
93
What is the cure rate of ALL in children?
90%
94
What is the cure rate of ALL in adults?
30-40%
95
What is AML?
Neoplastic proliferation of myeloid cells (e.g. monocytes, macrophages, neutrophils etc)
96
How does AML present?
Anaemia, infection, bleeding, hepatosplenomegaly, gym hypertrophy, skin involvment
97
What anaemia does AML show?
Normocytic normochromic anaemia
98
What is seen on the blood film and marrow biopsy of AML?
Auer Rods
99
How is AML managed?
Intensive treatment with chemotherapy
| Bone marrow transplants
100
How is AML t(15;17) treated?
Can use vitamin A analogues
101
What is the commonest overall leukaemia?
CLL
102
What is the main cell origin of CLL?
B cells (99%)
103
How does CLL present?
Often asymptomatic - often an incidental finding on blood film in elderly
May be anaemia, infection prone, weight loss, enlarged rubbery non tender lymph nodes
104
What anaemia does CLL produce?
Normocytic normochromic anaemia
105
What is seen on blood film in CLL?
Smudge/Smear cells
106
What is seen on electrophoresis in CLL?
Hypogammaglobulinaemia
107
What is Richters transformation of CLL?
Transformation into a high grade lymphoma
108
What fraction of patients do not progress in CLL?
1/3rd do not progress, 1/3rd progress in time, 1/3rd actively progressing
109
If CLL is actively progressing how is it treated?
Chemotherapy +/- stem cell transplant
110
What is the median survival of CLL?
6.5-12 years
111
What is CML?
Myeloproliferative disorder
112
What is a genetic cause for CML?
Philadelphia chromosome t(9;22)
113
How does CML present?
Chronic and insidious weight loss, tiredness, fevers, sweats, gout, MASSIVE splenomegaly
114
What anaemia does CML give patients?
Normocytic normochromic anaemia
115
How is CML treated?
Tyrosine kinase inhibitors - IMANTINIB
116
What is a blast crisis in CML?
When maturation fails and blast cells accumulate resulting in AML
117
When are the two peaks of incidence of Hodgkins lymphoma?
15-30yrs and >55 years
118
How does Hodgkins lymphoma present?
Painless lymphadenopathy, alcohol induced pain, weight loss, fever, night sweats, SVC obstruction, Pel-Ebstein fever (cyclical)
119
What does an excisional lymph node biopsy and immunohistochemistry of Hodgkins lymphoma show?
Reed-Sternberg Cells ('mirror nuclei')
120
How is Hodgkins lymphoma treated?
Chemoradiotherapy
121
What is Non-Hodgkins lymphoma?
Any lymphoma except Hodgkins
122
What cell type is more common in Non-Hodgkins lymphoma?
B cell (90%) T cell (10%)
123
What is Non-Hodgkins lymphoma associated with?
Immunodeficiency
124
How does Non-Hodgins lymphoma present?
Enlargement of a group of lymph nodes, night sweats, weight loss, indigestion, raised ICP, cranial nerve palsies, hepatosplenomegaly
125
How is Non-Hodgkins lymphoma treated?
Watch & wait in low grade disease
Chemoradiotherapy (dependent on stage)
Monoclonal antibodies - RITUXIMAB
126
What is Burkitts lymphoma?
High grade B cell lymphoma of childhood
127
What genetic problem is Burkitts lymphoma associated with?
c-myc gene translocation t(8;14)
128
What appearance is seen on microscopy of Burkitts lymphoma?
Starry sky appearance
129
What are the 4 stages of normal haemostasis?
Vasoconstriction
Primary haemostasis
Secondary haemostasis
Fibrinolysis
130
What is primary haemostasis?
Formation of platelet plug
131
What is secondary haemostasis?
Formation of fibrin clot
132
What are vascular causes of failure of primary haemostasis?
Hereditary haemorrhagic telangastasia
| Henoch schonlein purpura
133
What is the inheritance of HHT/OslerWeberRendu?
Autosomal dominant
134
What are some platelet causes of failure of primary haemostasis?
Decreased production - aplastic anaemia, leukaemia, myeloma
| Increased destruction - ITP, SLE, hypersplenism, TTP
135
What is the inheritance of Von Willebrands disease?
Autosomal dominant
136
What is the role of Von Willebrand factor?
Platelet adhesion
| Binds to factor VIII
137
How does Von Willebrands disease present?
Bruising, epistaxis, menorrhagia
138
What does a clotting screen show for Von Willebrands disease?
Increased APTT
Increased bleeding time
Decreased factor VIII assay
139
How is Von Willebrands managed?
Vasopressins for minor bleeds
| VWF rich factor VIII concentrate for surgeries
140
What causes a failure of secondary haemostasis?
Single or mutliple clotting factor deficiencies
141
What are two causes of multiple clotting factor deficiencies?
Liver failure
| Vitamin D deficiency
142
What is haemophilia A?
Deficiency of factor VIII
143
How is haemophilia inherited?
X-linked recessive
144
How does haemophilia present?
Abnormally prolonged bleeding, haemoarthosis, haematomas
145
What is haemophilia B?
Deficiency of factor IX
146
What is haemophilia B also known as?
Christmas disease
147
What does a clotting screen from haemophilia A show?
Increased APTT
Normal PT
Normal bleeding time
Decreased factor VIII assay
148
What does a clotting screen for haemophilia B show?
Increased APTT
Normal PT
Normal bleeding time
Decreased factor IX assay
149
How is haemophilia A treated?
Factor VIII concentrate IV when bleeding. Prophylaxis if severe. Desmopressin prophylaxis if mild
150
How is haemophilia B treated?
Factor IX concentrates
151
What is disseminated intravascular coagulation?
Excessive and inappropriate activation of the haemostatic system. Initally causes thrombosis of small vessels then a bleeding tendency
152
What are some causes of DIC?
Sepsis, obstetric emergencies, drugs, shock
153
How do patients with DIC present?
Acutely ill, shocked, bleeding
154
How is DIC treated?
Treat underlying cause
Transfuse platelets and plasma
Replace fibrinogen
155
What does the prothrombin time evaluate?
Extrinsic pathway (I,II,V,VII,X)
156
What is a normal prothrombin time?
12-16 seconds
157
What does the activated partial thromboplastin time evaluate?
Intrinsic pathway (I, II, V, VIII, IX, X, XI, XII)
158
What is a normal APTT?
26-37 secs
159
What are the natural anticoagulants?
Antithrombin
Activated Protein C
Protein S
160
What are the vitamin K dependent factors?
II, VII, IX, X, protein C, protein S
161
What is Factor V Leiden?
A disease where there is a resistance to activated protein C`
162
How does factor V leiden present?
Premature, unusual and recurrent thromboses
163
What is antiphospholipid syndrome?
Acquired thrombophilia associated with recurrent thromboses, fetal loss and mild thrombocytopenia
164
How should an initial venous thrombotic event in antiphospholipid syndrome be treated?
Warfarin 6 months
| INR 2-3
165
How should recurrent venous thrombosis in APLS be treated?
Warfarin lifelong
| INR 2-3 (or 3-4 if the event occured while on warfarin)
166
How should arterial thrombosis in APLS be treated?
Warfarin lifelong
| INR 2-3
167
How does heparin work?
Potentiates antithrombin
168
How is unfractionated heparin monitored?
Check APTT
169
How is LMWH heparin monitored?
Anti-Xa assay
170
What is the antidote for heparin?
Protamine sulphate
171
How does warfarin work?
Blocks ability of vitamin K to carboxylate factors II, VII, IX, X
172
What is INR?
International normalised ratio - patients PT time/mean normal PT time
173
What is dabigatran?
Direct thrombin inhibitor
174
What are rivaroxiban/apixiban?
Direct factor X inhibitors
175
What is aspirin?
Platelet agonist - inhibits cyclo-oxygenase
176
What are clopidogrel/prasugrel/ticagrelor?
ADP receptor blockers
177
What is dipyridamole?
Phosphodiesterase inhibitor
178
What is abciximab?
GP IIb/IIa inhibitor that inhibits aggregation
179
What score is used in DVT and PE?
Wells Score
180
If a patient has 2 or more in the DVT Wells score what do you do?
Refer for proximal leg vein scan within 4 hours
| If cannot be done in 4 hours - d-dimer/interum dose of LMWH
181
If a patient had a DVT Wells score of 1 or less what do you do?
Do a d-dimer first
If positive -> proximal leg vein scan within 4 hours
If cannot be done within 4 hours - interum dose of LMWH
182
If a patient has a confirmed DVT, what should be done?
Start warfarin within 24 hours of diagnosis
| Continue LMWH for 5 days or until INR 2 or above for 24 hours
183
How long should a patient with a DVT stay on warfarin and at what target INR?
At least 3 months
| Target INR - 2.0-3.0
184
If a patient has a PE wells score above 4, what should be done?
Hospital admission for immediate CTPA
| If there is a delay --> start LMWH
185
If a patient has a PE Wells score of 4 or less, what should be done?
Arrange a d-dimer test
| If positive do immediate CTPA. If delay --> start LMWH
186
Once a PE is confirmed, what should be done?
Give warfarin within 24 hours
| Continue LMWH for 5 days or until INR 2 or above
187
How long should warfarin be given following a PE and at what target?
At least 3 months
| Target INR 2-3
188
What should pregnant women with PE be treated with?
LMWH until the end of pregnancy
189
What should patients with unprovoked PE/DVT be investigated for?
Thrombophilias
| Malignancy
190
How should a patient with a major bleed on warfarin be managed?
Stop warfarin
| Refer urgently for IV vitamin K and/or prothrombin complex concentrate or FFP
191
How should a patient with an INR above 8 be managed?
Stop warfarin
Give 0.5-1mg Vitamin K by IV injection or 5mg orally
Repeat in 24hrs if INR still high
Restart warfarin when INR below 5
192
How should a patient with an INR of 6-8 be managed?
Stop warfarin
| Restart warfarin when INR below 5
193
How should a patient with an INR below 6 but 0.5 above target be managed?
Stop or reduce warfarin
| Restart when INR below 5
194
How should a patient with an INR below 5 but is high be treated?
Dose reduction and omit a few doses
195
How should a patient with a low INR be managed?
Temporarily increase dose and give LWMH boosters
196
What dose change results in a change in INR of 1?
a 15% dose change causes INR to change by 1
197
What do Howell-Jolly bodies suggest?
Hyposplenism
198
Which type of Hodgkins lymphoma carries the worst prognosis?
Lymphocyte depleted
199
What antibody is warm haemolytic anaemia associated with?
IgG
200
What antibody is cold haemolytic anaemia associated with?
IgM
201
What metabolic complication may a blood transfusion cause?
Hyperkalaemia
202
What does a JAK2 mutation indicate?
Polycythaemia Rubra Vera
203
Why is polycythaemia rubra vera?
Myeloproliferative disorder causing increased red cell volume
204
What are features of polycythaemia rubra vera?
Hyperviscosity, itch after hot bath, splenomegaly, plethora, hypertension
205
What is multiple myeloma?
Neoplasm of bone marrow cells
206
What are clinical features of multiple myeloma?
Bone disease (pain, fractures), lethargy, infection, renal failure, hypercalcaemia
207
What is seen in the urine in myeloma?
Bence Jones Proteins
208
What are tear drop cells on blood film associated with?
Myelofibrosis
209
What is aplastic anaemia characterised by?
Pancytopenia and hypoplastic bone marrow
210
How does hereditary spherocytosis present?
Failure to thrive, jaundice, splenomegaly,
211
How is hereditary sphercytosis investigated?
Osmotic fragiltity test
212
How is hereditary spherocytosis managed?
Splenectomy
| Folate replacement
213
What is the commonest Hodgkins lymphoma?
Nodular sclerosing
214
What is the Hodgkins lymphoma with the best prognosis?
Lymphocyte predominant
215
What factor is the major constituent of cryoprecipitate?
Factor VIII
216
'Rouleaux formation'
Myeloma
217
'IgM paraprotein'
Waldenstroms macroglobulinaemia
