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Transcript
West Herts GP Consultant
Forum (08/09/2010)
Haematological Irritations
Dr Anna Wood
1
Topics for discussion

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Polycythaemic indices
Neutropaenia
Lymphocytosis
Platelet clumping
Immunoglobulins
Thrombophilia
2
Normal indices



Female
Hb
12-15g/dl
Hct
0.36-0.44
Rbc
3.86-4.9 x



Hb
Hct
Rbc
Male
13-16g/dl
0.38-0.49
4.15-5.15 x
Polycythaemic indices
HCT > 0.48
HCT > 0.52
3
Polycythaemic indices

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When does it matter?
What are the consequences?
When to investigate and how far?
When to refer?
4
1) When does it matter?
Clinical Context
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Age
Persistence or
progression of
abnormality
VTE
Arterial disease
Abdominal mass
Family history






Drugs
Smoker
Alcohol
Cyanotic heart
disease
Chronic respiratory
disease
Hypertension
5
Definition and Classification of
Polycythaemia
Hct > 0.52 men and > 0.48
women

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
6
2) What are the
consequences?
A

Primary
High risk VTE and
stroke

Pseudo/Apparent
Unknown risk of VTE

Secondary
Less risk VTE
B Underlying pathology
7
3) When to investigate and
how far?






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
8
4) When to refer?




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
9
Case 1




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
10
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
11
Mild Neutropaenia
Facts










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
12
Importance of blood film
13
Neutropaenia


What are the consequences?
Of neutropaenia < 0.5 x 109/L
Of underlying condition
14
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
15
Lymphocytosis




Normal 1.2 - 3.5x109/L
Age
Causes
Clinical context
16
Causes isolated lymphocytosis
in elderly



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
CLL
Low grade lymphoma
Monoclonal B-cell Lymphocytosis (MBL)
Splenectomised
Smoking
Endocrine
17
CLL






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)




76 year old lady, IHD on various
medications
Lymphocytes 6 x 109/L
How often to monitor?
When to refer?
19
How often to monitor?


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
20
CLL blood film
21
Other lymphoproliferative
disorder
22
When to refer


If rapid rise in lymphocytes (LDT < 6 months)
If at diagnosis presence of ‘B’ symptoms,
hepatosplenomegaly or additional abnormality in
blood count

Patient choice ie would like a substantive diagnosis

Blood film suggestive of other low grade
lymphoproliferative disorder.

Initial Lymphocytes > 30 x 109/L
23
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

109/L
Compare
 Primary polycythaemia
Hb 20 g/dl MCV 90 x 109/l
RBC 6 x 109/L
24
Platelet clumping




In vitro phenomenon
Of no clinical consequence
Lab should delete platelet count
Citrated sample sometimes helps
25
Platelet clumping
26
Polyclonal increase
immunoglobulins



Connective tissue disease
Chronic infections (HIV TB)
Non haematological malignancies
27
Thrombophilia



Young girl with family history VTE
Prior to COCP
Should we test for thrombophilia?
28
Why do thrombophilia tests?


Predict the rate of recurrence in a
patient with VTE
Predict the risk of 1st VTE in family
member
29
1) Predicting rate of
recurrence VTE

Recurrence rate
Post op
Minimal


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
30
2) Prediction VTE in
unselected cases




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
31
3) Selective screening of
asymptomatic relatives



Thrombotic family
Defect identified
Risk VTE asymptomatic
family member is
UNCERTAIN unless
HIGH RISK
antithrombin deficiency
Protein C /S deficiency



Thrombotic family
Defect not identified
Risk VTE asymptomatic
family member is
UNCERTAIN
False reassurance
32
Testing females prior COCP
with family history

BSCH guidelines 2010
First degree relative VTE
Tested positive
Tested negative
Not tested
Thrombophilia testing not recommended
Consider an alternative contraception or transdermal
HRT
33
Thank you
Any Questions
34