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Misc. Notes Year 3 > Haem Misc. > Flashcards

Haem Misc. Flashcards

1
Q

What is Anaemia

A

Reduced red blood cells

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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

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3
Q

What is haematocrit

A

The ratio of whole blood made up of red cells if a sample were left to settle

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4
Q

Decreased production/ Increased destruction of RBC’s will give which different blood results

A

Increased destruction = increased reticulocytes

Reduced production = decreased reticulocytes

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5
Q

What is the normal range of MCV

A

80 - 100 femtolitres

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6
Q

<80 MCV means what

A

Microcytosis

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7
Q

> 100 MCV means what

A

Macrocytosis

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8
Q

Name the 2 main categories of hypochromic microcytic anaemias

A

Haem deficiency

Globin deficiency

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9
Q

Name 2 causes for a lack of iron for erythropoiesis

A

Iron deficiency

Anaemia of chronic disease

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10
Q

Name 3 possible causes of porphyrin synthesis problem

A

Lead poisoning
Sideroblastic anaemia
Pyridoxine responsive anaemias

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11
Q

Name the main cause of Globin deficiency which leads to hypochromic, microcytic anaemia

A

Thalassaemia

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12
Q

What is spurious macrocytosis

A

The size of the mature red cell is NORMAL, but the MCV is measured as being high

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13
Q

What are the 2 broad categories of causes of macrocytosis

A

Megaloblastic

Non-megaloblastic

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14
Q

What is a megaloblast

A

An abnormally large nucleated red cell precursor with an immature nucleus.

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15
Q

What are the two most common causes of megaloblastic macrocytic anaemia

A

B12/Folate deficiency

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16
Q

Which of B12 or folate should you replace first

A

B12

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17
Q

What can B12/ folate deficiency cause

A

Symptoms/signs of anaemia
weight loss, diarrhoea, infertility
Sore tongue, jaundice
Developmental problems

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18
Q

What can B12 deficiency cause specifically

A

Dorsal column abnormalities
Neuropathy
Subacute Combined Degeneration of Spinal cord
Dementia

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19
Q

Blood film will show what 2 things in b12/folate deficiency

A

Hypersegmented (>5) neutrophils

Macro-ovalocytes

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20
Q

Which red flag haem sign can b12/folate deficiency cause

A

Pancytopenia

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21
Q

Where are B12 and folate absorbed

A

B12 –> ileum

Folate –> Duodenum, Jejunum

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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)

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23
Q

FFP must be frozen within how many hours of collection

A

8 hours

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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)

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25
Q

How often can you give blood

A

Must wait 12 weeks between donations

max 5 a year

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26
Q

What are the shelf lives of red cells, platelets and FFP

A

Red cells –> 35 days
Platelets –> 7 days
FFP –> 3 years

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27
Q

What temperatures are red cells, platelets and FFP kept at

A

RBC’S –> 4 degrees (give or take 2)
Platelets –> 22 degrees
FFP –> -30 degrees

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28
Q

The genes for alpha chains in Hb is on which chromosome

A

Chromosome 16

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29
Q

The gene for beta chains in Hb is on which chromosome

A

Chromosome 11

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30
Q

Alpha thalassaemias result from what kind of mutation

A

Deletion

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31
Q

Beta thalassaemias result from what kind of mutation

A

Point

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32
Q

What is HbH

A

Clumping together of Beta chains into a tetramer

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33
Q

The point mutation in sickle cell results in Glutamine being replaced by what

A

Valine

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34
Q

Name 4 long term treatments of sickle cell anaemia

A

Prophylactic penicillin
Vaccination
Folic acid
Hydroxycarbamide

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35
Q

What should you vaccinate against in someone with sickle cell anaemia/ hyposplenism

A

Meningooccus
Pneumococcus
H. Influenza

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36
Q

Which type of haemolysis is more common

A

Extravascular

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37
Q

Compare blood products in Intravascular and Extravascular haemolysis

A

Intravascular –> abnormal blood products

Extravascular –> normal blood products in excess

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38
Q

Name 3 causes of intravascular haemolysis

A 
Transfusion reaction 
G6PD deficiency 
Severe Malaria (Blackwater fever)
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39
Q

Pink urine which turns black on standing is a result of which biochemical abnormality in intravascular haemolysis

A

Haemoglobinuria

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40
Q

Which two abnormal blood products end up in the circulation in intravascular haemolysis

A

Haemoglobin

Methaemalbumin

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41
Q

Which two abnormal blood products end up in the urine in intravascular haemolysis

A

Haemoglobin

Haemosiderin

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42
Q

What is the mucosal iron transporter called

A

DMT-1

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43
Q

What is the serosal iron transporter called

A

Ferroportin

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44
Q

Which factor downregulates ferroportin and when is it produced

A

Hepcidin

Produced in the liver in response to iron load and inflammation

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45
Q

Which genetic disease results in unchecked iron absorption and which gene is affected

A

Haemochromatosis

HFE gene

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46
Q

How does Haemochromatosis present and how is it treated

A

Weakness, joint pain, impotence, cirrhosis, diabetes

Tx –> Venesection

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47
Q

How does secondary iron overload occur and how can it be prevented

A

Repeated transfusions

Iron-chelating drugs –> desferrioxamine

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48
Q

What is the antidote for Heparin reversal

A

Protamine Sulphate

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49
Q

What are the 5 stages of normal haemopoiesis

A 
Self- renewal 
Proliferation 
Differentiation 
Maturation 
Apoptosis
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50
Q

What is the most common childhood cancer

A

Acute Lymphoblastic Leukaemia

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51
Q

What is Acute Lymphoblastic Leukaemia

A

Malignant disease of primitive lymphoid cells

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52
Q

Name 3 ways in which ALL could present

A

Marrow failure
Bone Pain
CNS/Testes involvement

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53
Q

What is a malignant disease of primitive myeloid precursors

A

Acute Myeloid Leukaemia

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54
Q

AML is more common in which age group

A

Older

>60

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55
Q

Name a characteristic blood film appearance of AML and 2 characteristic symptoms

A

Auer rods on blood film

DIC and Gum infiltration

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56
Q

What is a characteristic and specific symptom of Hodgkin’s Lymphoma

A

Drinking alcohol causes chest pain

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57
Q

Hodgkin’s lymphoma is associated with which infection

A

EBV infection

E.g glandular fever

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58
Q

Which cells are seen in Hodgkin’s lymphoma and what is their CD number

A

Reed Sternberg cells

CD 30

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59
Q

What is the CD number of the cells seen in Non-Hodgkins lymphoma and which drug targets this

A

CD 20

Rituximab

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60
Q

Which autoimmune disease will cause Hypo-cellular marrow

A

Aplastic anaemia

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61
Q

Hyper/Hyposplenism causes Pancytopenia

A

Hyper

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62
Q

Name 3 causes of Hypersplenism

A

Splenic congestion (CCF, Portal hypertension)
Felty’s (Rheumatoid Arthritis, neutropenia, splenomegaly)
Splenic lymphoma

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63
Q

What are the 2 types of cytotoxic therapy

A

Cell- Cycle specific

Non- Cell specific

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64
Q

Dose/Duration is more important for cell cycle-specific therapy

A

Duration is more important

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65
Q

Vinca alkaloids are which type of drugs and can cause which side effect

A

Mitotic spindle inhibitors

Neuropathy

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66
Q

Alkylating agents are which type of cytotoxic therapy and can cause what

A

Non-cell cycle specific

Can cause infertility

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67
Q

Which cytotoxic therapy can cause cardiomyopathy

A

Anthracyclines

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68
Q

Platinum derivatives such as Cisplatinum can cause what

A

Nephropathy

69
Q

What drug regime should be initiated in neutropenic sepsis

A

Piperacillin/ Tazobactam

+ gentamicin if severe

70
Q

Myeloproliferative disorders can be divided into which two groups

A

BCR-ABL 1 negative or positive

71
Q

Which MPD comes under BCR-ABL 1 negative

A

Polycythaemia Rubra Vera
Essential Thrombocythaemia
Idiopathic Myelofibrosis

72
Q

Which MPD comes under BCR-ABL 1 positive

A

Chronic Myeloid Leukaemia

73
Q

What are the three stages of Chronic Myeloid Leukaemia

A

Chronic
Accelerated
blast crisis

74
Q

What will be seen in the blood of someone with CML

A

Leucocytosis, Neutrophilia, Myeloid precursors

75
Q

Which chromosome is a hallmark of CML and how does it come about

A

Philadelphia

9,22 translocation

76
Q

What does the Philadelphia chromosome result in

A

Tyrosine kinase expression

77
Q

Which drug inhibits Tyrosine Kinase and can be used to treat CML

A

Imatinib

78
Q

How does Essential Thrombocythaemia result in acquired vWF deficiency

A

Excess abnormal platelets mop up all the available vWF

79
Q

How is Essential Thrombocythaemia treated

A

Aspirin

Hydroxycarbamide

80
Q

JAK2 mutation is associated with which MPD

A

Polycythaemia Rubra Vera

81
Q

Which MPD will show leukoerythroblastic blood film with teardrop shaped RBC’s

A

Idiopathic Myelofibrosis

82
Q

What is a unique symptom of Polycythaemia Rubra Vera

A

Aquagenic Itch

83
Q

How can Polycythaemia Rubra Vera be treated

A

JAK2 inhibitors

84
Q

How can Idiopathic Myelofibrosis be treated

A

Allogeneic stem cell transplantation
JAK 2 inhibitors
Splenectomy

85
Q

Which 3 systems are activated in DIC

A

Complement
Coagulation
Kinin

86
Q

What are the 4 most common causes of DIC

A

Hypovolaemic Shock
Sepsis
Malignancy
Obstetric emergency

87
Q

Asides from treating the underlying cause, what else can be done for DIC

A

Fresh Frozen Plasma

Contains clotting factors

88
Q

How is haemophilia inherited

A

X-linked recessive

89
Q

Which factors are deficient in Haemophilia A and B and which one is more common

A

A –> VIII (more common)

B –> IX

90
Q

Which clotting test will be abnormal in Haemophilia

A

isolated prolonged APTT

91
Q

How will Haemophilia present

A

Recurrent Haemarthroses
Soft tissue bleeds
Prolonged bleed after surgery

92
Q

Name 3 examples of naturally occurring anti-coagulants

A

Serine protease inhibitors –> AT III

Protein C and S

93
Q

Protein C and S inhibit which factors

A

V, VII, IX, Xa

94
Q

What is factor V Leiden

A

An autosomal dominant disease where a variant of factor V which can’t be inhibited very well by protein C is made

95
Q

Which receptors do platelets bind to each other via and which drug can block these

A

Glycoprotein IIb/IIIa

Abciximab

96
Q

How is INR calculated and how can the results be interpreted

A 
INR = [PTpatient ÷ MNPT]ISI
>1.5 = bleeding risk
97
Q

Howell- Jolly bodies are seen in which condition

A

Hyposplenism

98
Q

What are Heinz bodies and when can they be seen

A

Clumps of denatured Hb

Seen in intravascular haemolysis (decreased haptoglobin)

99
Q

In which state can iron bind O2 in haem

A

Fe2+

100
Q

How is iron kept in the Fe2+ state

A

NADH from glycolysis

101
Q

What are the 3 methods of Co2 transport

A

Dissolved
Bound to Hb
As bicarbonate

102
Q

What is the problem in Sideroblastic Anaemia

A

Porphyrin Ring disturbance

103
Q

Uridine is changed to what in the Folate cycle

A

Thymidine

104
Q

Which cycle is b12 involved in

A

Methionine cycle

105
Q

How is pernicious anaemia treated

A

b12 injections + folate tablets for life

Transfusions if severe

106
Q

Name 4 non-megaloblastic causes of macrocytosis and which one of these is associated with anaemia

A

Liver failure
Alcohol
Hypothyroidism
Marrow failure –> associated with anaemia

107
Q

Where and how are the cells break down in a delayed transfusion reaction

A

In spleen by macrophages

108
Q

How are thalassaemias diagnosed

A

HPLC

109
Q

Which disease are those with thalassaemia slightly less at risk of

A

Malaria

110
Q

Why does splenomegaly occur in thalassaemia

A

Extramedullary haematopoiesis

111
Q

Define warm and cold autoimmune haemolysis

A

Warm –> IgG

Cold –> IgM

112
Q

Which lymphoma is basically incurable and why

A

Low-grade B cell NHL

Slow growing so chemo doesn’t work

113
Q

How can you diagnose Fanconi Anaemia

A

Chromosome fragility testing

114
Q

What is paraprotein

A

Monoclonal Immunoglobulin

115
Q

How is paraprotein detected and classified

A

Serum electrophoresis and then serum immunofixation

116
Q

What are Bence Jones Proteins and how are they detected

A

Precipitates of Ig light chains

Urine electrophoresis

117
Q

How can you calculate marrow cellularity

A

100 - age

118
Q

Cast Nephropathy is seen in which disease

A

Multiple myeloma

119
Q

Which Immunoglobulins are usually involved in myeloma

A

IgG or IgA

120
Q

Why is lytic bone disease seen in myeloma

A

Plasma cells produce iL-6 which stimulates Osteoclasts and inhibits Osteoblasts

121
Q

What disease can be described as a clonal disorder of cells intermediate between lymphocytes and plasma cells (lymphoplasmacytoid neoplasm)

A

Waldenstrom’s Macroglobulinemia

122
Q

Which Ig is involved in Waldenstrom’s Macroglobulinemia

A

IgM

123
Q

How will failure of primary haemostasis present and give an example

A

Mucosal bleeding e.g epistaxis, GI bleeds, Menorrhagia

124
Q

A prolonged PT suggests a problem with which factors

A

Tissue Factor / VII

extrinsic pathway

125
Q

A prolonged APTT suggests a problem with which factors

A

VIII, IX (also XI, XII)

intrinsic pathway

126
Q

An ECG showing S1 Q3 T3 suggests what

A

P.E

127
Q

What are the 2 types of heparin and how are they monitored

A

Unfractionated –> APTT

LMWH –> Anti-Xa assay

128
Q

What is the difference in the mechanism of action of heparin and LMWH

A

Heparin inhibits both Xa and IIa

LMWHs inhibit factor Xa, but not thrombin (IIa)

129
Q

How will the effects of protamine sulphate differ on heparin and LMWH

A

A complete reversal of Heparin

A partial reversal of LMWH

130
Q

Define Haemostasis

A

The arrest of bleeding AND maintenance of vascular patency

131
Q

What kind of drug is dabigatran

A

direct thrombin inhibitor

NOAC

132
Q

WHich of the New Oral Anti-Coagulants is safest in a renally impaired patient

A

Apixaban

133
Q

What is the antidote to Dabigatran

A

Idarucizumab

134
Q

An elderly person presenting with anaemia and massive splenomegaly should make you think of what

A

Myelofibrosis

135
Q

Which types of Hodgkin’s lymphoma have the best and worst prognoses

A

Lymphocyte predominant –> best

Lymphocyte depleted –> worst

136
Q

What are the B type symptoms of lymphoma

A

Night Sweats
Weight loss
Fever

137
Q

Above what age is the prognosis worse for lymphoma

A

45

138
Q

Which gender tends to have a worse prognosis for lymphoma

A

Males

139
Q

Normochromic, normocytic anaemia + Leukopenia + Thrombocytopenia should make you think of what

A

Aplastic anaemia

140
Q

Name 3 specific drugs that can cause aplastic anaemia

A

Chloramphenicol
Gold
Phenytoin

141
Q

What biochemical changes can a blood transfusion cause

A

Hyperkalaemia

Hypocalcaemia

142
Q

What steps should you take in a suspected acute haemolytic transfusion reaction

A

Stop the transfusion
Plenty of fluids
Inform lab

143
Q

What is the most common cause of isolated thrombocytopenia

A

Immune/Idiopathic Thrombocytopenia

144
Q

Antibodies against which structures are produced in ITP

A

Glycoprotein IIb/IIIa

145
Q

Acute ITP is more common in which age group

A

Children post infection

146
Q

What is the mode of inheritance of G6PD deficiency and which population is it more common in

A

X-linked recessive

Afro-Caribbean

147
Q

How can G6PD deficiency be diagnosed

A

G6PD enzyme assay

148
Q

Name 3 drugs can cause haemolysis in someone with G6PD deficiency

A

Quinine drugs
Ciprofloxacin
Nitrofurantoin

149
Q

What factors does cryoprecipitate contain and what two other things does it contain

A

VIII
XIII
Fibrinogen
vWF

150
Q

What are the major criteria for considering cryoprecipitate administration

A

Low Fibrinogen

151
Q

Name 3 biochemical abnormalities seen in tumour lysis syndrome and how is it treated

A

Hyperkalaemia
Hyperphosphotaemia
Hypocalcaemia
IV allopurinol

152
Q

One of which 3 things must be present to diagnose tumour lysis syndrome

A

Arrhythmia
Increased creatinine
Seizure

153
Q

Which score is used to work out the likelihood of someone having a DVT

A

Wells score

154
Q

How should you manage a DVT

A 
Start LMWH (stop once INR is 2-3)
Start Warfarin for 3 months if provoked DVT (6 months if unprovoked or active cancer)
155
Q

Which 2 symptoms should make you think of myeloma until proven otherwise and how would you investigate

A

Raised ESR
Osteoporosis
serum electrophoresis

156
Q

A monoclonal proliferation of well-differentiated lymphocytes describes what

A

Chronic Lymphocytic Leukaemia

157
Q

Which cells will be seen on blood film of CLL

A

Smudge/smear cells

158
Q

Name two complications of CLL

A

Warm (IgG) haemolysis

Recurrent infection due to decreased Ig

159
Q

Richter’s transformation is the change from CLL to what

A

High-grade lymphoma

160
Q

How does desmopressin work in treatment for bleeding in vWF disease

A

Stimulates vWF release from endothelial cells

161
Q

What is the mode of inheritance of Hereditary Spherocytosis and how is it diagnosed

A

Autosomal dominant

EMA binding test

162
Q

Which virus can cause haemolysis in Hereditary Spherocytosis

A

Parvovirus

163
Q

If a blood transfusion patient seems well and has an increase in temperature of < 1.5, how do you treat this

A

Febrile, non-haemolytic reaction

IV/Po paracetamol

164
Q

A starry sky blood film and myc gene translocation suggests what disease

A

Burkitt’s Lymphoma

165
Q

Painful heavy calves, pruritis should make you think of what

A

Post-thrombotic syndrome

Graduated compression stockings

166
Q

How will RBC’s and platelets be affected by alcoholic liver disease

A

Macrocytic anaemia

Thrombocytopenia

167
Q

What are the Hb’s cutoffs for transfusion

A

With acute coronary syndrome –> < 80

Without acute coronary syndrome –> < 70

168
Q

What is the platelet cutoff for platelet transfusion

A

< 30

< 100 in severe bleeding or bleeds in critical areas e.g CNS

169
Q

Name a bleeding disorder which will have prolonged bleeding time and one which will not

A

Prolonged bleeding time suggests a primary haemostasis problem
vWF = prolonged
Haemophilia = normal

Misc. Notes Year 3 (25 decks)

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