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Haematology - Year 2 Revision

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by

Jordan Tsigarides

on 14 June 2016

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Transcript of Haematology - Year 2 Revision

Haematology
Anaemias
Microcytic
Normocytic
Macrocytic
Increased MCV = Increased Size
Malignancies
Bleeding Disorders
Clotting Disorders
Definition: Decreased Hb (lower than reference)
Symptoms
Signs
Fatigue
Dizziness
SOB
Chest Pain/Palpitations
Usually Asymptomatic but...
MCV
Pallor
Systolic Flow Murmur
Signs of underlying cause
Iron Deficiency
Absorption
Loss
Intake
Thalassaemia
Sideroblastic
Likelihood
Specific Signs
I'm a weirdo...
Key Mechanisms
Investigations
1. Confirm Iron Deficiency
Test
/
Haemoglobin
Serum Iron
Ferritin
Transferrin Saturation
Total Iron Binding Capacity
2. Investigate for Cause
Requirements
Microcytic, hypochromic cells

Anisocystosis (unequal size)

Poikilocytosis (abnormal shape) – Pencil Cells + Target Cells (hypochromic with dot in middle)

Where is Iron Absorbed and what pH does it favour?
Therefore... What can affect Iron Absorption?
Duodenum
+
Acidic
1.
Diseases
affecting Duodenum: Coeliac
2.
Surgery
to Duodenum: Small bowel resection
3. Things that
raise pH
(PPIs etc)
4.
Competition
: Lead
Chronic Blood Loss
Upper GI - Ulcer
Lower GI - Tumour/IBD
Menorrhagia
Key Points
Elderly: Must rule out malignancy

Females Reproductive Age: Think Menorrhagia
OGD
Colonoscopy
CT Scan
Treatment
1. Treat Underlying Cause

2. Iron Supplementation

3. Transfuse if:
Hb <70 (<80 if poor physiological reserve)
Symptomatic
Alpha Thalassaemia
Beta Thalassaemia
By Dr Jordan Tsigarides
OSCE
2013/14
OSCE
Finals
OSCE
Finals
Two genes that code for alpha chain (chromosome 16)
Two copies of each gene = 4 alpha-globin alleles
Deletion mutation of 1+ alleles
Decreased quantity of alpha chains
Silent Carrier
Asymptomatic
Alpha Thalassaemia Trait
Mild Anaemia
Normal Electrophoresis
Diagnosed with genetic testing
HbH Disease
More beta than alpha
Moderate Anaemia
Diagnosed with electrophoresis
Hb Bart's Disease
Incompatible with life
Hydrops Fetalis
Most common autosomal recessive haematological disorder

Affects typically mediterraneans but also middle-east, asia..

Two genes coding for beta-chain

b = normal beta chain
b+ = some beta chain dysfunction
b0 = total beta chain dysfunction
Pattern
Severity
Type
Treatment
b-Thalassaemia
Minor
(Trait)
b-Thalassaemia
Intermedia
b-Thalassaemia
Major
b0 b0
Homozygous
b0b+
b+b+
Homozygous
b+ b
b0 b
Heterozygous
Minor
Asymptomatic
Normal/Low MCV
Moderate
Moderate Anaemia
Splenomegaly + Bone Abnormalities
Severe
Childhood - Failure to thrive
Severe anaemia (3-6mth)
Usually None
Transfusions
Iron Chelation
Folic Acid
Above + Splenectomy + Bone Marrow Transplantation
Frontal bossing
Maxillary overgrowth
Hair-on-end appearance on skull x-ray
Expansion of marrow
Thinned cortex
Extramedullary Haematopoeisis
+ hepatomegaly
+ signs of iron overload
What are Sideroblastic Anaemias?
Group of acquired + inherited anaemias
Accumulation of iron in primative red cells
Characterised by ring sideroblasts (marrow)
MCV
Anaemia of Chronic Disease
Aplastic Anaemia
Acute Blood Loss
Haemolytic Anaemias
Dilutional
Mixed Picture
Pathophysiology
Normal cellularity
Stem cells present
No stem cells
Pancytopaenia
Causes
Idiopathic (67%)
Congenital: Fanconi's Anaemia
Drugs: Chemo, Anti-epileptics
Radiation
Infections - HBV, EBV, TB, HIV
Pregnancy
Treatment
Replace the blood! - Transfuse

Prevent Infections! - G-CSF + Abx

Replace the Marrow!

Immunosuppression? Why not...
What is the importance of the reticulocyte count?
Monitors the bone marrow response to low red cell count

In healthy marrow - reticulocyte count should increase when red cells/Hb decreases
Types
Defect in RBC internal contents
Defect in RBC membrane
Defect in external environment
Sickle Cell Disease
G6PD Deficiency
Hereditary Spherocytosis
Metallic Valve
Infection
Autoimmune Haemolytic Anaemia
Transfusion Reaction
Drugs
Hypersplenism
Abnormal cells trapped in the spleen and phagocytosed
Sickle Cell Disease
G6PD Deficiency
Hereditary Spherocytosis
Hereditary Spherocytosis
MCV
Megaloblastic
Non-megaloblastic
Defect in DNA synth leading to larger cells
B12/Folate Deficiency
Alcohol
Liver Disease
Reticulocytosis
Signs of anaemia
Jaundice
Haemoglobinuria (red urine)
Splenomegaly
Autosomal Recessive
HbS formed due to point mutation on chromosome 11
HbS becomes rigid/sickle under stress (cold/hypoxia/dehydration)
Crises = occlusion of veins/arteries by sickle cells
Treatment = Supportive during crises - Fluids/Antibiotics/Analgesia + Hydroxyurea (HbF increased)
Normocytic Anaemia
Splenomegaly
Auto-Splenectomy
Infections
Chest Crisis
Bone Pain
Avascular Necrosis
Dactylitis
Anaemia
Jaundice
Gallstones
Reticulocytosis
Aplastic Crisis
Howell Jolly Bodies
X-Linked
Glucose-6-Phosphate Dehydrogenase
= Enzyme that maintains levels of
reduced glutathione (GSH)
GSH helps to protect red cells from oxidative damage
No G6PD =
Haemolysis
of red cells when undergoing stress (infection/drugs)
Heinz Bodies
Bite Cells
Autosomal Dominant
Defect in cell membrane = increased red cell fragility
Weak 'spherocytes' destroyed by spleen
Splenectomy
Other things to cover:
The Spleen
Blood Transfusions
Leukaemias/Myeloproliferative Dx (see my other lecture)
B12 Deficiency/Pernicious Anaemia (PA)
Causes
Reduced intake
Impaired Absorption + Pernicious Anaemia
Investigations
Treatment
Megaloblastic Anaemia
Vit B12 levels LOW
Anti-Parietal Abs (90%)
Anti-IF Abs (50%)
Schilling's Test - Determines Cause
IM Hydroxycobalamin
Remember Folate!
Associations (PA)
Gastric Ca
Autoimmune Dx
Folate Deficiency
+ Anaemia symptoms...
Absorbed in the Jejunum

Much more likely in B12
Causes
Reduced intake (Alcoholism)
Increased demand (Pregnancy)
Drugs (Methotrexate)
Investigations
Megaloblastic Anaemia
LOW Serum Folate
Treatment
Oral Folate
Remember B12!
Lymphadenopathy
Lymphadenitis
Acute
Chronic
Viral
Bacterial
Traumatic
Follicular Hyperplasia = B-Cell
Paracortical = T-Cell
Granular = TB
Lymphoma
Non-hodgkins (NHL) (80%)
Hodgkins (20%)
Multiple Myeloma
Classical
Nodular Lymphocytic
Nodular Sclerosing
Mixed Cellularity
Lymphocyte Depleted
Lymphocyte Dominant
Types of B-Cell NHL
Naive B-Cell = Small Lymphocytic

Mantle Cell = Mantle Cell

Centroblast = Diffuse Large B-Cell

Centrocyte = Follicular

Marginal = Marginal Zone Lymphoma
Types of T-Cell NHL
V. Rare!!
Cutaneous T-Cell Lymphoma (mycosis fungoides)

Enteropathy associated Lymphoma (Coeliac)
Classification
-- EBV
The big difference
Reed-Sternberg Cell (Owl Eyes)
Presentation in Lymphomas
B-Symptoms:
Fever
Night Sweats
Weight Loss
Lymphadenopathy
Pain with Alcohol in HODGKINS
Compression on organs
Extranodal Spread
Good Prognosis - 80% Cure Rate
Hodgkin's Lymphoma
Malignancy of the Plasma Cell

Resulting in increased monoclonal paraprotein (usually IgG or IgA)

Also associated with excess free light chains (kappa/lambda)
Bence-Jones protein seen in urine

Presents in >60s

Clinical Features
C
R
A
B
alcium (hypercalcaemia)
enal impairment
naemia/Amyloidosis
one Lesions/Bence-Jones protein
FBC, U+Es, LFTs, ESR/CRP, Bone
Blood film
Serum Protein Electrophoresis
Serum Free Light Chain Ratio
If ratio abnormal = active myeloma
Bence-Jones Protein (urine)
Skeletal Survey
Normal
MM
Treatment
Treat Complications
Bisphosphonates/Analgesia/Steroids/Radiotherapy for Bone Pain
Fluids/Dialysis/Chelation for renal impairment
EPO/Transfusions for anaemia
Vaccines/Antibiotics for Infections
Treat Disease
Chemotherapy
Stem Cell Transplantation
Median Survival = 5 years
Haemophilias A & B
Platelet Disorders
Idiopathic Thrombocytopaenic Purpura
Key Points
Coagulopathies
Disseminated Intravascular Coagulation (DIC)
'Death is Coming'
Clotting Diseases
Deep Vein Thrombosis
Haemostasis
1. Vasoconstriction
2. Platelet Adhesion
3. Stabilisation
4. Self Limitation
Tissue plasminogen activator: Plasminogen to Plasmin
Antithrombin
Protein C
Protein S
Coagulation Cascade
Factor II
INR/PT
aPTT
Increased Consumption
Decreased Function
Immune
Non-Immune
ITP
Drugs
DIC
TTP
HUS
Inherited (rare)
Acquired
Aspirin
Autoantibodies made to Platelets
1. Acute: Children, Post Infection, Self limiting
2. Chronic: Adults (usually women), Steroids, Splenectomy (if severe)
Heparin: HITT
Penicillin
Transfusions
Abnormal and unregulated activation of the clotting cascade throughout the body
Characterised by:
1. Widespread
Clotting
2.
Bleeding
Platelets/Clotting Factors used up
Over-expression of Tissue Factor
Causes:
Anything with intense stress/inflammation/trauma
Test
/
Platelets
INR/PT
aPTT
D-Dimer/FDPs
Fibrinogen
INR >6 = Severe
<50 = Severe
<1 = Severe
Practical Points
Platelets: Normally 150-400

<50: Echymoses + Petechiae
<20: Spontaneous Mucocutaneous Bleeding

Don't usually treat until <50 or actively bleeding
Treatment:
Replace products if severe
Treat underlying condition
10-50% Die
Acquired
Inherited
Haemophilia A
Haemophilia B
vWB Disease
Liver Disease
Vit K Deficiency
X-linked recessive - Only men get the disease! :-(
Most diagnosed v.young (depending on severity)
<1% - Severe
- Haemarthroses/spontaneous - 1 month
1-5% - Moderate
- Easy bleeding -
8 months
5% - Mild
- 36 months

How do you remember which one is which?
Coagulation Cascade
Factor II
INR/PT
aPTT
Inherited
Acquired
Factor V Leiden (10%)

Prothrombin Mutation (5%)

Protein C + S
Antithrombin

Factor V doesn't inactivate
Elevated Prothrombin
Immobility
Surgery
Malignancy
Trauma
Drugs (COCP + HITT)
APLS
Pregnancy
V. Common - >50% below knee
Swelling
Warmth
Tenderness
Erythema
D-dimer: Sensitive but not specific
USS Doppler
Prevention/Treatment
Everyone gets Dalteparin...
Surgical patients - Stockings also
Bridging
Dalteparin Treatment Dose
Warfarin
When INR in range (2.5) stop Dalteparin
OR
NOAC (Rivaroxaban etc)
Duration of Treatment
Anticoagulants
Heparin: Increased effect of antithrombin III by 1000 times (aPTT)

Warfarin: Inhibits Vitamin K Dependent Clotting Factors... (INR)
2
7
9
10
Managing a raised INR
Mrs CJ - 68yrs
Last week she went on holiday and had acute onset weakness and numbness in her hand
Went to local hospital - TIA
CT Head - NAD
CTCA - NAD
Returned home - Not feeling right - Hand still not able to grip well
Woke up this morning - Felt much worse - Unable to grip - Niece noted some facial droop
Admitted - CT Head = R MCA Infarction
PMH
Sjogrens
Not much else
NKFH
Drug Hx
HRT
Stuff for Sjogrens
Not much else
Bloods
Test
Result
/
/
WCC
Platelets
Haemoglobin
MCV
Sodium
Potassium
Urea
Creatinine
INR
aPTT
Cholesterol
13.3
126
437
88.0
137
4.7
4.8
77
0.93
56
6.3
What's the clever diagnosis?
What tests to do next?
Antiphospholipid Syndrome

Diluted 50:50 aPTT
Lupus Anticoagulant
Anticardiolipin
ANA
Naive B-Cell
Lymph Node Follicle
Plasma Cell
3 months
Reversible cause
6 months
No cause found
Lifelong
Recurrent
Thrombophilia/Unreversible Cause
Well's Score
Full transcript