Transcript Haematological Irritations

West Herts GP Consultant
Forum (08/09/2010)
Haematological Irritations
Dr Anna Wood
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Topics for discussion
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Polycythaemic indices
Neutropaenia
Lymphocytosis
Platelet clumping
Immunoglobulins
Thrombophilia
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Normal indices
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Female
Hb
12-15g/dl
Hct
0.36-0.44
Rbc
3.86-4.9 x
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Hb
Hct
Rbc
Male
13-16g/dl
0.38-0.49
4.15-5.15 x
Polycythaemic indices
HCT > 0.48
HCT > 0.52
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Polycythaemic indices
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When does it matter?
What are the consequences?
When to investigate and how far?
When to refer?
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1) When does it matter?
Clinical Context
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Age
Persistence or
progression of
abnormality
VTE
Arterial disease
Abdominal mass
Family history
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Drugs
Smoker
Alcohol
Cyanotic heart
disease
Chronic respiratory
disease
Hypertension
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Definition and Classification of
Polycythaemia
Hct > 0.52 men and > 0.48
women
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True Polycythaemia
Primary (MPD)
Secondary
Congenital eg high o2 affinity Hb
 Erythropoetin- driven
 Drugs eg androgens
 Post renal transplant
Relative Polycythaemia
Reduction in plasma
volume but no increase in red
cell mass
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2) What are the
consequences?
A
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Primary
High risk VTE and
stroke
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Pseudo/Apparent
Unknown risk of VTE
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Secondary
Less risk VTE
B Underlying pathology
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3) When to investigate and
how far?
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History and Examination
If no obvious cause repeat (uncuffed)
Age if young (?) do renal / abdominal ultrasound
Urate levels
(a) well hydrated 87 year old man, non smoker
(b) 37 year old female, non smoker, asthmatic on
ventolin, normal renal USS
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4) When to refer?
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No obvious cause (pseudo) and
persistence of abnormality
If white cells and platelets elevated as
well
If obvious cause AND VTE/ Arterial
history
If there is obvious cause but concern re
necessity for venesection
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Case 1
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82 year old lady
3 episodes VTE and stroke/ now on
indefinite warfarin
GP referral with Hb 17.8 g/dl HCT 0.549
WCC 7.1 x109/L Platelets 268 x 109/L
JAK 2 V617 mutation positive
Primary proliferative polycythaemia
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Case 2
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59 year old male/ heavy smoker
Went to GP as tired
Progressively abnormal Hb and HCT
2007
Hb 16.2 g/dl HCT 0.47
2009
Hb 18.3 g/dl HCT 0.53
Haematology referral
Epo levels 40iu/l (5-25)
Renal cancer
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Mild Neutropaenia
Facts
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Normal Neutrophil count
1.9 – 7.0 x109/L
Ethnic variant
0.8 x 109/L
When does it matter?
Clinical context
? Recent acute/ severe viral infection
Drugs
Other abnormal haematological indices
History of recurrent bacterial infections
Disease associations eg SLE
Degree of neutropaenia
Blood film appearance
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Importance of blood film
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Neutropaenia
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What are the consequences?
Of neutropaenia < 0.5 x 109/L
Of underlying condition
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Case provided
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68 year old man of Asian origin,
negative viral screen, on no medication,
well.
Neutrophils 1.16 x 109/L
How often should this be monitored?
Request blood film and if normal
recheck in 3-4 months
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Lymphocytosis
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Normal 1.2 - 3.5x109/L
Age
Causes
Clinical context
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Causes isolated lymphocytosis
in elderly
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CLL
Low grade lymphoma
Monoclonal B-cell Lymphocytosis (MBL)
Splenectomised
Smoking
Endocrine
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CLL
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Diagnosis of lymphocytes > 5 x109/L
Median age
72
Stage A accounts for 80% cases
Outcome for stage A
50% stable and never require treatment
50% progress and require treatment
Median survival 9 years
20% Stage B and C require treatment 18
Case provided (CLL)
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76 year old lady, IHD on various
medications
Lymphocytes 6 x 109/L
How often to monitor?
When to refer?
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How often to monitor?
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First must request blood film.
If no lymphadenopathy, organomegaly,
B symptoms and otherwise normal
blood count 6 monthly and there is an
‘assumption’of CLL
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CLL blood film
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Other lymphoproliferative
disorder
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When to refer
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If rapid rise in lymphocytes (LDT < 6 months)
If at diagnosis presence of ‘B’ symptoms,
hepatosplenomegaly or additional abnormality in
blood count
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Patient choice ie would like a substantive diagnosis
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Blood film suggestive of other low grade
lymphoproliferative disorder.
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Initial Lymphocytes > 30 x 109/L
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Importance of Red blood cell
count
Raised RBC with Microcytosis
 Iron deficient primary
Thalassaemia trait
polycythaemia
Hb 9 g/dl MCV 62 RBC 6.0
Hb 16 g/dl MCV 77 RBC 5 x
x 109/L
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109/L
Compare
 Primary polycythaemia
Hb 20 g/dl MCV 90 x 109/l
RBC 6 x 109/L
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Platelet clumping
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In vitro phenomenon
Of no clinical consequence
Lab should delete platelet count
Citrated sample sometimes helps
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Platelet clumping
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Polyclonal increase
immunoglobulins
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Connective tissue disease
Chronic infections (HIV TB)
Non haematological malignancies
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Thrombophilia
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Young girl with family history VTE
Prior to COCP
Should we test for thrombophilia?
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Why do thrombophilia tests?
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Predict the rate of recurrence in a
patient with VTE
Predict the risk of 1st VTE in family
member
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1) Predicting rate of
recurrence VTE
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Recurrence rate
Post op
Minimal
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15-20%
First 2 years
Other provoked Spontaneous
10%
20%
No additional risk factor for recurrence if
thrombophilia
Single biggest risk factor for recurrence VTE
is VTE
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2) Prediction VTE in
unselected cases
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Background incidence VTE women
childbearing age 1 per 10,000 per year
COCP
3x risk
COCP + Factor V Leiden
30x risk
Screening all women not economically
viable
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3) Selective screening of
asymptomatic relatives
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Thrombotic family
Defect identified
Risk VTE asymptomatic
family member is
UNCERTAIN unless
HIGH RISK
antithrombin deficiency
Protein C /S deficiency
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Thrombotic family
Defect not identified
Risk VTE asymptomatic
family member is
UNCERTAIN
False reassurance
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Testing females prior COCP
with family history
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BSCH guidelines 2010
First degree relative VTE
Tested positive
Tested negative
Not tested
Thrombophilia testing not recommended
Consider an alternative contraception or transdermal
HRT
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Thank you
Any Questions
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