Haematology Flashcards
1
Q
Differentiate the causes of megaloblastic and non-megaloblastic macrocytic anaemia
A
Megaloblastic: B12/folate deficiency (pregnancy, old age, veganism)
Non-megaloblastic: liver disease/ alcoholism/ multiple myeloma
2
Q
Recall the 2 key symptoms and the 3 key signs of any anaemia
A
Symptoms: lethargy, breathlessness
Signs: pallor, tachycardia, breathlessness
3
Q
Recall 3 signs of pernicious anaemia that might be present in addition to the normal signs of anaemia
A
Glossitis
Angular stomatitis
Mild jaundice
4
Q
What signs of B12 deficiency may be present alongside an anaemia?
A
Peripheral neuropathy
Optic atrophy
Dementia
5
Q
How are LFTs commonly deranged in macrocytic anaemia and why?
A
High bilirubin
Ineffective erythropoiesis, haemolysis
6
Q
What blood tests are done to detect B12 and folate levels?
A
Serum cobalamin
Red cell folate
7
Q
As well as macrocytic erythrocytes, what can be seen on a blood film in megaloblastic anaemia?
A
Hypersegmented neutrophils
8
Q
What test is done to diagnose pernicious anaemia?
A
Schilling test
9
Q
Recall the management of pernicious anaemia
A
IM hydroxycobalamin for life
10
Q
What is the most likely cause of a microcytic anaemia?
A
Iron deficiency
11
Q
Recall 4 causes of iron deficiency
A
GI blood loss
Small bowel disease
Vegan
Pregnancy
12
Q
What size are the red cells in anaemia of chronic disease?
A
Microcytic
13
Q
In what sort of anaemia may the patient develop brittle nails and hair?
A
Iron deficiency anaemia
14
Q
What might an FBC show in microcytic anaemia?
A
Low Hb
Low MCV
High reticulocytes
15
Q
Recall the iron, TIBC and ferritin seen in iron deficiency anaemia
A
Iron: low
TIBC: high
Ferritin: low
16
Q
Does thalassaemia produce a microcytic or a macrocytic anaemia?
A
Microcytic
17
Q
Systematically recall some causes of normacytic anaemia
A
Decreased production: vit B2/6 deficiency, ACD, aplastic anaemia
Increased destruction: haemolysis
Increased blood volume: fluids, pregnancy
18
Q
Define aplastic anaemia
A
A pancytopaenia due to fewer haemotopoietic precursors
19
Q
What symptoms and signs of aplastic anaemia will not be present in any other aetiology of anaemia?
A
Symptoms of thrombocytopaenia Purpura Easy bruising Epistaxis Petechiae Also multiple infections due to leukocytopaenia
20
Q
What are the 2 different types of disseminated intravascular coagulation?
A
Acute overt
Chronic non-overt
21
Q
Differentiate the pathophysiology of acute overt and chronic non-overt disseminated intravascular coagulation
A
Acute overt: bleeding –> depletion of platelets and clotting factors
Chronic non-overt: thromboembolism –> generalised activation of coagulation system
22
Q
What sort of infection is most likely to cause disseminated intravascular coagulation?
A
Gram negative sepsis
23
Q
Describe the pathophysiology of DIC
A
Endothelial damage –> tissue factor release
Tissue factor activates thrombin
Thrombin depletes clotting factors and platelets
Thrombin activates fibrinolysis
Fibrinolysis releases fibrin, which occludes the microvasculature
24
Q
Which patients are particularly at risk of disseminated intravascular coagulation?
A
Pretty much all severely unwell patients will get
25
Differentiate the signs that are present in acute and chronic disseminated intravascular coagulation
Acute: purpura, petechiae, ecchymoses, epistaxis
Chronic: signs of DVT/ arterial thromboses
26
Describe the FBC of someone with disseminated intravascular coagulation
Low Hb and platelets
27
What should be tested for alongside FBC in the blood in suspected disseminated intravascular coagulation
APTT (will be high)
| FDP (will also be high)
28
Recall the pathophysiology of haemachromatosis
GENETIC increase in iron absorption, iron accumulates in tissues, organs damaged
29
Recall some late symptoms of haemachromatosis
```
T2DM
Bronzed skin
Hepatomegaly
Cirrhosis
Hypogonadism
Cardiac arrhythmias/ cardiomyopathy
```
30
Recall the results of haematinic investigations in haemachromatosis
Ferritin: high
Transferrin: low
[Transferrin]: high
TIBC: low
31
Systematically recall possible causes of haemolytic anaemia
1. Hereditary
a) membrane defects - elliptocytosis, hereditary spherocytosis
b) haemaglobinopathies: thalassaemia, sickle-cell disease
c) metabolic defects: G6PD deficiency, PKU deficiency
2. Acquired
Infection: sepsis, malaria
Trauma
Drugs
Autoimmune
32
Recall 2 symptoms of haemolytic anaemia that are not present in other types of anaemia
Jaundice (due to increased red cell breakdown)
| Haematuria (due to haemolysis)
33
Is haemolytic anaemia macrocytic or microcytic?
Macrocytic
34
Recall 3 abnormalities found on urinalysis in haemolytic anaemia
1. High urobilinogen
2. Haemaglobinuria
3. Haemosiderinuria
35
What specific test is done for auti-immune haemolytic anaemia?
Direct Coomb's test
36
What are the 2 categories of haemolytic uraemic syndrome
D+ (with diarrhoea, usually children)
| D- (no prodromal illness)
37
Define haemolytic uraemic syndrome
Triad of microangiopathic haemolytic anaemia, thrombocytopaenia and acute renal failure
38
Explain the pathophysiology of HUS and TTP
Endothelial damage --> platelets aggregate
1a. Platelet aggregation --> thrombi formation --> promotion of intravascular haemolysis
1b. Platelet aggregation --> loads of vWF released --> small vessel thrombosis
Small vessel thrombi most likely to affect afferent glomerular arterioles which is why HUS + TTP --> renal failure
39
What is the difference between thrombotic thrombocytopaenic purpura and haemolytic uraemic syndrome
TTP has extra clinical manifestation of fever and CNS signs
40
Which pathogen is the most common cause of HUS and where it is usually found?
E Coli 0157
| Contaminated water
41
Recall the symptoms of HUS
General sickness/ malaise
GI symptoms: bloody diarrhoea, severe colic
Renal symptoms: oligouria, haematuria
42
Describe the FBC results for someone with HUS and TTP
Normacytic anaemia
High neutrophils
VERY LOW platelets
43
What would an ABG show in HUS?
Lactic acidosis
44
What would a blood film show in HUS?
High reticulocytes and schistocytes
45
Recall 3 symptoms of haemophilia
1. Haemarthroses
2. Haematuria
3. Deep bleeding
46
Recall 2 investigations that should be done in suspected haemophilia
Clotting factor assay
| APTT
47
What is immune thrombocytopaenic purpura?
Immune destruction of platelets
48
What is the antibody that is implicated in immune thrombocytopaenic purpura?
Anti-GpIIb/ anti-GPIIIa
49
Recall the general signs/ symptoms of ITP
Easy bruising, epistaxis, visible purpura, menorrhagia
50
What will the result of a clotting screen be in ITP?
Normal
51
Why is it important to do a blood film in suspected ITP?
Need to rule out pseudothrombocytopaenia
52
What is the most common malignancy of childhood?
Acute lymphoblastic leukaemia
53
Systematically recall the symptoms of acute lymphoblastic leukaemia
Due to reduced synthesis of blood cells: anaemia, easy bruising, recurrent infections
Due to organ infiltration: tender bones, lymphadenopathy, meningism
54
Systematically recall the signs of acute lymphoblastic leukaemia
Due to bone marrow failure: bruising, pallor
| Due to tissue infiltration: lymphadenopathy, hepatosplenomegaly, retinal haemorrhage + papilloedema, testicular swelling
55
What sort of anaemia results from acute lymphoblastic leukaemia?
Normacytic normachromic
56
Which enzyme is elevated in the blood in cancer patients?
LDH
57
Recall one blood test result that is a poor prognostic factor for acute lymphoblastic leukaemia
Uric acid high
58
Recall 4 appropriate investigations in suspected acute lymphoblastic leukaemia
1. Bloods (FBC, U&Es, LDH)
2. Blood film and BM aspirate
3. Immunophenotyping
4. CXR to look for mediastinal lymphadenopathy
59
Recall some signs of acute myeloblastic leukaemia that result from tissue infiltration, and aren't seen in acute lymphoblastic leukaemia
Gum swelling/ bleeding
Skin rashes
CNS involvement: headache and diplopia
60
What is the expected white cell count in acute myeloblastic leukaemia?
Can be variable
61
In which demographic is acute myeloblastic leukaemia most common?
The elderly: incidence increases with age
62
Define chronic lymphocytic leukaemia
Progressive accumulation of functionally incompetent lymphocytes which have monoclonal origin
63
What causes lymphocytes to accumulate in CLL?
Failure of apoptosis
64
Which type of leukaemia is most likely to be asymptomatic?
CLL
65
Recall 2 signs of CLL on examination
Non-tender lymphadenopathy
| Hepatosplenomegaly
66
Recall 2 findings on the blood film in CLL
Small lymphocytes
| Smudge cells
67
With which other disease does CLL overlap?
Non-hodgkn's lymphoma
68
What type of cell is hyperproliferating in CML?
Granulocyte precursors
69
What translocation occurs to produce the Philadelphia chromosome, and what sort of cancer does this predispose to?
Chromosomes 9 and 22
BCR-ABl fusion
Chronic myeloid leukaemia
70
In what sort of pateints is CML most likely to occur?
Men (4 x more common than in women)
71
Recall the 3 phases of CML
Stable chronic (4-6 years)
Accelerated (3-6 months)
Acute (blast transformation)
72
Recall a sign that is present in 90% of CML patients
Splenomegaly
73
What is the most likely aetiology of hypercobalaminaemia?
Haematological neoplastic cause
74
Define Hodgkin's lymphoma
Neoplasm of lymphoid cells originating in the lymph nodes
75
How is Hodgkin's lymphoma diagnosed?
Histopathologically, based on presence of REED-STENBERG cells
76
What are reed-stenberg cells and in which disease do they appear in the blood film?
Bi-nucleate lymphocytes
| Hodgkin's lymphoma
77
What is the main symptom of Hodgkin's lymphoma?
Painless enlarging neck mass, that may become painful following alcohol consumption
78
How are the lymph nodes described in lymphoma?
Painless, firm and rubbery
79
What staging system is used to stage lymphoma?
Ann Arbor
80
What sign may be present on the skin of someone with hodgkin's lymphoma/ non-hodgkin's lymphoma
Hodgkin's: excoriations
| Non-Hodgkin's: rashes eg. mycosis fungoides
81
In which types of cancer are ESR and CRP likely to be elevated?
Hodgkin's/non-Hodgkin's lymphomas
82
What % of Non-Hodgkin's lymphomas involve B cells, T cells and NK cells?
85% B cells
| 15% T and NK cells
83
Recall one viral cause of non-Hodgkin's lymphoma
EBV virus, causing Burkitt's lymphoma
84
Recall 3 places where a mass may appear due to non-Hodgkin's lymphoma
Neck
Groin
Axilla
85
Which type of lymphoma is most common?
Non-Hodgkin's
86
Recall one common electrolyte imbalance in non-Hodgkin's lymphoma
Hypercalcaemia
87
Recall one difference in the FBC between Hodgkin's lymphoma and non-Hodgkin's lymphoma
In Hodgkin's = neutrophilia, in non-Hodgkin's = neutropaenia
88
What is multiple myeloma a proliferation of?
Plasma cells
89
Recall 2 symptoms of multiple myeloma
Symptoms: bone pain, recurrent infection
90
Recall 3 signs of multiple myeloma
Heart problems (tachycardia, flow murmur, heart failure)
Carpal tunnel
Macroglossia
(why???)
91
What is a key finding on the blood film in multiple myeloma?
Rouleaux
| = suggestive of high protein
92
Why is an X ray important to do in suspected multiple myeloma?
Osteolytic lesions will be seen
93
What is myelodysplasia?
A group of syndromes where the immature blood cells in the blood do not mature normally causing chronic pancytopaenia
94
Recall the 5 subgroups of myelodysplasia
Refractory anaemia
Refractory anaemia and ringed suderoblasts
Refractory anaemia with excess blasts
Refractory anaemia with excess blasts in transformation
Chronic myeloMONOCYTIC leukaemia
95
What is the most common cause of secondary myelodysplasia?
Chemotherapy/ radiotherapy
96
What are the symptoms of a myelodysplastic syndrome?
May be asymptomatic, or exhibit similar symptoms to any blood neoplasm due to loss of functional mature blood cells - anaemia, recurrent infections, easy bruising
97
Describe the epidemiology of myelofibrosis
Really rare
98
What is myelofibrosis?
Disorder of haematopoietic stem cells characterised by progressive bone marrow fibrosis
99
What is the main histopathological finding on the blood film of someone with myelofibrosis?
Increased number of abnormal megakaryocytes
100
What sign will always be present in myelofibrosis and why?
Splenomegaly, due to extramedullary haematopoiesis
101
Define polycythaemia
Higher than normal concentration of haemoglobin
102
Differentiate between relative polycythaemia and polycythaemia vera
Relative - normal MCV and decreased plasma volume
| True - increased MCV
103
Differentiate the aetiology of primary and secondary polycythaemia
Primary: clonal proliferation of myeloid cells
Secondary: increased erythropoietin
104
Recall 5 symptoms of polycythaemia
```
Angina
Chorea
Gout
Headache
Visual changes
```
105
Recall 3 signs of polycythaemia
1. Red complexion
2. Conjunctional suffusion (redness)
3. 75% have splenomegaly
106
What investigation is done to differentiate between polycythaemia vera and relative polycythaemia?
Isotope dilution
107
Describe the erythropoietin level in polycythaemia vera and relative polycythaemia
Polycythaemia vera: low
| Relative polycythaemia: high
108
Differentiate sickle cell anaemia and sickle cell disease
SCA: homozygous HbS
SCD: heterozygous HbS and HbC OR HbS and Beta-thalassaemia
109
What is the gene mutation in HbS?
Glutamic acid replaced with valine at position 6 on beta globin gene
110
Recall 4 symptoms of sickle cell anaemia
1. Pneumococcal infection due to autosplenectomy
2. Abdominal and bone pain
3. CNS symptoms: fits and strokes
4. In children: dactylitis
111
Recall 2 signs of a sickle cell crisis
Acute chest syndrome
| Priapism
112
Recall 2 abnormal erythrocytes seen on a blood film of someone with sickle cell disease
Target cells
| Howel-Jolly bodies
113
Recall one medical treatment option for sickle cell
Hydroxyurea - induces HbF production
114
Which virus is especially dangerous to sickle cell patients and why?
Parvovirus B19
| Aplastic crisis
115
What is the life expectancy of someone with sickle cell anaemia?
50
116
What is the inheritance pattern of thalassaemia?
Autosomal recessive
117
Recall the 4 types of alpha thalassaemia
```
Number of gene deletions:
4 = HbBarts: fatal in utero
3 = HbH (causes anaemia and splenomegaly)
2 = alpha 0, no anaemia
1 = alpha +, no anaemia
```
118
Recall the 3 types of beta thalassaemia
Major: homozygous
Intermedia: causes a microcytic anaemia, with fewer alpha chains and more gamma chains
Trait: heterozygous and asymptomatic
119
Systematically recall the symptoms of beta thalssaemia
Due to anaemia: pallor, malaise, dyspnoea
Due to increased red cell lysis: jaundice
Due to extramedullary erythropoiesis: splenomegaly
Frontal bossing
120
Is the anaemia caused by thalassaemia macro/microcytic?
Microcytic
121
Where is vitamin B12 absorbed following consumption?
Terminal ileum
122
What cofactor is required for Vit B12 absorption?
Intrinsic factor
123
What is the pathophysiology of pernicious anaemia?
Autoimmune condition directed against intrinsic factor - so Vit B12 can't be absorbed
124
Why does metformin have a side effect of anaemia?
Impairs Vit B12 absorption
125
Recall the symptoms of B12 deficiency
Anaemia symptoms
Palpitations
Headache
Neurological symptoms - paraesthesia and visual disturbance
126
What would be seen on a blood film of someone with vit B12 deficiency?
Hypersegmented neutrophils
127
Which 2 antibodies are implicated in pernicious anaemia?
Anti-IF
| Anti-parietal cell
128
What type of bleeding is exhibited in Von Willebrand disease?
Mucocutaneous
129
Differentiate the 3 different types of VWF disease
Type 1: depleted volume of well-functioning VWF
Type 2: adequate volume of ill-functioning VWF
Type 3: NO VWF
130
Recall 3 symptoms of VWF disease
Easy bruising
Epistaxis
Blood in stools/ urine
