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)
2
Q
Name some classical anaemia symptoms
A
Fatigue, lethargy
Headaches
Palpitations
Dyspnoea
Brittle hair and nails
Faintness, syncope
Anorexia
3
Q
How are the types of anaemia classified?
A
Mean cell volume
Microcytic - < 80
Normocytic 80 - 100
Macrocytic > 100
4
Q
Name the types of microcytic anaemia
A
TAILS
Thalassaemia
Anaemia of Chronic Disease
Iron-deficiency
Lead poisoning
Sideroblastic
5
Q
What’s the most common type of microcytic anaemia?
A
Iron-deficiency
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
7
Q
RF Iron-def anaemia
A
LICs
High veg diet
Premature infants
Late intro to mixed feed - breast milk contains low iron
8
Q
Key presentation of Iron-Def anaemia
A
Same baseline symptoms
9
Q
Additional signs/symptoms of IDA
A
Atrophic glossitis - tongue inflammation
Koilonychia - spoon shaped nail
Angular stomatitis - inflammation of corners of mouth
10
Q
What conditions protect from Plasmodium falciparum malaria?
A
Sickle Cell Disease
Thalassaemia
G6PDH Deficiency
11
Q
Ix Iron-Def anaemia
A
Blood count & film - RBC microcytic and hypochromic
Poikilocytosis and anisocytosis
Serum ferritin - low
Reticulocyte count - reduced
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
13
Q
Tx Iron-Def anaemia
A
Ferrous sulphate - oral iron
For at least 3 months
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
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
16
Q
Once Iron-Def anaemia is treated, how long does it usually take for iron stores to be replenished?
A
6 months
17
Q
Iron studies for IDA
A
Ferritin - Low/Normal/High
Serum iron - Low
Transferrin - High
Transferrin sat - Low
TIBC - High
18
Q
Iron studies for Anaemia of Chronic Disease
A
Ferritin - Normal/Raised
Serum Iron - Low
Transferrin - Low
Transferrin sat - Normal/Low
TIBC - Low
19
Q
Iron studies for Thalassaemia
A
Ferritin - Normal/Raised
Serum Iron - Normal/Raised
Transferrin - Normal/Low
Transferrin Sat - Normal/Raised
TIBC - Normal/Low
20
Q
Causes of alpha thalassaemia
A
Caused by mutation - gene deletions
↓ a - chain synthesis
21
Q
Where is the gene for alpha-globin chains?
A
Duplicated on both chromosome 16s
22
Q
If 1 alpha-globin gene deletion,
A
Usually normal blood picture
Asymptomatic
23
Q
If 2 alpha-globin gene deletion,
A
Microcytosis with or without mild anaemia
Carrier for alpha-thalassaemia
24
Q
If 3 alpha-globin gene deletion,
A
↓↓ reduction of alpha-chain synthesis
∴ HbH disease
25
Signs of HbH disease
Moderate/Severe haemolytic anaemia
Splenomegaly
Patient is usually not transfusion dependent
26
Where is HbH often found?
Common in parts of Asia
27
If 4 alpha-globin gene deletion,
NO alpha-chain synthesis
Only Hb Barts present (4x gamma chains)
CANNOT CARRY OXYGEN ∴ NOT COMPATIBLE WITH LIFE
28
Key presentation of 4 alpha-globin gene deletion
Infants are stillborn - pale, oedematous w/ huge livers and spleen
29
What are carriers of a-Thalassaemia protected from?
Falciparum malaria
30
Pathophysiology Beta-Thalassaemia
Little/No B-chains results in XS a-chains
∴ these bind w/ delta and gamma chains
∴ ↑ HbA2 and HbF
31
Cause of beta thalassaemia
Point mutations
which result in frame shifts
∴ highly unstable and unusable B-globin
32
Name the subtypes of B-Thalassaemia and brief description
1. B-Thalassaemia Minor
Asymptomatic heterozygous carrier state
2. B-Thalassaemia Intermedia
Moderate anaemia
3. B-Thalassaemia Major
Homozygous
33
How does B-Thalassaemia Minor present?
Mild/absent anaemia - Low MCV + hypochromic
34
DDx of B-Thalassaemia Minor
How can you differentiate?
Iron-Def anaemia
Serum ferritin and iron stores in B-Thalassaemia Minor are normal
35
How does B-Thalassaemia Intermedia present?
Splenomegaly!
Bone abnormalities, recurrent leg ulcers, gallstones!
36
How does B-Thalassaemia Major present?
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)
37
Ix B-Thalassaemia
FBC, Blood film - hypochromic microcytic anaemia
Reticulocytes - increased & nucleated RBCs in periph circulation
**Hb electrophoresis** - ↑ HbF and absent/low HbA
38
Why does B-Thalassaemia (Major) present with bone expansion?
Extramedullary haematopoiesis (outside marrow)
39
Tx B-Thalassaemia
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
40
Complications of blood transfusions
↑ 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)
41
S/E of iron-chelating agents
Pain, deafness, cataracts, retinal damage
42
Other than iron-chelating agents, whats a way to prevent XS iron during blood transfusion?
Ascorbic acid
↑ Urinary excretion of iron
43
Big complication w/ Tx of B-Thalassaemia
IRON OVERLOAD!
44
Brief pathophysiology of Sideroblastic anaemia
Defective protoporphyrin production
∴ impaired incorporation of iron to form haem
∴ build up of iron in RBCs
∴ immature and dysfunctional
45
Congenital cause of Sideroblastic anaemia
X-Linked, mutation in ALAS2 gene
46
Acquired causes of Sideroblastic anaemia
XS alcohol
Vit B6 def
Lead poisoning
47
Key presentation of Sideroblastic anaemia
Fatigue, hepatosplenomegaly
48
Ix of Sideroblastic anaemia
**1st**
Complete blood count -
if normal/low = congenital cause
if normal/high = acquired cause
Iron studies
**GS**
Peripheral blood smear !
49
What would you find in a peripheral blood smear of Sideroblastic anaemia?
RBCs w/ :
Basophilic stippling
Ringed sideroblasts
Pappenheimer bodies
50
Tx of Sideroblastic anaemia
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
51
What type of anaemia is Anaemia of Chronic disease usually?
Often normocytic but CAN be microcytic - esp in Crohn's and RA
52
Name some chronic diseases often related to A of CD
TB
Crohn's
RA
SLE
Malignant diseases
53
Pathophysiology of A of CD
↓ Release of iron from BM to developing erythroblasts
∴ Inadequate erythropoietin response to anaemia
54
Tx of A of CD
Treat underlying cause
EPO therapy - used in renal and inflammatory diseases
55
S/E of EPO therapy
Flu-like symptoms
HTN
Thromboembolism
56
Main causes of Normocytic anaemia
Acute blood loss
A of CD
Endocrine disorders e.g. hypopituitarism, hypothyroidism, hypoadrenalism
Renal failure
Pregnancy
57
Ix Autoimmune haemolytic anaemia
Coombs' test = positive
Spherocytes often present
58
What are some markers for cell turnovers?
↑ Lactate dehydrogenase
↑ Unconj bilirubin
↓ Haptoglobin
59
What is the role of haptoglobin?
Binds to free haemoglobin
i.e. when RBC's broken down, binds to it to prevent ROS
∴ ↓ levels during haemolytic anaemia
60
How is sickle cell disease inherited?
Autosomal recessive
∴ 1 in 4 chance of disease, 50% of being a carrier if parents is carrier
61
In which ethnicity is sickle cell disease most common?
Afro-Caribbean
62
Cause of sickle cell disease
Mutation of B-globin gene (glutamic -> valine)
Results in HbS variant
63
RF of sickle cell disease
FHx
Genetics
64
What are sickle cell disease carriers protected from?
Plasmodium falciparum malaria
65
Key presentation of sickle cell disease
Usually identified in 1st year of life
Asymptomatic except in 'stress' - hypoxia, cold, dehydration, acidosis
Acute pain in hands and feet - dactylitis
66
Why don't sickle cell disease Pxs usually have anaemia symptoms?
Because chronic haemolysis = stable Hb levels
67
What happens if Hb levels in sickle cell disease patients suddenly falls?
Splenic sequestration/infarction
Gallstones
Aplastic anaemia
Leg ulcers
68
Further sign of sickle cell disease
Jaundice
69
Ix sickle cell disease
FBC - ↓ Hb, ↑ reticulocytes
Blood film - sickled erythrocytes
**Hb electrophoresis** - confirms diagnosis
Hb SS present, absent HbA
70
What screening process do we aim to do for sickle cell disease and why?
Neonate blood/heel prick test
Pneumococcal prophylaxis
71
What screening process do we aim to do for sickle cell disease and why?
Neonate blood/heel prick test
Pneumococcal prophylaxis
72
Tx for sickle cell disease
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
73
Complications of sickle cell disease
Pulmonary HTN and chronic lung disease
= most common cause of death in adults w sickle cell disease
74
Cause of Spherocytosis & Elliptocytosis
Def in RBC structural membrane protein - **SPECTRIN**
75
Inheritance of Spherocytosis & Elliptocytosis
Autosomal dominant
But can occur spontaneously
76
Difference between Spherocytosis & Elliptocytosis
Spherocytosis - vertical deformity
Elliptocytosis - horizontal deformity
77
Pathophysiology of Spherocytosis & Elliptocytosis
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**
78
Key presentation of Spherocytosis & Elliptocytosis
Neonatal jaundice (Can sometimes be delayed/Be completely asymptomatic)
Splenomegaly symptoms (exacerbated during infection)
79
Other signs/symptoms of Spherocytosis & Elliptocytosis
XS bilirubin - gallstones
Leg ulcers
80
Ix Spherocytosis & Elliptocytosis
Blood film - presence of Spherocytosis & reticulocytes
FBC & Reticulocyte count - ↓ Hb, **↑ Reticulocytes**
Direct anti-globulin (Coombs' test) = NEGATIVE
Rules out autoimmune haemolytic anaemia
81
Tx Spherocytosis & Elliptocytosis
Splenectomy
Folic acid
82
G6PDH full form
Glucose-6-Phosphate Dehydrogenase
83
Epidemiology of G6PDH Deficiency
Mainly males - **X-Linked recessive condition**
But can effect F
↑ Prevalence in Mediterranean, African, Middle/Far Eastern
84
Triggers of G6PDH Deficiency
Infections
Food
Medications e.g. anti-malarials
85
What does G6PDH Deficiency protect Px from?
Plasmodium falciparum malaria
86
Pathophysiology of G6PDH Deficiency
G6PDH vital in hexose monophosphate shunt
∴ maintains glutathione in reduced state
Glutathione protects RBCs from oxidative stress
87
G6PDH Deficiency susceptible to?
Oxidative stress!!
88
Key presentation of G6PDH Deficiency
Asymptomatic until exposed to oxidative stressor
Neonatal jaundice
89
What can make G6PDH Deficiency worse?
FAVA BEANS
90
Where is gene for G6PDH located?
Chromosome Xq8, near factor VIII gene
91
Ix G6PDH Deficiency
1st
FBC - anaemia, ↑ reticulocytes
GS
Blood film - bite and blister cells, Heinz bodies
92
Describe the G9PDH enzymes in G6PDH Deficiency
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
Tx G6PDH Deficiency
Stop triggering factors/fava beans
Also, henna can trigger
Transfusion if severe
Splenectomy usually NOT HELPFUL
94
Other signs/symptoms of G6PDH Deficiency
Back pain
Dark urine
Nausea
95
FEVER + EXOTIC TRAVEL =
**MALARIA** until proven otherwise
96
What is Aplastic anaemia?
Bone Marrow failure
97
Definition of Aplastic anaemia
Pancocytopenia with hypocellularity of BM
98
Causes of Aplastic anaemia
Can be congenital but usually acquired
Infections - EBV, HIV, TB, Hepatitis
Chemo drugs
Abx - carbamazepine, azathioprine
99
Pathophysiology of Aplastic anaemia
↓ number of pluripotent stem cells
∴ ↓ haemopoiesis
∴ ↓ no. new RBCs to replace old ones undergoing apoptosis
100
Key presentation of aplastic anaemia
Classic anaemia symptoms
Recurrent infections
101
Other signs/symptoms of Aplastic anaemia
↑ Bruising
↑ Bleeding (esp from nose and gums)
102
Ix Aplastic anaemia
FBC - pancytopenia
Reticulocyte - low or absent!
**BM biopsy** hypocellular marrow w/ ↑ fat spaces
103
Other than Aplastic anaemia, what are some other causes of pancocytopenia?
Drugs
H lymphoma
NH lymphoma
Myeloma
SLE
104
Tx Aplastic anaemia
Manage underlying cause
Blood & platelet transfusion
BM transfusion
**Immunosuppressive therapy** -
anti-thymocyte globulin (ATG) and ciclosporin
105
Two words, explain Aplastic anaemia
Reduced haemopoiesis
106
What is the main concern with Aplastic anaemia?
Why? How do we manage this?
Infection
Bc Px has severe neutropenia
Give broad-spec parenteral Abx URGENTLY
107
When is immunosuppressive therapy !! (Aplastic anaemia) (first line? idk not sure)
Over 40
Below 40 w/ severe disease who do NOT have HLA identical sibling donor
Transfusion dependent Px
108
When is BM transplant appropriate in Aplastic anaemia Px?
From HLA identical sibling or donor treatment of choice in those under 40
109
What is important to be aware of for candidates of BM transplant?
Blood/platelet transfusions to be used cautiously
To avoid sensitisation
110
Name the 3 main causes of Macrocytic anaemia
Megaloblastic
1. B12 deficiency (pernicious anaemia)
2. Folate deficiency
Non-Megaloblastic
3. XS alcohol
111
Where is B12 found?
Meat, fish, dairy products
NOT plants!
112
RF Pernicious anaemia
Elderly (>60)
Female
Fair-haired, blue eyes (e.g. Scandinavian)
Blood group A
Autoimmune diseases
Vegan
113
Pathophysiology Pernicious anaemia
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
Key presentation of Pernicious anaemia
Baseline anaemia symptoms
115
Other signs of Pernicious anaemia
Glossitis
Angular stomatitis
Jaundice
Neurological symptoms - only with severe def
116
Ix Pernicious anaemia
FBC & blood film - macrocytic RBCs
Serum B12 - low
**GS**
Intrinsic factor antibody screen (but low sensitivity)
117
What should you never give to a Px with Pernicious anaemia?
DO NOT GIVE FOLIC ACID!!
Causes fulminant neurological deficits
118
Tx Pernicious anaemia
If B12 def due to malabsorption, injections are needed
∴ IM hydroxocobalamin
If dietary, oral B12
119
Where is folate found?
Green veg e.g. spinach, broccoli
Also, nuts, yeast & liver
120
Causes of folate deficiency anaemia
Poor dietary intake - poverty, alcoholics, elderly
Malabsorption - Crohn's, coeliac
Increased demand - pregnancy
Anti-folate drugs - methotrexate, trimethoprim
121
How can you differentiate between folate deficiency anaemia and pernicious anaemia?
Folate deficiency anaemia does not have neuropathy, while pernicious anaemia does.
122
Key presentation of folate deficiency anaemia
Anaemia symptoms
May be asymptomatic
123
Ix folate deficiency anaemia
Blood count & film - RBCs are macrocytic
Serum & RBC folate - LOW
124
Tx folate deficiency anaemia
Treat underlying cause
Folic acid supplements - NEVER WITHOUT B12 (unless normal B12 level)
125
Define megaloblastic anaemia
Inhibition of DNA synthesis
∴ RBCs can't progress onto mitosis
∴ continued growth without division
∴ macrocytosis
126
Epidemiology of acute myeloid leukaemia
Older adults (peak at 60 years)
M > F
Most common acute leukaemia of adults!
127
What is AML?
Neoplastic, uncontrolled proliferation of myeloid blast cells
128
What do myeloblasts give rise to?
Basophils, Neutrophils and eosinophils
129
RF AML
Radiation e.g. prev chemo
Down's syndrome
130
Key presentation AML
Anaemia - dyspnoea, fatigue, angina, claudication
Low WBC - recurrent infection, mouth ulcers
Low platelets - bleeding, bruising
*Hepatosplenomegaly
*Gum hypertrophy
131
Ix AML
**1st**
Peripheral blood smear! - to see if myeloblasts present
FBC
**GS**
BM biopsy!! to check for ↑ blast cell %
AUER RODS!
132
Blast cell % normal vs AML
Normal = 1-2%
AML = can be > 20%
133
What Ix required to differentiate between ALL and AML?
Microscopy
Immunophenotyping
Molecular methods
134
Tx AML
Blood / BM / stem cell transfusions
Chemotherapy
Prophylactic antivirals, antibiotics, antifungals! (bc of neutropenia)
IV fluids
ALLOPURINOL - prevents tumour lysis syndrome
135
Name a subtype of AML
Disseminated intravascular coagulation
Occurs when there's a release of thromboplastin
136
What is ALL?
Uncontrolled prolif of blast cells - lymphoblasts
Life-threatening!!
137
Epidemiology ALL
CHILDREN!
Most common between 2 - 4 years
138
RF ALL
Ionising radiation e.g. X-rays during pregnancy
Down's syndrome
139
What do lymphoid cells give rise to?
T and B cells
140
Key presentation ALL
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
Ix ALL
**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
Tx ALL
Blood / BM / stem cell transfusions
Chemotherapy
Prophylactic antivirals, antibiotics, antifungals! (bc of neutropenia)
IV fluids
ALLOPURINOL - prevents tumour lysis syndrome
143
What is CML?
**Overproduction** of myeloid progenitor
144
Epidemiology CML
Occurs most often between 40 - 60 years
Slight M > F
145
Cause of CML
Mutation in **Philadelphia chromosome** causes ineffective and XS granulocyte to be produced
146
Key presentation CML
Anaemia symptoms
Splenomegaly ∴ abd discomfort
Hyperviscosity - headaches, thrombotic events
Hypermetabolic - weight loss, malaise, night sweats
Gout - due to purine breakdown
147
Ix CML
FBC - ↑↑ WBC w whole spectrum of myeloid cells
BM aspiration - increased cells
GS : **Genetic testing** -
PHILADELPHIA CHROMOSOME, BCR-ABL gene, t(9;22)
148
Tx CML
ORAL IMATINIB - specific BRCR-ABL tyrosine kinase inhibitor
Stem cell transplant
149
What is CLL?
Insidious accumulation of incompetent lymphocytes
150
RF CLL
M > F
Genetics (trisomies, mutations, deletions)
Pneumonia may be a trigger
> 70 years
151
Which is the most common leukaemia?
CLL
152
Key presentation of CLL
Usually asymptomatic
∴ Found on routine FBC
May be anaemic or infection prone
153
If CLL is severe, how might it present?
Weight loss, Night sweats, Anorexia
Large, rubbery, non-tender lymph nodes
Hepatosplenomegaly
154
Ix CLL
**Blood film** - SMUDGE CELLS
↑ WBC w/ v high lymphocytes
155
Complications of CLL
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
Progression of CLL
May stay stable for years, might regress!
Death usually due to complications
157
Tx CLL
Blood transfusions
**HUMAN IV IG**
**Rituximab**
Chemo or Radiotherapy
Stem cell transplant
158
Prognosis of CLL
RULE OF 3
1/3 will never progress
1/3 progress slowly
1/3 progress actively
159
Types of lymphoma
Hodgkin's and Non-Hodgkin's
160
Name the two types of Non-Hodgkin's lymphoma
B Cell lymphoma - Majority of cases.
Can be indolent, aggressive, v aggressive
T Cell lymphoma
161
Non-Hodgkin's lymphoma has a strong link with?
EBV
Burkitt's lymphoma
162
Key presentation of Non-Hodgkin's lymphoma
**Nodal involvement -** superficial lymphadenopathy
**Extranodal -**
GI - bowel obstruction
BM - fatigue, easy bruising/bleeding, recurrent infections
Spinal cord - weakness, loss of sensation (usually legs)
163
Ix Non-Hodgkin's lymphoma
Lymph node biopsy OR BM biopsy
CT/MRI of chest, abd and pelvis for STAGING
164
What indicates a worse prognosis for Non-Hodgkin's lymphoma? Why?
↑ Lactose dehydrogenase
sign of ↑ cell turnover ∴ ↑ proliferation
165
What are some differences between Hodgkin's and Non-Hodgkin's lymphoma?
**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
Tx Non-Hodgkin's lymphoma
**R-CHOP** - Combination chemo
**R**ituximab
**C**yclophosphamide
**H**ydroxy-danorubicin
**O** Vincristine (Oncovin is brand name)
**P**rednisolone
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
Rituximab
What is it?
Target?
S/E?
Monoclonal antibody
CD20! expressed on B cell surface
Minimal S/E
168
How is B cell lymphoma classified?
**Indolent/Low grade :**
Follicular lymphoma
Lymphoplasmacytic lymphoma
--
**Aggressive/High grade**
Diffuse large B-cell lymphoma
**Very aggressive/High grade**
Burkitt's lymphoma
169
What is the most common type of B cell lymphoma?
Diffuse large B-cell lymphoma
170
Burkitt's lymphoma involves extranodal involvement of :
In Africa, jaw
Out of Africa, abdomen (ileocaecal junction)
171
Presentation and prognosis of Follicular lympoma
Slow growing, incurable
Usually advanced at presentation
Median survival = 9 - 11 years
172
RF Non-Hodgkin's lymphoma
FHx
Age > 50 years
EBV, HTVL-1, SLE, Sjorgren's
173
Describe Ann-Abor staging
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
Epidemiology of Hodgkin's lymphoma
M > F
Cases occur in teenagers (13-19) AND elderly (65+)
175
RF Hodgkin's lymphoma
Affected sibling
EBV
SLE
Obese
Post-transplant
176
Why is EBV suggested to have a role in the pathophysiology of Hodgkin's lymphoma?
'Hides' in WBC and can result in malignancy
177
EBV = glandular fever, mono
ok?
178
Key presentation of Hodgkin's lymphoma
Painless cervical rubbery lymphadenopathy
Pruritus
179
Emergency presentation of Hodgkin's lymphoma!
Infection
Sup. vena cava obstruction w/ ↑ JVP
Sensation of fullness in head
Dyspnoea
Blackouts
Facial oedema
180
Subtypes of Hodgkin's lymphoma
*Classical Hodgkin's lymphoma - Reed Sternberg cells w/ mirror image nuclei
Nodular Lymphocyte Predominant Hodgkin's Lymphoma - RS variant, POPCORN CELL
181
Ix Hodgkin's lymphoma
Lymph node excision or BM biopsy
CT/MRI of chest, abd and pelvis for STAGING
182
What indicates a worse prognosis for Hodgkin's lymphoma?
↑ ESR, ↓ Hb
↑ Lactate dehydrogenase
183
Tx Hodgkin's lymphoma
**ABVD**
**A**driamycin
**B**leomycin
**V**inblastine
**D**arcarbazine
Shorter course if IA - IIA (less than 3 areas inv) w/ radiotherapy
Longer course if IIA - IVB (more than 3 areas inv)
184
Radiotherapy complications
May increase risk of 2nd malignancies
Increase risk of IHD, hypothyroidism and lung fibrosis
185
Chemo side/effects
Myelosuppression, nausea, alopecia, infection
Infertility
186
Epidemiology Multiple myeloma
M > F
Peak age = 60
More common in Afro-Caribbean
187
Pathophysiology Multiple myeloma
Malignant plasma cells produce XS Ig - **monoclonal paraprotein**
Usually IgA and IgG
Other Ig levels low ∴ immunocompromised
188
Key presentation Multiple myeloma
**Old CRAB**
**Old**
**C**alcium raised, **C**ancer symptoms, **C**onfusion
**R**enal failure - nephrotic syndrome (∴ THIRST)
**A**naemia, infections and bleeding bc BM infiltration
**B**one pain
189
GS Ix Multiple myeloma
BENCE JONES PROTEINS !!!!! IN URINE
190
Ix Multiple myeloma
Bloods - normocytic/chromic anaemia
**Rouleaux formation**
Serum and urine electrophoresis - B2 microglobulin
XRay - **pepperpot skull**
191
Pre-form of Multiple myeloma is
MGUS
Monoclonal gammopathy of undetermined significance
192
Tx Multiple myeloma
Chemo
BM transplant
Bisphosphonates e.g. **zolendronate**
Correction of anaemia w/ RBC/EPO transfusion
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What is the prognosis of Multiple myeloma ?
Poor
~ 55% 5 year expectancy
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Causes of Polycythaemia
**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
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What is Polycythaemia?
↑ Hb, packed cell volume and RBCs
i.e. opposite of anaemia
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Key presentation of Polycythaemia
Easy bleeding/bruising
Can be asymptomatic
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A Px has swollen but painless lymph nodes. However, after drinking some alcohol, the nodes become painful. What condition does he likely have?
Hodgkin's lymphoma
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When are Howell-Jolly bodies found?
After splenectomy or damage to the spleen e.g. sickle cell disease
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What might you find in a blood film of a Px with megaloblastic anaemia?
Hyper-segmented neutrophil polymorphs
(w/ 6 or more lobes in the nucleus)
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How is von Willebrand's disease inherited?
Autosomal dominant
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What enzyme degrades von Willebrand factor?
ADAMTS-13
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What type of anaemia can Azathioprine cause?
Macrocytic
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When should you suspect tumour lysis syndrome?
Any patient undergoing chemo with easy infections, major electrolyte imbalances on U+E and severe neutropenia
