Haem Misc. Flashcards
(169 cards)
1
Q
What is Anaemia
A
Reduced red blood cells
2
Q
Define anaemia for men and women in terms of Hb and Hct
A
Men –> Hb < 130 g/l ; Hct 0.38 - 0.52
Women –> Hb < 120 g/l ; Hct 0.37 - 0.47
3
Q
What is haematocrit
A
The ratio of whole blood made up of red cells if a sample were left to settle
4
Q
Decreased production/ Increased destruction of RBC’s will give which different blood results
A
Increased destruction = increased reticulocytes
Reduced production = decreased reticulocytes
5
Q
What is the normal range of MCV
A
80 - 100 femtolitres
6
Q
<80 MCV means what
A
Microcytosis
7
Q
> 100 MCV means what
A
Macrocytosis
8
Q
Name the 2 main categories of hypochromic microcytic anaemias
A
Haem deficiency
Globin deficiency
9
Q
Name 2 causes for a lack of iron for erythropoiesis
A
Iron deficiency
Anaemia of chronic disease
10
Q
Name 3 possible causes of porphyrin synthesis problem
A
Lead poisoning
Sideroblastic anaemia
Pyridoxine responsive anaemias
11
Q
Name the main cause of Globin deficiency which leads to hypochromic, microcytic anaemia
A
Thalassaemia
12
Q
What is spurious macrocytosis
A
The size of the mature red cell is NORMAL, but the MCV is measured as being high
13
Q
What are the 2 broad categories of causes of macrocytosis
A
Megaloblastic
Non-megaloblastic
14
Q
What is a megaloblast
A
An abnormally large nucleated red cell precursor with an immature nucleus.
15
Q
What are the two most common causes of megaloblastic macrocytic anaemia
A
B12/Folate deficiency
16
Q
Which of B12 or folate should you replace first
A
B12
17
Q
What can B12/ folate deficiency cause
A
Symptoms/signs of anaemia
weight loss, diarrhoea, infertility
Sore tongue, jaundice
Developmental problems
18
Q
What can B12 deficiency cause specifically
A
Dorsal column abnormalities
Neuropathy
Subacute Combined Degeneration of Spinal cord
Dementia
19
Q
Blood film will show what 2 things in b12/folate deficiency
A
Hypersegmented (>5) neutrophils
Macro-ovalocytes
20
Q
Which red flag haem sign can b12/folate deficiency cause
A
Pancytopenia
21
Q
Where are B12 and folate absorbed
A
B12 –> ileum
Folate –> Duodenum, Jejunum
22
Q
Which autoimmune disease could be at play with B12 deficiency and which antibodies could you look for
A
Pernicious Anaemia
Anti- Gastric Parietal Cell (more sensitive)
Anti- Intrinsic Factor (more specific)
23
Q
FFP must be frozen within how many hours of collection
A
8 hours
24
Q
Which 3 criteria must be met by someone who is wanting to be a blood donor
A
Minimum 50kg
Must be able to spare 465mls of blood
Males ( >13.5 Hb) Females (>12.5 Hb)
25
How often can you give blood
Must wait 12 weeks between donations
| max 5 a year
26
What are the shelf lives of red cells, platelets and FFP
Red cells --> 35 days
Platelets --> 7 days
FFP --> 3 years
27
What temperatures are red cells, platelets and FFP kept at
RBC'S --> 4 degrees (give or take 2)
Platelets --> 22 degrees
FFP --> -30 degrees
28
The genes for alpha chains in Hb is on which chromosome
Chromosome 16
29
The gene for beta chains in Hb is on which chromosome
Chromosome 11
30
Alpha thalassaemias result from what kind of mutation
Deletion
31
Beta thalassaemias result from what kind of mutation
Point
32
What is HbH
Clumping together of Beta chains into a tetramer
33
The point mutation in sickle cell results in Glutamine being replaced by what
Valine
34
Name 4 long term treatments of sickle cell anaemia
Prophylactic penicillin
Vaccination
Folic acid
Hydroxycarbamide
35
What should you vaccinate against in someone with sickle cell anaemia/ hyposplenism
Meningooccus
Pneumococcus
H. Influenza
36
Which type of haemolysis is more common
Extravascular
37
Compare blood products in Intravascular and Extravascular haemolysis
Intravascular --> abnormal blood products
| Extravascular --> normal blood products in excess
38
Name 3 causes of intravascular haemolysis
```
Transfusion reaction
G6PD deficiency
Severe Malaria (Blackwater fever)
```
39
Pink urine which turns black on standing is a result of which biochemical abnormality in intravascular haemolysis
Haemoglobinuria
40
Which two abnormal blood products end up in the circulation in intravascular haemolysis
Haemoglobin
| Methaemalbumin
41
Which two abnormal blood products end up in the urine in intravascular haemolysis
Haemoglobin
| Haemosiderin
42
What is the mucosal iron transporter called
DMT-1
43
What is the serosal iron transporter called
Ferroportin
44
Which factor downregulates ferroportin and when is it produced
Hepcidin
| Produced in the liver in response to iron load and inflammation
45
Which genetic disease results in unchecked iron absorption and which gene is affected
Haemochromatosis
| HFE gene
46
How does Haemochromatosis present and how is it treated
Weakness, joint pain, impotence, cirrhosis, diabetes
| Tx --> Venesection
47
How does secondary iron overload occur and how can it be prevented
Repeated transfusions
| Iron-chelating drugs --> desferrioxamine
48
What is the antidote for Heparin reversal
Protamine Sulphate
49
What are the 5 stages of normal haemopoiesis
```
Self- renewal
Proliferation
Differentiation
Maturation
Apoptosis
```
50
What is the most common childhood cancer
Acute Lymphoblastic Leukaemia
51
What is Acute Lymphoblastic Leukaemia
Malignant disease of primitive lymphoid cells
52
Name 3 ways in which ALL could present
Marrow failure
Bone Pain
CNS/Testes involvement
53
What is a malignant disease of primitive myeloid precursors
Acute Myeloid Leukaemia
54
AML is more common in which age group
Older
| >60
55
Name a characteristic blood film appearance of AML and 2 characteristic symptoms
Auer rods on blood film
| DIC and Gum infiltration
56
What is a characteristic and specific symptom of Hodgkin's Lymphoma
Drinking alcohol causes chest pain
57
Hodgkin's lymphoma is associated with which infection
EBV infection
| E.g glandular fever
58
Which cells are seen in Hodgkin's lymphoma and what is their CD number
Reed Sternberg cells
| CD 30
59
What is the CD number of the cells seen in Non-Hodgkins lymphoma and which drug targets this
CD 20
| Rituximab
60
Which autoimmune disease will cause Hypo-cellular marrow
Aplastic anaemia
61
Hyper/Hyposplenism causes Pancytopenia
Hyper
62
Name 3 causes of Hypersplenism
Splenic congestion (CCF, Portal hypertension)
Felty's (Rheumatoid Arthritis, neutropenia, splenomegaly)
Splenic lymphoma
63
What are the 2 types of cytotoxic therapy
Cell- Cycle specific
| Non- Cell specific
64
Dose/Duration is more important for cell cycle-specific therapy
Duration is more important
65
Vinca alkaloids are which type of drugs and can cause which side effect
Mitotic spindle inhibitors
| Neuropathy
66
Alkylating agents are which type of cytotoxic therapy and can cause what
Non-cell cycle specific
| Can cause infertility
67
Which cytotoxic therapy can cause cardiomyopathy
Anthracyclines
68
Platinum derivatives such as Cisplatinum can cause what
Nephropathy
69
What drug regime should be initiated in neutropenic sepsis
Piperacillin/ Tazobactam
| + gentamicin if severe
70
Myeloproliferative disorders can be divided into which two groups
BCR-ABL 1 negative or positive
71
Which MPD comes under BCR-ABL 1 negative
Polycythaemia Rubra Vera
Essential Thrombocythaemia
Idiopathic Myelofibrosis
72
Which MPD comes under BCR-ABL 1 positive
Chronic Myeloid Leukaemia
73
What are the three stages of Chronic Myeloid Leukaemia
Chronic
Accelerated
blast crisis
74
What will be seen in the blood of someone with CML
Leucocytosis, Neutrophilia, Myeloid precursors
75
Which chromosome is a hallmark of CML and how does it come about
Philadelphia
| 9,22 translocation
76
What does the Philadelphia chromosome result in
Tyrosine kinase expression
77
Which drug inhibits Tyrosine Kinase and can be used to treat CML
Imatinib
78
How does Essential Thrombocythaemia result in acquired vWF deficiency
Excess abnormal platelets mop up all the available vWF
79
How is Essential Thrombocythaemia treated
Aspirin
| Hydroxycarbamide
80
JAK2 mutation is associated with which MPD
Polycythaemia Rubra Vera
81
Which MPD will show leukoerythroblastic blood film with teardrop shaped RBC's
Idiopathic Myelofibrosis
82
What is a unique symptom of Polycythaemia Rubra Vera
Aquagenic Itch
83
How can Polycythaemia Rubra Vera be treated
JAK2 inhibitors
84
How can Idiopathic Myelofibrosis be treated
Allogeneic stem cell transplantation
JAK 2 inhibitors
Splenectomy
85
Which 3 systems are activated in DIC
Complement
Coagulation
Kinin
86
What are the 4 most common causes of DIC
Hypovolaemic Shock
Sepsis
Malignancy
Obstetric emergency
87
Asides from treating the underlying cause, what else can be done for DIC
Fresh Frozen Plasma
| Contains clotting factors
88
How is haemophilia inherited
X-linked recessive
89
Which factors are deficient in Haemophilia A and B and which one is more common
A --> VIII (more common)
| B --> IX
90
Which clotting test will be abnormal in Haemophilia
isolated prolonged APTT
91
How will Haemophilia present
Recurrent Haemarthroses
Soft tissue bleeds
Prolonged bleed after surgery
92
Name 3 examples of naturally occurring anti-coagulants
Serine protease inhibitors --> AT III
| Protein C and S
93
Protein C and S inhibit which factors
V, VII, IX, Xa
94
What is factor V Leiden
An autosomal dominant disease where a variant of factor V which can't be inhibited very well by protein C is made
95
Which receptors do platelets bind to each other via and which drug can block these
Glycoprotein IIb/IIIa
| Abciximab
96
How is INR calculated and how can the results be interpreted
```
INR = [PTpatient ÷ MNPT]ISI
>1.5 = bleeding risk
```
97
Howell- Jolly bodies are seen in which condition
Hyposplenism
98
What are Heinz bodies and when can they be seen
Clumps of denatured Hb
| Seen in intravascular haemolysis (decreased haptoglobin)
99
In which state can iron bind O2 in haem
Fe2+
100
How is iron kept in the Fe2+ state
NADH from glycolysis
101
What are the 3 methods of Co2 transport
Dissolved
Bound to Hb
As bicarbonate
102
What is the problem in Sideroblastic Anaemia
Porphyrin Ring disturbance
103
Uridine is changed to what in the Folate cycle
Thymidine
104
Which cycle is b12 involved in
Methionine cycle
105
How is pernicious anaemia treated
b12 injections + folate tablets for life
| Transfusions if severe
106
Name 4 non-megaloblastic causes of macrocytosis and which one of these is associated with anaemia
Liver failure
Alcohol
Hypothyroidism
Marrow failure --> associated with anaemia
107
Where and how are the cells break down in a delayed transfusion reaction
In spleen by macrophages
108
How are thalassaemias diagnosed
HPLC
109
Which disease are those with thalassaemia slightly less at risk of
Malaria
110
Why does splenomegaly occur in thalassaemia
Extramedullary haematopoiesis
111
Define warm and cold autoimmune haemolysis
Warm --> IgG
| Cold --> IgM
112
Which lymphoma is basically incurable and why
Low-grade B cell NHL
| Slow growing so chemo doesn't work
113
How can you diagnose Fanconi Anaemia
Chromosome fragility testing
114
What is paraprotein
Monoclonal Immunoglobulin
115
How is paraprotein detected and classified
Serum electrophoresis and then serum immunofixation
116
What are Bence Jones Proteins and how are they detected
Precipitates of Ig light chains
| Urine electrophoresis
117
How can you calculate marrow cellularity
100 - age
118
Cast Nephropathy is seen in which disease
Multiple myeloma
119
Which Immunoglobulins are usually involved in myeloma
IgG or IgA
120
Why is lytic bone disease seen in myeloma
Plasma cells produce iL-6 which stimulates Osteoclasts and inhibits Osteoblasts
121
What disease can be described as a clonal disorder of cells intermediate between lymphocytes and plasma cells (lymphoplasmacytoid neoplasm)
Waldenstrom's Macroglobulinemia
122
Which Ig is involved in Waldenstrom's Macroglobulinemia
IgM
123
How will failure of primary haemostasis present and give an example
Mucosal bleeding e.g epistaxis, GI bleeds, Menorrhagia
124
A prolonged PT suggests a problem with which factors
Tissue Factor / VII
| extrinsic pathway
125
A prolonged APTT suggests a problem with which factors
VIII, IX (also XI, XII)
| intrinsic pathway
126
An ECG showing S1 Q3 T3 suggests what
P.E
127
What are the 2 types of heparin and how are they monitored
Unfractionated --> APTT
| LMWH --> Anti-Xa assay
128
What is the difference in the mechanism of action of heparin and LMWH
Heparin inhibits both Xa and IIa
| LMWHs inhibit factor Xa, but not thrombin (IIa)
129
How will the effects of protamine sulphate differ on heparin and LMWH
A complete reversal of Heparin
| A partial reversal of LMWH
130
Define Haemostasis
The arrest of bleeding AND maintenance of vascular patency
131
What kind of drug is dabigatran
direct thrombin inhibitor
| NOAC
132
WHich of the New Oral Anti-Coagulants is safest in a renally impaired patient
Apixaban
133
What is the antidote to Dabigatran
Idarucizumab
134
An elderly person presenting with anaemia and massive splenomegaly should make you think of what
Myelofibrosis
135
Which types of Hodgkin's lymphoma have the best and worst prognoses
Lymphocyte predominant --> best
| Lymphocyte depleted --> worst
136
What are the B type symptoms of lymphoma
Night Sweats
Weight loss
Fever
137
Above what age is the prognosis worse for lymphoma
45
138
Which gender tends to have a worse prognosis for lymphoma
Males
139
Normochromic, normocytic anaemia + Leukopenia + Thrombocytopenia should make you think of what
Aplastic anaemia
140
Name 3 specific drugs that can cause aplastic anaemia
Chloramphenicol
Gold
Phenytoin
141
What biochemical changes can a blood transfusion cause
Hyperkalaemia
| Hypocalcaemia
142
What steps should you take in a suspected acute haemolytic transfusion reaction
Stop the transfusion
Plenty of fluids
Inform lab
143
What is the most common cause of isolated thrombocytopenia
Immune/Idiopathic Thrombocytopenia
144
Antibodies against which structures are produced in ITP
Glycoprotein IIb/IIIa
145
Acute ITP is more common in which age group
Children post infection
146
What is the mode of inheritance of G6PD deficiency and which population is it more common in
X-linked recessive
| Afro-Caribbean
147
How can G6PD deficiency be diagnosed
G6PD enzyme assay
148
Name 3 drugs can cause haemolysis in someone with G6PD deficiency
Quinine drugs
Ciprofloxacin
Nitrofurantoin
149
What factors does cryoprecipitate contain and what two other things does it contain
VIII
XIII
Fibrinogen
vWF
150
What are the major criteria for considering cryoprecipitate administration
Low Fibrinogen
151
Name 3 biochemical abnormalities seen in tumour lysis syndrome and how is it treated
Hyperkalaemia
Hyperphosphotaemia
Hypocalcaemia
IV allopurinol
152
One of which 3 things must be present to diagnose tumour lysis syndrome
Arrhythmia
Increased creatinine
Seizure
153
Which score is used to work out the likelihood of someone having a DVT
Wells score
154
How should you manage a DVT
```
Start LMWH (stop once INR is 2-3)
Start Warfarin for 3 months if provoked DVT (6 months if unprovoked or active cancer)
```
155
Which 2 symptoms should make you think of myeloma until proven otherwise and how would you investigate
Raised ESR
Osteoporosis
serum electrophoresis
156
A monoclonal proliferation of well-differentiated lymphocytes describes what
Chronic Lymphocytic Leukaemia
157
Which cells will be seen on blood film of CLL
Smudge/smear cells
158
Name two complications of CLL
Warm (IgG) haemolysis
| Recurrent infection due to decreased Ig
159
Richter's transformation is the change from CLL to what
High-grade lymphoma
160
How does desmopressin work in treatment for bleeding in vWF disease
Stimulates vWF release from endothelial cells
161
What is the mode of inheritance of Hereditary Spherocytosis and how is it diagnosed
Autosomal dominant
| EMA binding test
162
Which virus can cause haemolysis in Hereditary Spherocytosis
Parvovirus
163
If a blood transfusion patient seems well and has an increase in temperature of < 1.5, how do you treat this
Febrile, non-haemolytic reaction
| IV/Po paracetamol
164
A starry sky blood film and myc gene translocation suggests what disease
Burkitt's Lymphoma
165
Painful heavy calves, pruritis should make you think of what
Post-thrombotic syndrome
| Graduated compression stockings
166
How will RBC's and platelets be affected by alcoholic liver disease
Macrocytic anaemia
| Thrombocytopenia
167
What are the Hb's cutoffs for transfusion
With acute coronary syndrome --> < 80
| Without acute coronary syndrome --> < 70
168
What is the platelet cutoff for platelet transfusion
< 30
| < 100 in severe bleeding or bleeds in critical areas e.g CNS
169
Name a bleeding disorder which will have prolonged bleeding time and one which will not
Prolonged bleeding time suggests a primary haemostasis problem
vWF = prolonged
Haemophilia = normal
