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Phase 2 > Haematology > Flashcards

Haematology Flashcards

1
Q

Describe the types of Haemoglobin

A

HbA - 2 x alpha & 2 x beta chains (common, adults)
HbA2 - 2 x alpha & 2 x delta chains (less common, adults)
HbF - 2 x alpha & x gamma chains (foetal)

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2
Q

Name some classical anaemia symptoms

A

Fatigue, lethargy
Headaches
Palpitations
Dyspnoea
Brittle hair and nails
Faintness, syncope
Anorexia

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3
Q

How are the types of anaemia classified?

A

Mean cell volume

Microcytic - < 80
Normocytic 80 - 100
Macrocytic > 100

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4
Q

Name the types of microcytic anaemia

A

TAILS

Thalassaemia
Anaemia of Chronic Disease
Iron-deficiency
Lead poisoning
Sideroblastic

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5
Q

What’s the most common type of microcytic anaemia?

A

Iron-deficiency

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6
Q

Causes Iron-def anaemia

A

**Hookworm - leading cause
Poor diet (esp in children/babies in poverty)
↑ Demands for O2 e.g. growth, pregnancy
Malabsorption e.g. coeliac disease
GI bleeding
Menorrhagia

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7
Q

RF Iron-def anaemia

A

LICs
High veg diet
Premature infants
Late intro to mixed feed - breast milk contains low iron

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8
Q

Key presentation of Iron-Def anaemia

A

Same baseline symptoms

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9
Q

Additional signs/symptoms of IDA

A

Atrophic glossitis - tongue inflammation
Koilonychia - spoon shaped nail
Angular stomatitis - inflammation of corners of mouth

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10
Q

What conditions protect from Plasmodium falciparum malaria?

A

Sickle Cell Disease
Thalassaemia
G6PDH Deficiency

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11
Q

Ix Iron-Def anaemia

A

Blood count & film - RBC microcytic and hypochromic
Poikilocytosis and anisocytosis

Serum ferritin - low
Reticulocyte count - reduced

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12
Q

Why might serum ferritin be normal even if a patient has Iron-Def anaemia?

A

might have malignancy or infection, usually rises ∴ negates and is normal

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13
Q

Tx Iron-Def anaemia

A

Ferrous sulphate - oral iron
For at least 3 months

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14
Q

Side effects of ferrous sulphate
What can you give is S/E are too bad?

A

Black stool (melena)
Constipation/Diarrhoea
Nausea
GI upset
Abd discomfort

Ferrous gluconate

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15
Q

If very severe Iron-Def (e.g. severe malabsorption), what is the Tx?

A

Parenteral iron e.g. IV iron or deep IM iron

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16
Q

Once Iron-Def anaemia is treated, how long does it usually take for iron stores to be replenished?

A

6 months

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17
Q

Iron studies for IDA

A

Ferritin - Low/Normal/High
Serum iron - Low
Transferrin - High
Transferrin sat - Low
TIBC - High

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18
Q

Iron studies for Anaemia of Chronic Disease

A

Ferritin - Normal/Raised
Serum Iron - Low
Transferrin - Low
Transferrin sat - Normal/Low
TIBC - Low

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19
Q

Iron studies for Thalassaemia

A

Ferritin - Normal/Raised
Serum Iron - Normal/Raised
Transferrin - Normal/Low
Transferrin Sat - Normal/Raised
TIBC - Normal/Low

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20
Q

Causes of alpha thalassaemia

A

Caused by mutation - gene deletions
↓ a - chain synthesis

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21
Q

Where is the gene for alpha-globin chains?

A

Duplicated on both chromosome 16s

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22
Q

If 1 alpha-globin gene deletion,

A

Usually normal blood picture
Asymptomatic

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23
Q

If 2 alpha-globin gene deletion,

A

Microcytosis with or without mild anaemia
Carrier for alpha-thalassaemia

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24
Q

If 3 alpha-globin gene deletion,

A

↓↓ reduction of alpha-chain synthesis
∴ HbH disease

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25
Q

Signs of HbH disease

A

Moderate/Severe haemolytic anaemia
Splenomegaly
Patient is usually not transfusion dependent

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26
Q

Where is HbH often found?

A

Common in parts of Asia

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27
Q

If 4 alpha-globin gene deletion,

A

NO alpha-chain synthesis
Only Hb Barts present (4x gamma chains)
CANNOT CARRY OXYGEN ∴ NOT COMPATIBLE WITH LIFE

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28
Q

Key presentation of 4 alpha-globin gene deletion

A

Infants are stillborn - pale, oedematous w/ huge livers and spleen

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29
Q

What are carriers of a-Thalassaemia protected from?

A

Falciparum malaria

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30
Q

Pathophysiology Beta-Thalassaemia

A

Little/No B-chains results in XS a-chains
∴ these bind w/ delta and gamma chains

∴ ↑ HbA2 and HbF

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31
Q

Cause of beta thalassaemia

A

Point mutations
which result in frame shifts
∴ highly unstable and unusable B-globin

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32
Q

Name the subtypes of B-Thalassaemia and brief description

A
  1. B-Thalassaemia Minor
    Asymptomatic heterozygous carrier state
  2. B-Thalassaemia Intermedia
    Moderate anaemia
  3. B-Thalassaemia Major
    Homozygous
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33
Q

How does B-Thalassaemia Minor present?

A

Mild/absent anaemia - Low MCV + hypochromic

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34
Q

DDx of B-Thalassaemia Minor
How can you differentiate?

A

Iron-Def anaemia
Serum ferritin and iron stores in B-Thalassaemia Minor are normal

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35
Q

How does B-Thalassaemia Intermedia present?

A

Splenomegaly!
Bone abnormalities, recurrent leg ulcers, gallstones!

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36
Q

How does B-Thalassaemia Major present?

A

Presents in 1st year of life w/ severe anaemia (Cooley’s anaemia), failure to thrive, recurrent infections
Bony abnormalities e.g. skull bossing
Hair on end sign on skull XR
Hepatosplenomegaly (due to haemolysis)

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37
Q

Ix B-Thalassaemia

A

FBC, Blood film - hypochromic microcytic anaemia
Reticulocytes - increased & nucleated RBCs in periph circulation

Hb electrophoresis - ↑ HbF and absent/low HbA

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38
Q

Why does B-Thalassaemia (Major) present with bone expansion?

A

Extramedullary haematopoiesis (outside marrow)

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39
Q

Tx B-Thalassaemia

A

Regular (every 2-4 weeks) and life-long blood transfusions

Iron-chelating agents (prevent XS iron)
e.g. oral Deferiprone & SC desderrioxamine

Splenectomy - if transfusion not effective, done after childhood

Long term folic acid

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40
Q

Complications of blood transfusions

A

↑ Iron in body
which is deposited mainly in spleen and liver
∴ liver fibrosis and cirrhosis :0

Also deposited in endocrine glands (-> diabetes, hypothyroidism, hypocalcaemia, premature death)

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41
Q

S/E of iron-chelating agents

A

Pain, deafness, cataracts, retinal damage

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42
Q

Other than iron-chelating agents, whats a way to prevent XS iron during blood transfusion?

A

Ascorbic acid
↑ Urinary excretion of iron

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43
Q

Big complication w/ Tx of B-Thalassaemia

A

IRON OVERLOAD!

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44
Q

Brief pathophysiology of Sideroblastic anaemia

A

Defective protoporphyrin production
∴ impaired incorporation of iron to form haem
∴ build up of iron in RBCs
∴ immature and dysfunctional

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45
Q

Congenital cause of Sideroblastic anaemia

A

X-Linked, mutation in ALAS2 gene

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46
Q

Acquired causes of Sideroblastic anaemia

A

XS alcohol
Vit B6 def
Lead poisoning

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47
Q

Key presentation of Sideroblastic anaemia

A

Fatigue, hepatosplenomegaly

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48
Q

Ix of Sideroblastic anaemia

A

1st
Complete blood count -
if normal/low = congenital cause
if normal/high = acquired cause

Iron studies

GS
Peripheral blood smear !

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49
Q

What would you find in a peripheral blood smear of Sideroblastic anaemia?

A

RBCs w/ :
Basophilic stippling
Ringed sideroblasts
Pappenheimer bodies

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50
Q

Tx of Sideroblastic anaemia

A

Removal of toxins
Pyridoxine, thiamine & folic acid
If severe : bone marrow/liver transplant

-> Don’t forget! Can cause iron overload so iron-chelating agents/ascorbic acid etc

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51
Q

What type of anaemia is Anaemia of Chronic disease usually?

A

Often normocytic but CAN be microcytic - esp in Crohn’s and RA

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52
Q

Name some chronic diseases often related to A of CD

A

TB
Crohn’s
RA
SLE
Malignant diseases

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53
Q

Pathophysiology of A of CD

A

↓ Release of iron from BM to developing erythroblasts
∴ Inadequate erythropoietin response to anaemia

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54
Q

Tx of A of CD

A

Treat underlying cause
EPO therapy - used in renal and inflammatory diseases

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55
Q

S/E of EPO therapy

A

Flu-like symptoms
HTN
Thromboembolism

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56
Q

Main causes of Normocytic anaemia

A

Acute blood loss
A of CD
Endocrine disorders e.g. hypopituitarism, hypothyroidism, hypoadrenalism
Renal failure
Pregnancy

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57
Q

Ix Autoimmune haemolytic anaemia

A

Coombs’ test = positive
Spherocytes often present

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58
Q

What are some markers for cell turnovers?

A

↑ Lactate dehydrogenase
↑ Unconj bilirubin
↓ Haptoglobin

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59
Q

What is the role of haptoglobin?

A

Binds to free haemoglobin
i.e. when RBC’s broken down, binds to it to prevent ROS
∴ ↓ levels during haemolytic anaemia

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60
Q

How is sickle cell disease inherited?

A

Autosomal recessive
∴ 1 in 4 chance of disease, 50% of being a carrier if parents is carrier

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61
Q

In which ethnicity is sickle cell disease most common?

A

Afro-Caribbean

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62
Q

Cause of sickle cell disease

A

Mutation of B-globin gene (glutamic -> valine)
Results in HbS variant

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63
Q

RF of sickle cell disease

A

FHx
Genetics

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64
Q

What are sickle cell disease carriers protected from?

A

Plasmodium falciparum malaria

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65
Q

Key presentation of sickle cell disease

A

Usually identified in 1st year of life
Asymptomatic except in ‘stress’ - hypoxia, cold, dehydration, acidosis
Acute pain in hands and feet - dactylitis

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66
Q

Why don’t sickle cell disease Pxs usually have anaemia symptoms?

A

Because chronic haemolysis = stable Hb levels

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67
Q

What happens if Hb levels in sickle cell disease patients suddenly falls?

A

Splenic sequestration/infarction
Gallstones
Aplastic anaemia
Leg ulcers

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68
Q

Further sign of sickle cell disease

A

Jaundice

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69
Q

Ix sickle cell disease

A

FBC - ↓ Hb, ↑ reticulocytes
Blood film - sickled erythrocytes

Hb electrophoresis - confirms diagnosis
Hb SS present, absent HbA

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70
Q

What screening process do we aim to do for sickle cell disease and why?

A

Neonate blood/heel prick test
Pneumococcal prophylaxis

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71
Q

What screening process do we aim to do for sickle cell disease and why?

A

Neonate blood/heel prick test
Pneumococcal prophylaxis

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72
Q

Tx for sickle cell disease

A

Prophylaxis for/avoid triggers e.g. stay warm, vaccines

FOLIC ACID! for all Px

If acute attack :
IV fluids
Analgesia
O2 and Abx if req

Hydroxycarbamide - ↑HbF conc
Blood trans if severe

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73
Q

Complications of sickle cell disease

A

Pulmonary HTN and chronic lung disease
= most common cause of death in adults w sickle cell disease

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74
Q

Cause of Spherocytosis & Elliptocytosis

A

Def in RBC structural membrane protein - SPECTRIN

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75
Q

Inheritance of Spherocytosis & Elliptocytosis

A

Autosomal dominant
But can occur spontaneously

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76
Q

Difference between Spherocytosis & Elliptocytosis

A

Spherocytosis - vertical deformity
Elliptocytosis - horizontal deformity

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77
Q

Pathophysiology of Spherocytosis & Elliptocytosis

A

Spectrin def = Abnormal RBC cell membrane
∴ ↑ permeability to Na+
∴ RBCs become rigid and spherical

Spleen mistakenly believes RBCs are damaged and are prematurely destroyed
Extravascular haemolysis

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78
Q

Key presentation of Spherocytosis & Elliptocytosis

A

Neonatal jaundice (Can sometimes be delayed/Be completely asymptomatic)
Splenomegaly symptoms (exacerbated during infection)

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79
Q

Other signs/symptoms of Spherocytosis & Elliptocytosis

A

XS bilirubin - gallstones
Leg ulcers

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80
Q

Ix Spherocytosis & Elliptocytosis

A

Blood film - presence of Spherocytosis & reticulocytes
FBC & Reticulocyte count - ↓ Hb, ↑ Reticulocytes

Direct anti-globulin (Coombs’ test) = NEGATIVE
Rules out autoimmune haemolytic anaemia

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81
Q

Tx Spherocytosis & Elliptocytosis

A

Splenectomy
Folic acid

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82
Q

G6PDH full form

A

Glucose-6-Phosphate Dehydrogenase

83
Q

Epidemiology of G6PDH Deficiency

A

Mainly males - X-Linked recessive condition
But can effect F

↑ Prevalence in Mediterranean, African, Middle/Far Eastern

84
Q

Triggers of G6PDH Deficiency

A

Infections
Food
Medications e.g. anti-malarials

85
Q

What does G6PDH Deficiency protect Px from?

A

Plasmodium falciparum malaria

86
Q

Pathophysiology of G6PDH Deficiency

A

G6PDH vital in hexose monophosphate shunt
∴ maintains glutathione in reduced state

Glutathione protects RBCs from oxidative stress

87
Q

G6PDH Deficiency susceptible to?

A

Oxidative stress!!

88
Q

Key presentation of G6PDH Deficiency

A

Asymptomatic until exposed to oxidative stressor
Neonatal jaundice

89
Q

What can make G6PDH Deficiency worse?

A

FAVA BEANS

90
Q

Where is gene for G6PDH located?

A

Chromosome Xq8, near factor VIII gene

91
Q

Ix G6PDH Deficiency

A

1st
FBC - anaemia, ↑ reticulocytes

GS
Blood film - bite and blister cells, Heinz bodies

92
Q

Describe the G9PDH enzymes in G6PDH Deficiency

A

Will be LOW
but straight after attack, might be normal bc oldest RBCs (w/ less G6PDH activity) are destroyed first
∴ might show falsely high concs of G6PDH

93
Q

Tx G6PDH Deficiency

A

Stop triggering factors/fava beans
Also, henna can trigger

Transfusion if severe

Splenectomy usually NOT HELPFUL

94
Q

Other signs/symptoms of G6PDH Deficiency

A

Back pain
Dark urine
Nausea

95
Q

FEVER + EXOTIC TRAVEL =

A

MALARIA until proven otherwise

96
Q

What is Aplastic anaemia?

A

Bone Marrow failure

97
Q

Definition of Aplastic anaemia

A

Pancocytopenia with hypocellularity of BM

98
Q

Causes of Aplastic anaemia

A

Can be congenital but usually acquired
Infections - EBV, HIV, TB, Hepatitis
Chemo drugs
Abx - carbamazepine, azathioprine

99
Q

Pathophysiology of Aplastic anaemia

A

↓ number of pluripotent stem cells
∴ ↓ haemopoiesis
∴ ↓ no. new RBCs to replace old ones undergoing apoptosis

100
Q

Key presentation of aplastic anaemia

A

Classic anaemia symptoms
Recurrent infections

101
Q

Other signs/symptoms of Aplastic anaemia

A

↑ Bruising
↑ Bleeding (esp from nose and gums)

102
Q

Ix Aplastic anaemia

A

FBC - pancytopenia
Reticulocyte - low or absent!

BM biopsy hypocellular marrow w/ ↑ fat spaces

103
Q

Other than Aplastic anaemia, what are some other causes of pancocytopenia?

A

Drugs
H lymphoma
NH lymphoma
Myeloma
SLE

104
Q

Tx Aplastic anaemia

A

Manage underlying cause
Blood & platelet transfusion
BM transfusion

Immunosuppressive therapy -
anti-thymocyte globulin (ATG) and ciclosporin

105
Q

Two words, explain Aplastic anaemia

A

Reduced haemopoiesis

106
Q

What is the main concern with Aplastic anaemia?
Why? How do we manage this?

A

Infection
Bc Px has severe neutropenia
Give broad-spec parenteral Abx URGENTLY

107
Q

When is immunosuppressive therapy !! (Aplastic anaemia) (first line? idk not sure)

A

Over 40
Below 40 w/ severe disease who do NOT have HLA identical sibling donor
Transfusion dependent Px

108
Q

When is BM transplant appropriate in Aplastic anaemia Px?

A

From HLA identical sibling or donor treatment of choice in those under 40

109
Q

What is important to be aware of for candidates of BM transplant?

A

Blood/platelet transfusions to be used cautiously
To avoid sensitisation

110
Q

Name the 3 main causes of Macrocytic anaemia

A

Megaloblastic
1. B12 deficiency (pernicious anaemia)
2. Folate deficiency

Non-Megaloblastic
3. XS alcohol

111
Q

Where is B12 found?

A

Meat, fish, dairy products
NOT plants!

112
Q

RF Pernicious anaemia

A

Elderly (>60)
Female
Fair-haired, blue eyes (e.g. Scandinavian)
Blood group A
Autoimmune diseases
Vegan

113
Q

Pathophysiology Pernicious anaemia

A

Vit B12 absorbed in terminal ileum bound to IF, produced by parietal cells

Autoimmune condition where parietal cells are attacked
∴ atrophic gastritis + loss of IF production
∴ B12 malabsorption

114
Q

Key presentation of Pernicious anaemia

A

Baseline anaemia symptoms

115
Q

Other signs of Pernicious anaemia

A

Glossitis
Angular stomatitis
Jaundice
Neurological symptoms - only with severe def

116
Q

Ix Pernicious anaemia

A

FBC & blood film - macrocytic RBCs
Serum B12 - low

GS
Intrinsic factor antibody screen (but low sensitivity)

117
Q

What should you never give to a Px with Pernicious anaemia?

A

DO NOT GIVE FOLIC ACID!!
Causes fulminant neurological deficits

118
Q

Tx Pernicious anaemia

A

If B12 def due to malabsorption, injections are needed
∴ IM hydroxocobalamin

If dietary, oral B12

119
Q

Where is folate found?

A

Green veg e.g. spinach, broccoli
Also, nuts, yeast & liver

120
Q

Causes of folate deficiency anaemia

A

Poor dietary intake - poverty, alcoholics, elderly
Malabsorption - Crohn’s, coeliac
Increased demand - pregnancy
Anti-folate drugs - methotrexate, trimethoprim

121
Q

How can you differentiate between folate deficiency anaemia and pernicious anaemia?

A

Folate deficiency anaemia does not have neuropathy, while pernicious anaemia does.

122
Q

Key presentation of folate deficiency anaemia

A

Anaemia symptoms
May be asymptomatic

123
Q

Ix folate deficiency anaemia

A

Blood count & film - RBCs are macrocytic
Serum & RBC folate - LOW

124
Q

Tx folate deficiency anaemia

A

Treat underlying cause
Folic acid supplements - NEVER WITHOUT B12 (unless normal B12 level)

125
Q

Define megaloblastic anaemia

A

Inhibition of DNA synthesis
∴ RBCs can’t progress onto mitosis
∴ continued growth without division
∴ macrocytosis

126
Q

Epidemiology of acute myeloid leukaemia

A

Older adults (peak at 60 years)
M > F
Most common acute leukaemia of adults!

127
Q

What is AML?

A

Neoplastic, uncontrolled proliferation of myeloid blast cells

128
Q

What do myeloblasts give rise to?

A

Basophils, Neutrophils and eosinophils

129
Q

RF AML

A

Radiation e.g. prev chemo
Down’s syndrome

130
Q

Key presentation AML

A

Anaemia - dyspnoea, fatigue, angina, claudication
Low WBC - recurrent infection, mouth ulcers
Low platelets - bleeding, bruising

*Hepatosplenomegaly
*Gum hypertrophy

131
Q

Ix AML

A

1st
Peripheral blood smear! - to see if myeloblasts present
FBC

GS
BM biopsy!! to check for ↑ blast cell %
AUER RODS!

132
Q

Blast cell % normal vs AML

A

Normal = 1-2%
AML = can be > 20%

133
Q

What Ix required to differentiate between ALL and AML?

A

Microscopy
Immunophenotyping
Molecular methods

134
Q

Tx AML

A

Blood / BM / stem cell transfusions
Chemotherapy
Prophylactic antivirals, antibiotics, antifungals! (bc of neutropenia)
IV fluids

ALLOPURINOL - prevents tumour lysis syndrome

135
Q

Name a subtype of AML

A

Disseminated intravascular coagulation
Occurs when there’s a release of thromboplastin

136
Q

What is ALL?

A

Uncontrolled prolif of blast cells - lymphoblasts
Life-threatening!!

137
Q

Epidemiology ALL

A

CHILDREN!
Most common between 2 - 4 years

138
Q

RF ALL

A

Ionising radiation e.g. X-rays during pregnancy
Down’s syndrome

139
Q

What do lymphoid cells give rise to?

A

T and B cells

140
Q

Key presentation ALL

A

Anaemia - dyspnoea, fatigue, angina, claudication
Low WBC - recurrent infection, mouth ulcers
Low platelets - bleeding, bruising

*Hepatosplenomegaly
Bone pain more common in ALL than AML
*Swollen testicles

141
Q

Ix ALL

A

1st
Peripheral blood smear! - to see if lymphoblasts present
FBC

GS
BM biopsy!! to check for ↑ blast cell %
CXR and CT scan - look for mediastinal and abdominal lymphadenopathy

Other - Lumbar puncture to look for CNS involvement

142
Q

Tx ALL

A

Blood / BM / stem cell transfusions
Chemotherapy
Prophylactic antivirals, antibiotics, antifungals! (bc of neutropenia)
IV fluids

ALLOPURINOL - prevents tumour lysis syndrome

143
Q

What is CML?

A

Overproduction of myeloid progenitor

144
Q

Epidemiology CML

A

Occurs most often between 40 - 60 years
Slight M > F

145
Q

Cause of CML

A

Mutation in Philadelphia chromosome causes ineffective and XS granulocyte to be produced

146
Q

Key presentation CML

A

Anaemia symptoms
Splenomegaly ∴ abd discomfort
Hyperviscosity - headaches, thrombotic events
Hypermetabolic - weight loss, malaise, night sweats
Gout - due to purine breakdown

147
Q

Ix CML

A

FBC - ↑↑ WBC w whole spectrum of myeloid cells
BM aspiration - increased cells

GS : Genetic testing -
PHILADELPHIA CHROMOSOME, BCR-ABL gene, t(9;22)

148
Q

Tx CML

A

ORAL IMATINIB - specific BRCR-ABL tyrosine kinase inhibitor
Stem cell transplant

149
Q

What is CLL?

A

Insidious accumulation of incompetent lymphocytes

150
Q

RF CLL

A

M > F
Genetics (trisomies, mutations, deletions)
Pneumonia may be a trigger
> 70 years

151
Q

Which is the most common leukaemia?

A

CLL

152
Q

Key presentation of CLL

A

Usually asymptomatic
∴ Found on routine FBC

May be anaemic or infection prone

153
Q

If CLL is severe, how might it present?

A

Weight loss, Night sweats, Anorexia
Large, rubbery, non-tender lymph nodes
Hepatosplenomegaly

154
Q

Ix CLL

A

Blood film - SMUDGE CELLS
↑ WBC w/ v high lymphocytes

155
Q

Complications of CLL

A

Autoimmune haemolysis
↑ Infection risk due to low IgG - bacterial & viral, esp herpes zoster!
BM failure (aplastic anaemia)

May transform into aggressive lymphoma - Richter’s Syndrome

156
Q

Progression of CLL

A

May stay stable for years, might regress!
Death usually due to complications

157
Q

Tx CLL

A

Blood transfusions

HUMAN IV IG
Rituximab

Chemo or Radiotherapy
Stem cell transplant

158
Q

Prognosis of CLL

A

RULE OF 3
1/3 will never progress
1/3 progress slowly
1/3 progress actively

159
Q

Types of lymphoma

A

Hodgkin’s and Non-Hodgkin’s

160
Q

Name the two types of Non-Hodgkin’s lymphoma

A

B Cell lymphoma - Majority of cases.
Can be indolent, aggressive, v aggressive

T Cell lymphoma

161
Q

Non-Hodgkin’s lymphoma has a strong link with?

A

EBV
Burkitt’s lymphoma

162
Q

Key presentation of Non-Hodgkin’s lymphoma

A

Nodal involvement - superficial lymphadenopathy

Extranodal -
GI - bowel obstruction
BM - fatigue, easy bruising/bleeding, recurrent infections
Spinal cord - weakness, loss of sensation (usually legs)

163
Q

Ix Non-Hodgkin’s lymphoma

A

Lymph node biopsy OR BM biopsy
CT/MRI of chest, abd and pelvis for STAGING

164
Q

What indicates a worse prognosis for Non-Hodgkin’s lymphoma? Why?

A

↑ Lactose dehydrogenase
sign of ↑ cell turnover ∴ ↑ proliferation

165
Q

What are some differences between Hodgkin’s and Non-Hodgkin’s lymphoma?

A

Non - Hodgkin’s lymphoma
NO Reed-Sternberg cells!
Skin rashes e.g. mycosis fungoides
Neutropenia

Hodgkin’s lymphoma
Reed-Sternberg cells
Skin excoriations
Neutrophilia

166
Q

Tx Non-Hodgkin’s lymphoma

A

R-CHOP - Combination chemo

Rituximab
Cyclophosphamide
Hydroxy-danorubicin
O Vincristine (Oncovin is brand name)
Prednisolone

Low grade - might not need, radiotherapy may be enough if localised
High grade (Early) - 3 month R-CHOP w/ radiotherapy
High grade (Late) - 6 month R-CHOP w// radiotherapy

167
Q

Rituximab
What is it?
Target?
S/E?

A

Monoclonal antibody
CD20! expressed on B cell surface
Minimal S/E

168
Q

How is B cell lymphoma classified?

A

Indolent/Low grade :
Follicular lymphoma
Lymphoplasmacytic lymphoma

–
Aggressive/High grade
Diffuse large B-cell lymphoma

Very aggressive/High grade
Burkitt’s lymphoma

169
Q

What is the most common type of B cell lymphoma?

A

Diffuse large B-cell lymphoma

170
Q

Burkitt’s lymphoma involves extranodal involvement of :

A

In Africa, jaw
Out of Africa, abdomen (ileocaecal junction)

171
Q

Presentation and prognosis of Follicular lympoma

A

Slow growing, incurable
Usually advanced at presentation
Median survival = 9 - 11 years

172
Q

RF Non-Hodgkin’s lymphoma

A

FHx
Age > 50 years
EBV, HTVL-1, SLE, Sjorgren’s

173
Q

Describe Ann-Abor staging

A

I - Confined to single lymph node
II - Involvement of 2 or more lymph nodes on SAME SIDE of diaphragm
III - Nodes on BOTH SIDES of diaphragm
IV - Metastases

A - NO systemic symptoms other than pruritus
B - Systemic symptoms

174
Q

Epidemiology of Hodgkin’s lymphoma

A

M > F
Cases occur in teenagers (13-19) AND elderly (65+)

175
Q

RF Hodgkin’s lymphoma

A

Affected sibling
EBV
SLE
Obese
Post-transplant

176
Q

Why is EBV suggested to have a role in the pathophysiology of Hodgkin’s lymphoma?

A

‘Hides’ in WBC and can result in malignancy

177
Q

EBV = glandular fever, mono

A

ok?

178
Q

Key presentation of Hodgkin’s lymphoma

A

Painless cervical rubbery lymphadenopathy
Pruritus

179
Q

Emergency presentation of Hodgkin’s lymphoma!

A

Infection
Sup. vena cava obstruction w/ ↑ JVP
Sensation of fullness in head
Dyspnoea
Blackouts
Facial oedema

180
Q

Subtypes of Hodgkin’s lymphoma

A

*Classical Hodgkin’s lymphoma - Reed Sternberg cells w/ mirror image nuclei

Nodular Lymphocyte Predominant Hodgkin’s Lymphoma - RS variant, POPCORN CELL

181
Q

Ix Hodgkin’s lymphoma

A

Lymph node excision or BM biopsy
CT/MRI of chest, abd and pelvis for STAGING

182
Q

What indicates a worse prognosis for Hodgkin’s lymphoma?

A

↑ ESR, ↓ Hb
↑ Lactate dehydrogenase

183
Q

Tx Hodgkin’s lymphoma

A

ABVD

Adriamycin
Bleomycin
Vinblastine
Darcarbazine

Shorter course if IA - IIA (less than 3 areas inv) w/ radiotherapy
Longer course if IIA - IVB (more than 3 areas inv)

184
Q

Radiotherapy complications

A

May increase risk of 2nd malignancies
Increase risk of IHD, hypothyroidism and lung fibrosis

185
Q

Chemo side/effects

A

Myelosuppression, nausea, alopecia, infection
Infertility

186
Q

Epidemiology Multiple myeloma

A

M > F
Peak age = 60
More common in Afro-Caribbean

187
Q

Pathophysiology Multiple myeloma

A

Malignant plasma cells produce XS Ig - monoclonal paraprotein
Usually IgA and IgG
Other Ig levels low ∴ immunocompromised

188
Q

Key presentation Multiple myeloma

A

Old CRAB

Old
Calcium raised, Cancer symptoms, Confusion
Renal failure - nephrotic syndrome (∴ THIRST)
Anaemia, infections and bleeding bc BM infiltration
Bone pain

189
Q

GS Ix Multiple myeloma

A

BENCE JONES PROTEINS !!!!! IN URINE

190
Q

Ix Multiple myeloma

A

Bloods - normocytic/chromic anaemia
Rouleaux formation

Serum and urine electrophoresis - B2 microglobulin

XRay - pepperpot skull

191
Q

Pre-form of Multiple myeloma is

A

MGUS
Monoclonal gammopathy of undetermined significance

192
Q

Tx Multiple myeloma

A

Chemo
BM transplant
Bisphosphonates e.g. zolendronate
Correction of anaemia w/ RBC/EPO transfusion

193
Q

What is the prognosis of Multiple myeloma ?

A

Poor
~ 55% 5 year expectancy

194
Q

Causes of Polycythaemia

A

1° Causes - ↑ sensitivity of BM cells to EPO ∴ ↑ RBC production
Polycythaemia rubra vera - mutation of JAK2 gene
1 and congenital Polycythaemia

2° Causes - ↑ RBCs bc ↑ circulating EPO
Chronic hypoxia/EPO producing tumours e.g. renal carcinoma
Poor O2 delivery e.g. high altitude

195
Q

What is Polycythaemia?

A

↑ Hb, packed cell volume and RBCs
i.e. opposite of anaemia

196
Q

Key presentation of Polycythaemia

A

Easy bleeding/bruising
Can be asymptomatic

197
Q

A Px has swollen but painless lymph nodes. However, after drinking some alcohol, the nodes become painful. What condition does he likely have?

A

Hodgkin’s lymphoma

198
Q

When are Howell-Jolly bodies found?

A

After splenectomy or damage to the spleen e.g. sickle cell disease

199
Q

What might you find in a blood film of a Px with megaloblastic anaemia?

A

Hyper-segmented neutrophil polymorphs
(w/ 6 or more lobes in the nucleus)

200
Q

How is von Willebrand’s disease inherited?

A

Autosomal dominant

201
Q

What enzyme degrades von Willebrand factor?

A

ADAMTS-13

202
Q

What type of anaemia can Azathioprine cause?

A

Macrocytic

203
Q

When should you suspect tumour lysis syndrome?

A

Any patient undergoing chemo with easy infections, major electrolyte imbalances on U+E and severe neutropenia

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