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History
Assess iron status -diet
Film appearance
Ferritin -drugs
-alcohol
B thal trait
Establish response Investigate for ACD
(Putative a thal trait)
Counsel
Refer to Haematologist for further investigation
Microcytic anaemia -questions
Anaemia
Infections
Painful crises
Stroke
Leg ulcers
Visual loss
Chronic organ damage
– Kidneys, lungs, joints, heart
Sickle cell basic facts
Not exclusive to black races
Hb SS steady state Hb 7-9g/dl
Transfuse only for certain indications
Milder forms may have normal Hb so
always do Hb Screen if suspicion or pre op
% Hb S is constant and bears no relation
to acute crisis
Patients have functional asplenia,
therefore high risk of infection
Macrocytic anaemia - questions
Is it a new problem?
Is it due to alcohol?
Is it due to diet /haematinic deficiency
Is it due to drugs?
When to refer to the haematologist?
Management of low B12
History
– Diet, previous surgery, GI disease
IF antibodies
– Schilling test no longer available
A: absence of B symptoms
B: fever, night sweats, weight loss
Investigation of suspected lymphoma
Follicular lymphoma
Diffuse large B-cell lymphoma
Hodgkins lymphoma
Neutropenia questions
Is it isolated or associated with other
cytopenias?
Is it recent or longstanding?
Is it associated with history of infection?
Common causes of isolated neutropenia:
– Viral infection (acute and chronic eg HIV)
– Drug related (look up list)
– Ethnic (longstanding, asymptomatic, no other
cause)
Severe neutropenia
Infection risk increased if neuts<1.0 x 109/l
High risk of infection of neuts<0.5
Risk is also related to duration of neutropenia
and neutrophil function
Consider prophylaxis of infection if neuts<0.5
Fever in Neutropenic patient is a medical
emergency – investigate and treat empirically
with broad spectrum antibiotics pending micro
results. Hospital should have a protocol.
Paraproteins
Common incidental finding, esp in elderly
Due to clonal proliferation of plasma cells
Is it significant?
Causes:
– secondary: (autoimmune disorders/infections)
– Malignant: myeloma/lymphoma
– Uncertain: MGUS (20% evolve into clonal
disorder)
Myeloma
Clinical features due to combinations of:
– Marrow failure
– immunsuppression
– Paraprotein (renal disease)
– Bone disease (pain, fractures, lytic lesions/hypercalcaemia)
Investigation of PP
– History
– FBC, Biochem, Serum electrophoresis
– Bone X rays
– Bone marrow
– MGUS is defined by lack of evidence for malagnancy or reactive
cause.
Haemostasis and
thrombosis
Physiology of haemostasis
Bleeding disorders (congenital)
Massive blood loss
DIC
Anticoagulant drugs
Haemostasis needs…
Platelets
Vessel Wall
Clotting Factors
PT
The starter motor……..
Switched off
by Tissue Factor
Pathway
Inhibitor
TT
•TT : I
ACQUIRED
•Hepatocellular disease
•Vitamin K deficiency (II, VII, IX, X): obstructive
jaundice, haemorrhagic disease of the newborn
•Disseminated intravascular coagulation (DIC)
•Massive blood transfusion
•Warfarin (monitoring test based on PT)
•Gross overheparinisation, some lupus anticoagulants
CAUSES OF A PROLONGED ACTIVATED
PARTIAL THROMBOPLASTIN TIME
CONGENITAL
•Coagulation factor deficiencies: XII, XI, IX, VIII, X,
V, II, I
ACQUIRED
•Hepatocellular disease
•Vitamin K deficiency
•Disseminated intravascular coagulation
•Massive blood transfusion
•Heparin (monitoring test based on APTT)
•Lupus anticoagulants
CAUSES OF A PROLONGED
THROMBIN TIME
CONGENITAL
•Dys/hypofibrinogenaemia
ACQUIRED
•Hepatocellular disease: dys/hypofibrinogenaemia
•Disseminated intravascular coagulation:
hypofibrinogenaemia
FDPs
•Heparin
Haemophilia
A = reduced VIII; B = reduced IX
Both sex-linked recessive
Queen Victoria
Intronic rearrangements, point mutations, gene deletions
Haemophilia A 1 in 10,000 male infants
Prolonged APTT (normal PT and TT)
Mild Rx: Tranexamic acid, DDAVP (not HB)
Severe Rx: factor replacement recombinant or pooled donor
Home prophylaxis
Past problem with HIV, now HCV ??? CJD
Von Willebrand’s Disease
Functions of VWF:
Therefore in VWD see long APTT (low VIII) and bleeding where
VWF platelet interaction important
Von Willebrand’s Disease
Treatment options
DDAVP, antifibrinolytics
NB DDAVP causes fluid retention. NOT for type 2B
Intermediate purity VIII concentrate
VWF concentrate (NB takes hours for VIII to follow so may
need to give both)
If severe bleeding consider platelet infusion and
cryoprecipitate
(NB type I may “auto-correct” in pregnancy)
Massive blood loss
NB arterial
lines !!!!!!!
Heparin
Unfractionated
monitor with APTT (ratio 1.5-2.5 patient’s baseline)
reversal by stopping infusion and (very rapid with protamine
sulphate)
can be hard to anticoagulate children due to low antithrombin levels
bolus then continuous infusion