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Haematology

Surgery and anaesthesia make heavy demands on departments of haematology


and blood transfusion. Consultation between the anaesthetist and haematologist
should be frequent both in the operating theatre and in the intensive therapy unit if
the provision of, for example, appropriate blood components and the correct
investigation of the bleeding patient are to proceed smoothly and expeditiously. The
UK Department of Health's policy that hospi- tal transfusion committees should now
exist in every hospital should result in improved efficiency and appropriateness in
the use of blood and blood products.
ANAEMIA
Anaemia is present when the red cell mass (the erythron) is reduced below the
reference range for the patient's age and sex. There are many causes, which are
classified conventionally as:
Blood loss- may be acute or chronic. Failure of erythropoiesis - resulting from, for
example, inade- quate supplies to the bone marrow of nutrients: iron, vitamin Bi2,
folate, some hormones or protein. Erythropoiesis may be impaired by bone marrow
infiltration in leukaemia or other malignant disease. Anaemia of chronic disease is
the term used for the secondary anaemias of chronic inflammation, infection and
malignancy (when the marrow is not infiltrated). Such anaemia is also seen in
rheumatoid arthritis and chronic renal failure. Shortened red cell lifespan. The
haemolytic anaemias are subdi- vided into: inherited, e.g. hereditary
spherocytosis, sickle cell anaemia and some red cell enzyme defects acquired,
e.g. autoimmune haemolytic anaemia, paroxysmal nocturnal haemoglobinuria and
drug- induced haemolysis.
Alternative classifications are possible and forms of anaemia may be allocated to
more than one category. Thus, chronic blood loss produces negative iron balance
with eventual failure of erythro- poiesis from iron deficiency. Pernicious anaemia is
an erythropoi- etic failure resulting from lack of correct digestion of vitamin 612, but
red cell precursors and mature red cells in this disease have a shortened life span.
Anaemia is demonstrated by the measurement of the amount of haemoglobin in a
known volume of blood. Haemoglobin concen- tration is reported as grams per
decilitre (g dH) or grams per litre
(g L-l). Anaemia is said to be present in an adult male if the haemoglobin
concentration is less than 13.5 g dh1 (135 g L"1) and in an adult female if less than
11.5 g dH (115 g L-1). In the first year of life, the haemoglobin concentration
decreases from 13.5-19.5 g dH at birth to 9.5 g dH at 1 month to attain levels at 12
months close to those of female adults. In pregnancy, haemoglobin levels should
not decrease below 11.5 g dH if iron and/or folate are not deficient. Modern
electronic blood counting equipment provides accu- rate red cell indices in addition
to haemoglobin estimation and these provide guidance on the type of anaemia

before resort to further investigation. It is worth noting that the commonest form of
anaemia worldwide is that caused by iron deficiency. With the notable exception of
acute blood loss, reduction in red cell mass is accompanied by an increase in
plasma volume, thus pre- serving blood volume. The mechanism for this is not clear
but it is one of the compensatory mechanisms adopted during anaemia of any
duration. Of equal importance is a shift of the oxygen dissocia- tion curve to the
right through increased synthesis of 2,3-diphos- phoglycerate (2,3-DPG) in the red
cell via the Embden-Meyerhof pathway of anaerobic glycolysis and the RapaportLuebering shunt. Increases in 2,3-DPG render the haemoglobin molecule less avid
for oxygen at any given partial pressure and improve tissue oxygenation. Two
remaining compensatory mechanisms in anaemia are an increase in cardiac stroke
volume and an increase in heart rate. In acute blood loss, red cells and plasma are
lost together, such that in the first few hours haemoglobin and haematocrit
measure- ments change little and cannot be used to estimate blood loss. Surgeons
have always attributed much importance to the haemat- ocrit, but in continued
acute bleeding the haemoglobin and haematocrit move in parallel. Haemodilution is
complete by 24-48 h if transfusion of red cells is not carried out.
GENETIC ABNORMALITIES OF
HAEMOGLOBIN
The inherited abnormalities of haemoglobin include a complex and diverse series of
genetic defects resulting from impaired pro- duction of normal globin chains
(thalassaemias) or synthesis of an abnormal haemoglobin. The thalassaemias are
characterized by absent or reduced production of the affected globin chain whilst
the other chains which make up the haemoglobin molecule are structurally normal.
In the haemoglobinopathies, the affected
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23 HAEMATOLOGY
chain, usually the |3 or a chain, has an amino acid substitution which, if it affects
the structure or function of the haemoglobin molecule as a whole, may produce
clinical effects.
(3-THALASSAEMIA
(3-Thalassaemia, in which (3-globin chain synthesis is impaired, is classified into
three clinical grades. fi-Thalassaemia ra*(thalassaemia minor) is the
heterozygous state and produces little clinical effect. There may be slight anaemia
and the condition may be mistaken for iron deficiency. In pregnancy, the
haemoglobin may decrease below the reference range. Thalassaemia intermedia, as
the name implies, is associated with more marked anaemia than thalassaemia trait
and is generally caused by double heterozygosity or homozygosity of less severe (3-

thalassaemia genes. Occasionally, patients may require transfu- sions of red cells.
Thalassaemia major (Cooley's anaemia) is the homozygous inheritance of severe (3thalassaemia genes; (3-chain production is reduced ((3+) or absent ((3), thus
impairing the synthesis of adult haemoglobin. Without blood transfusion the
condition is gener- ally fatal in the early years of childhood, and even with regular
transfusion support, patients may not live beyond their early 20s as a result of iron
overload. Long-term iron chelation therapy may prevent transfusional iron overload
and bone marrow transplanta- tion may be considered.
a-THALASSAEMIA
a-Thalassaemia is a genetically variegate disorder which ranges in severity from
fetal death in utero in the homozygous form to a mild hypochromic disorder in the
heterozygous form. Patients with three of the four a-chain genes deleted suffer HbH
disease of intermediate severity and some require transfusion with red cells.
SICKLE CELL DISEASE
More than 100 haemoglobin variants have been described, but only one has
significant global clinical impact - haemoglobin S. Ten per cent of patients of African
extraction carry the S gene. It is also seen in Italy, Greece, Arabia and the Indian
subconti- nent. Haemoglobin S has valine substituted for glutamine in position 6 of
the p-globin chain and this confers physical differ- ences on the haemoglobin
molecule with profound clinical con- sequences in homozygotes. Haemoglobin S
becomes insoluble at oxygen tensions in the venous range (5-5.5 kPa) and crystallizes, imposing a sickle cell shape on the red cell. The sickled red cell is rigid and
does not pass easily through capillaries, leading to occlusion, tissue infarction and
the pain which is characteristic of clinical episodes known as crises. Red cell survival is reduced greatly and homozygous patients invariably have anaemia (6-10 g
dh1) and jaundice. Heterozygotes are mostly asymptomatic but their red cells may
sickle when oxygen tensions are unphysiologically low. Haemoglobin S may exist in
combination with other genetic defects of haemoglobin, the most significant
combinations being HbS/f3-thalassaemia and HbSC disease. Symptoms may be
similar to those of homozy- gous HbSS although HbSC has a particular tendency to
throm- bosis and retinopathies.
Patients with haemoglobinopathies are at risk of unique peri- operative
complications and increased mortality. Haemoglobin electrophoresis should
therefore be performed in all patients of affected ethnic groups to establish the
presence or absence of haemoglobin S before anaesthesia. In an emergency, sickle
haemoglobin may be demonstrated rapidly in patients' blood using a commercial
kit, e.g. Sickledex (Ortho Diagnostics). It may be necessary to pre-transfuse
homozygotes electively or consider exchange transfusion to raise the percentage of
haemo- globin A compared with haemoglobin S, particularly for surgery involving
cardiopulmonary bypass. In all cases, it is essential to maintain good oxygenation

pre-, intra- and postoperatively, and consideration should be given to oxygen


therapy for 24 h after anaesthesia. Postoperative infarctive episodes may occur
even with the most meticulous attention to detail. Acute chest syn- drome is often
underdiagnosed, and documentation of intraop- erative temperature, hypoxia and
volume status is vital. Sickling is enhanced by low blood pH, high red cell 2,3-DPG,
stasis, dehydration and increased plasma osmolality. Avoidance of these factors is
imperative.
HAEMOSTASIS AND THROMBOSIS
NORMAL MECHANISMS
Haemostasis involves an interaction between vascular endothe- lium, platelets and
coagulation proteins to seal the point of injury of the vessel wall.
The vessel wall
Extracellular matrix proteins such as collagen in the blood vessel wall fulfil an
essential role in haemostasis by promoting platelet adhesion at the site of vessel
injury. The endothelial cell also has a powerful influence on haemostasis by virtue of
the factors that it synthesizes. These include tissue factor, von Willebrand factor
(VWF), prostacyclin, antithrombin, protein S, thrombomodulin and tissue
plasminogen activator.
Platelets
The initial response of platelets to a break in the endothelial lining is adherence to
subendothelial microfibrils and collagen through binding with VWF. Platelets then
change shape from a disc to a sphere and extend long pseudopodia to enhance
interaction between adjacent platelets. Reorganization of the internal con- stituents
forces the granules to the plasma membrane where, trig- gered by exposed
collagen and production of thrombin, they release their contents onto the surface of
the platelet. These include proteins such as VWF, factor V, (3-thromboglobulin and
platelet factor 4 from a-granules, and ADP, ATP and serotonin from dense granules.
The arachidonic acid pathway is also stimulated, resulting in thromboxane formation
that further potentiates platelet aggre- gation and release. Together with serotonin,
it also has a profound vasoconstrictive effect. ADP aids binding of fibrinogen to
platelet receptors, allowing bound platelet aggregates to form. Activated platelets
also provide a phospholipid surface for many of the reactions involved in the
coagulation pathway (Fig. 23.1).

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