Finals Revision Haematology Flashcards
(115 cards)
1
Q
What are the Vitamin K dependent clotting factors?
A
Factors II, VII, IX, X
2
Q
What are the 3 natural anticoagulants?
A
Antithrombin
Protein C
Protein S
3
Q
How does LMWH act in anticoagulation?
A
It binds to antithrombin to potentiate it’s effect i.e. stops production of thrombin from prothrombin
4
Q
True / False: Patients who are being commenced on dabigatran require an initial loading with LMWH?
A
True
5
Q
True / False: Patients who are being commenced on a Factor Xa inhibitor e.g. Rivaroxaban require an initial loading with LMWH?
A
False - These patients do not require initial loading
6
Q
What should be given to patients on warfarin who have a ‘major bleed’?
A
Prothrombin complex concentrate e.g. Beriplex PLUS 5mg Vitamin K
7
Q
Why is Vitamin K given alongside prothrombin complex concentrate in patients on warfarin who have a ‘major bleed’?
A
Clotting factors in prothrombin complex concentrate only have a short half-life so although they can reverse the effects of warfarin quickly, they should be given along with Vitamin K for prolonged effects.
8
Q
What is given to patients on warfarin who have a ‘non-major bleed’?
A
Vitamin K 1-3mg IV, combined with dose reduction or discontinuation of warfarin
9
Q
What is the management of a patient on warfarin with INR more than 5 who is not bleeding?
A
Withhold 1-2 doses of warfarin and recommence at a reduced dose. Investigate for the cause of the raised INR.
10
Q
What is the management of a patient on warfarin with INR more than 8 who is not bleeding?
A
Vitamin K 1-5mg orally
11
Q
In which condition might you get a ‘pepper-pot skull’ appearance on x-ray?
A
Myeloma
12
Q
Give some clinical features of myeloma
A
Bone marrow failure - Tiredness, fatigue, thrombocytopenia
Recurrent infections
Bone pain
Hypercalcaemia - Polyuria, polydipsia
Renal failure - Due to light chain deposition in kidney
Amyloidosis
Plasma cytomas
13
Q
What investigations might you perform in suspected myeloma?
A
FBC - Normocytic, normochromic anaemia Blood film - Rouleaux formation ESR Calcium U+E Serum electrophoresis - 'M' component signifies immunoglobulins Urine - Bence Jones protein X-Ray - 'Pepper-pot skull', fractures Bone marrow - Lots of plasma cells
14
Q
What is another name for a Burr cell?
A
Echinocyte
15
Q
What is another name for a Spurr cell?
A
Acanthocyte
16
Q
What is a schistocyte and in which conditions might it be seen on a blood film?
A
A fragmented RBC which is broken up as it travels through intravascular fibrin. Seen microangiopathic haemolytic anaemia, mechanical valves etc.
17
Q
In which condition might you see a Heinz body? Why?
A
G6PD deficiency - Heinz bodies are denatured haemoglobin caused in oxidative stress
18
Q
In which conditions might you see a Burr cell (echinocyte) on blood film?
A
Uraemia
Liver damage
Artefact
19
Q
What is ‘Rouleaux formation’ and when is it seen?
A
Clumped RBCs, seen in multiple myeloma, chronic liver disease, lymphoma, and chronic inflammatory conditions
20
Q
What is typically seen on a blood film in a patient with hyposplenism?
A
Howell-Jolly bodies
21
Q
What is a Howell-Jolly Body?
A
A nuclear remnant from RBCs which is normally removed when the RBC matures
22
Q
What is the appearance of a Spurr cell (acanthocyte)?
A
Spiculated RBC
23
Q
When might you see a Spurr cell (acanthocyte)?
A
Liver disease
Post-splenectomy
24
Q
When might you see a Bite cell?
A
G6PD deficiency
Oxidative drugs
Unstable haemolytic states
25
Give 2 things you might find on blood film of a patient with G6PD deficiency
Signs of oxidative stress:
- Bite cell
- Heinz body
26
What is meant by anistocytosis?
Large size of RBCs
27
PT i.e. Prothrombin time (and INR) represent the intrinsic or extrinsic pathway in the clotting cascade?
Extrinsic
28
APTT (Activated partial thromboplastin time) represents the intrinsic or extrinsic pathway in the clotting cascade?
Intrinsic
29
Prolonged PT but normal APTT might be due to what?
Isolated Factor VII deficiency (rare)
Warfarin use or Vitamin K deficiency
Liver disease
30
What results would you expect to see in the clotting profile of a patient with Haemophilia A?
Normal PT
Prolonged APTT
Reduced Factor VIII
31
What does von Willebrand's Factor do?
1) Brings platelets into contact with exposed subendothelium
2) Platelet aggregation
3) Binds to Factor VIII preventing it's destruction
32
What results would you expect to see in the clotting profile of a patient with von Willebrand's disease?
```
Normal PT/INR
Prolonged APTT
Low vWF
Low Factor VIII
Bleeding time prolonged
```
33
What are the clinical features of von Willebrand's disorder?
Bleeding - mucosal, menorrhagia, epistaxis
| Bruising
34
Which clotting factor is deficient in Haeophilia B?
Factor IX
35
What are the 2 'acute' haematological malignancies?
Acute myeloid leukaemia
| Acute lymphoblastic leukaemia
36
Give 3 myeloproliferative disorders
Myelofibrosis
Polycythaemia rubra vera
Essential thrombocytosis
37
What are the 3 types of chronic lymphoid haematological malignancies?
Lymphoma
Chronic lymphcytic leukaemia
Myeloma
38
What are the 3 types of chronic myeloid haematological malignancies?
Chronic myeloid leukaemia
Myelodysplasia
Myeloproliferative disorders
39
Give some symptoms of leukaemia
Generalised: Tiredness, recurrent infections, bruising, bleeding
CNS infiltration: Cranial nerve IV and VII palsies, seizures
Splenomegaly
Hepatomegaly
Lymphadenopathy
40
Which cells are typical of acute leukaemias?
Blast cells
41
Which leukaemia typically affects children?
Acute lymphoblastic leukaemia
42
Auer rods are associated with which haematological malignancy?
Acute myeloid leukaemia
43
Children with Down's Syndrome are at higher risk of which haematological malignancy?
Acute myeloid leukaemia
44
Compare blood film findings in acute myeloid Vs. acute lymphoblastic leukaemias
AML:
- Blast cells with some cytoplasm
- Auer rods
- Stain positive
ALL:
- Blast cells with no cytoplasm
- Stain negative
45
How long is the treatment for acute lymphoblastic leukaemia?
```
Boys = 3 years (due to infiltration of testes)
Girls = 2 years
```
46
Orchidomegaly is typical of which haematological malignancy?
Acute lymphoblastic leukaemia
47
Gum hypertrophy is associated with which haematological malignancy?
Acute myeloid leukaemia
48
The Philadelphia chromosome is associated with which haematological malignancy?
Chronic myeloid leukaemia
49
What is the Philadelphia chromosome?
Translocation between chromosomes 9 and 22...BCR-ABL
50
Give 5 causes of massive splenomegaly
2 x haematological: Chronic myeloid leukaemia, myelofibrosis
2 x infective: Malaria, Leuschmaniasis
1 x lysosomal storage disorder
51
Infliximab is used to treat which haematological malignancy?
Chronic myeloid leukaemia
52
What is infliximab?
A tyrosine kinase inhibitor - used in the treatment of chronic myeloid leukaemia
53
What are the treatment options for chronic myeloid leukaemia?
- Hydroxycarbamide
- Infliximab
- Stem cell transplant
54
What is the Richter transformation?
Development of aggressive lymphoma in patients with chronic lymphocytic leukaemia (usually diffuse large B-cell lymphoma)
55
What is the most common type of leukaemia in adults?
Chronic lymphocytic leukaemia
56
In which haematological malignancy might you see 'smudge' cells?
Chronic lymphocytic leukaemia
57
List some features of lymphoma
Lymphadenopathy
General malaise, tiredness
Weight loss
Fever
58
Which is more aggressive, Hodgkin's or non-Hodgkin's lymphoma?
Non-Hodgkins is more aggressive
59
Reed-Sternberg cells are associated with which type of lymphoma?
Hodgkin's
60
EBV is associated with an increased risk of which lymphoma?
Hodgkin's
61
Which type of Hodgkin's lymphoma has the worst prognosis?
Lymphocyte depleted
62
List 5 types of Hogkin's lymphoma
```
Nodular sclerosing
Mixed cellularity
Lymphocyte rich
Lymphocyte depleted
Nodular lymphocyte predominant
```
63
What are the 2 types of high grade non-Hodgkin's lymphoma?
Burkitt's lymphoma
| Diffuse large B cell lymphoma
64
What are the 2 types of low grade Hogkin's lymhoma?
Follicular
| Marginal
65
What are the 'B' symptoms in lymphoma
More than 10% weight loss in 6 months
Unexplained fever more than 38 degrees
Night sweats
66
How is Hodgkin's lymphoma staged?
I = Confined to single lymph node region
II = 2 or more nodal areas on same side of the diaphragm involved
III = Nodes on both sides of diaphragm involved
IV = Spread beyond lymph nodes e.g. liver, bone marrow
Each stage is either 'A' or 'B' based on the absence or presence of B symptoms
67
What is the treatment for Hodgkin's lymphoma?
```
Chemotherapy: ABVD
Adriamycin
Bleomycin
Vinblastine
Dacarbazine
```
68
What is the definition of a myeloproliferative disorder?
Malignant proliferation of a particular type of myeloid stem cell in the bone marrow, which results in a dramatically increased amount of that cell
69
What is the mutation in polycythaemia rubra vera?
JAK2 mutation, which causes malignany proliferation of RBC, WBC and platelets
70
Give some features of polycythaemia rubra vera
May be asymptomatic and picked up on routine FBC test
Symptoms of hyperviscosity: Headache, dizziness, visual disturbance
Itch after a hot bath
Erythromelalgia
Facial plethora
Splenomegaly
Symptoms of arterial and venous thrombosis
71
How might you distinguish polycythaemia rubra vera from other causes of polycythaemia?
In polycythaemia, there are increased numbers of WBCs and platelets, not just RBCs
72
What is the treatment for polycythaemia rubra vera?
Venesections to keep haematocrit low
73
What mutation is present in essential thrombocytosis?
JAK-2 mutation (same as for polycythaemia rubra vera) which causes proliferation of megakaryocytes so there is an increase in platelets
74
What is erythromelalgia?
Burning sensation in extremities
75
List some features of essential thrombocytosis
```
Arterial and venous thrombosis
Microvascular occlusion headache
Atypical chest pain
Light headedness
Erythromelalgia
```
76
What is the treatment for essential thrombocytosis?
Aspirin daily
| Hydroxycarbamide if over 60 or previous thrombosis
77
What happens in myelofibrosis?
Bone marrow is replaced with connective tissue
78
What is the difference between a myeloproliferative disorder and myelodysplasia?
Myelodysplasia = There is increased production of abnormal cells
Myeloproliferative disorder = There is malignant proliferation of a cell type but the cells are normal
79
What are the features of myelofibrosis?
General features of bone marrow failure:
- Malaise, weight loss, weakness
- Bleeding
- Recurrent infections
- Hepatomegaly
- Massive splenomegaly (due to extramedullary haematopoiesis)
- Anaemia
80
What would bone marrow biopsy show in myelofibrosis?
A dry tap i.e. unable to obtain a sample
Trephine biopsy shows fibrosis
81
What is the management of myelofibrosis?
```
Transfusions
Hydroxycarbamide
Splenic irradiation
Splenectomy
Stem cell transplant
```
82
Teardrop cells are typical of ....?
Myelofibrosis
83
Give some causes of microcytic anaemia
Iron deficiency anaemia
Thalassemia
Sideroblastic anaemia
Lead poisoning
84
How might macrocytic anaemia be divided up?
Megaloblastic
| Non-megaloblastic
85
What are the causes of megaloblastic macrocytic anaemia?
B12 and folate deficiency
86
What are the causes of non-megalobastic macrocytic anaemia?
```
Alcohol
Chronic liver disease
Myelodysplasia
Hypothyroidism
Drugs
Metabolic disorder
```
87
How might normocytic anaemias be divided up?
Haemolytic
| Non-haemolytic
88
Give some causes of a non-haemolytic normocytic anaemia
```
Anaemia of chronic disease
Aplastic anaemia
Kidney disease
Pregnancy (due to increased volume)
Lymphoma
Acute blood loss
```
89
What do iron studies show in iron deficiency anaemia?
Low iron
High TIBC
Low ferritin
Low transferrin saturation
90
Where is Vitamin B12 absorbed?
Terminal ileum
91
Approximately how long do the body's own stores of Vitamin B12 last?
1-2 years
92
What is the significance of intrinsic factor?
Free Vitamin B12 becomes bound to intrinsic factor to be absorbed at the terminal iluem. A lack of intrinsic factor (in pernicious anaemia) will mean less Vitamin B12 is absorbed.
93
Give some clinical features of B12 deficiency
```
Anaemia symptoms e.g. tiredness
'Yellow tinge' to skin
Glossitis
Angula cheilitis
Neurological involvement: Paraesthesia, depression, psychosis, dementia
```
94
What tests are specific for pernicious anaemia?
Parietal cell antibodies
| Intrinsic factor antibodies
95
Why shouldn't you give folic acid on it's own to a B12 deficient patient?
B12 deficiency worsens folate deficiency and precipitates subacute combined degeneration of the cord
96
How long do the body's own stores of foic acid last?
About 4 months
97
What symptoms are seen in subacute combined degeneration of the cord?
```
Sensory symptoms:
- Loss of proprioception, leads to ataxia
- Loss of vibration sense
Motor symptoms (UMN and LMN)
- Reduced knee and ankle reflexes
- Upgoing plantars
Loss of vision due to optic atrophy
Pain and temperature sensation intact
```
98
What is the chronic management of sickle cell disease?
- Analgesia
- Avoid factors which might preciptate a crisis e.g. cold
- Hydroxycarbamide if frequent crises
- Pneumococcal and influenza vaccination, plus daily penicillin due to hyposplenism
99
What is the treatment for thalassemia?
Regular blood transfusions to maintain Hb
| Iron chelators to reduce iron overload e.g. desferrioxamine
100
What might be seen on blood film of a patient with iron deficiency anaemia?
Microcytic, hypochromic red blood cells
Pencil cells
Target cells
101
How might haemolytic anaemia be divided up?
Inherited Vs. acquired causes
102
List some inherited causes of haemolytic anaemia
Haemoglobinopathies: Sickle cell disease, thalassemia
Membrane defects: Hereditary spherocytosis or elliptocytosis
Enzyme defects: G6PD deficiency, pyruvate kinase deficiency
103
What is the inheritance pattern of glucose-6-phosphate dehydrogenase deficiency?
X-linked recessive
104
Give some drugs which might precipitate an oxidative crisis in G6PD deficiency
Ciprofloxacin
Sulphonamides
Sulphonylureas
Sulfasalazine
105
What is the inheritance pattern of hereditary spherocytosis?
Autosomal dominant
106
What is the diagnostic test for hereditary spherocytosis?
Osmotic fragility test
107
How might acquired haemolytic anaemias be divided up?
Immune
| Non-immune
108
What is the key test in diagnosing immune haemolytic anaemia?
Immune haemolytic anaemias will cause a positive Coombs (direct antibody) test
109
Compare warm and cold autoimmune haemolytic anaemias
Warm:
- Extravascular haemolysis i.e. in spleen
- Treatment = Steroids, immunosuppression, splenectomy
Cold:
- Complement-mediated intravascular haemolysis
- Infections e.g. EBV might increase antibodies
- Responds less well to steroids
- Treatment is to keep warm
110
List some causes of non-immune haemolytic anaemia
Infection e.g. malaria
Paroxysmal nocturnal haemoglobinuria
Microangiopathic haemolytic anaemia
Heart valve disorders
111
What is the triad of features in haemolytic uraemic syndrome?
Haemolytic anaemia (jaundice, low Hb)
Renal failure
Thrombocytopenia
112
List some causes of thrombocytopenia
Impaired production:
- Bone marrow failure, including in leukaemia, myeloma
- Megaloblastic anaemias
- Aplastic anaemia
- Myelosuppression e.g. myelodysplasia, myelofibrosis
Increased destruction:
- Non-immune: TTP, DIC, haemolytic uraemic syndrome, sequestration e.g. in hypersplenism
- Primary immune i.e. ITP
- Secondary immune i.e. SLE, CLL, viruses, drugs
113
What is the treatment for immune thrombocytopenic purpura?
- If platelets over 30, might not need treatment
- Prednisolone
- Intravenous immunoglobulin
- Rituximab
- Splenectomy
114
Why shouldn't you give platelets to a patient with immune thrombocytopenic purpura?
It's an autoimmune condition so antibodies to the platelets surface antigens would cause any transfused platelets to be consumed so it's pointless administering them
115
What are the 5 key features of thrombotic thrombocytopenic purpura?
```
Thrombocytopenia
Microangiopathic haemolytic anaemia
Neurological disturbance
Fever
Renal failure
```
