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Anaesthetic management of the child with

haematological malignancy 
Louise Oduro-Dominah, MB ChB FRCA  , Liam J Brennan, BSc MBBS FRCA

Continuing Education in Anaesthesia Critical Care & Pain, Volume 13, Issue 5, 1 October 2013, Pages
158–164, https://doi.org/10.1093/bjaceaccp/mkt011
Published: 18 February 2013

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Key points
Haematological malignancy is common in children, with around 630 new cases in
the UK per year.

Younger children may require frequent anaesthetics to facilitate diagnosis and

treatment.

Children with haematological malignancies are frequently immunocompromised;

care must be taken to reduce the risk of introducing infection during perioperative
management.

Induction of general anaesthesia in children with mediastinal involvement is

potentially hazardous: careful preoperative assessment and speci c strategies for
perioperative management can reduce the risk.
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A ected children may require irradiated or cytomegalovirus-depleted blood
products, depending on their underlying condition or current treatment.
 There are 1400 new cases of childhood cancer diagnosed in the UK every year, a ecting one
in 600 children by the age of 14. Haematological malignancy accounts for 45% of these. With
the incidence of leukaemia increasing, and improving 5 yr survival (currently around 85%),
anaesthetists are increasingly likely to encounter these children, providing care during
diagnostic procedures, chemoradiotherapy, surgical treatment, and in intensive care.

Care of the immunocompromised child

Children with a haematological malignancy are frequently immunocompromised. Children

1
with leukaemia are more prone to neutropenic bacteraemia than those with solid tumours.
This may be directly related to the disease process where the bone marrow is not producing
the cells required to maintain immunocompetence. Reduced immune function may also be
due to the treatment (chemotherapy or radiotherapy), a coexisting condition, or a
combination of these factors.

Many leukaemias are associated with congenital abnormalities which have an

immunode ciency component, for example, Fanconi anaemia, neuro bromatosis, and
Noonan syndrome. Other pre-morbid factors can compromise immune function, for
example, latent infection with Epstein–Barr virus (EBV) and cytomegalovirus (CMV). The
various factors that contribute to immunocompromise are summarized in Table 1.

Table I
Immunological impacts of treatments for haematological malignancy

Type of treatment  Adverse e ect 

Chemotherapy  Di erent agents have variable e ects on immune function

High intensity causes prolonged neutropenia/lymphopenia and leads to
1
11-fold increased risk of bacteraemia  

Radiotherapy  Large fields a ecting significant amounts of bone marrow production can
a ect neutrophil synthesis 

Steroids  Decreased neutrophil migration, decreased/inhibition of chemotaxis,

lymphopenia, inhibition of phagocytosis 
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Haemopoietic stem cell Very immunocompromized; allogenic recipients more at risk than
transplantation (HSCT)  autologous ones 
 View Large

Infection control measures

Anaesthetists may use a number of strategies to reduce the risk of immunocompromised
children becoming infected. In addition to standard infection control measures such as strict
hand hygiene, it is important to minimize patient contact with known infective cases such as
chickenpox, measles, respiratory tract, and gastrointestinal infections, and also sta
members who may be unwell. Thus, placement of these children on operating lists should be
before any known infective cases.

Before anaesthetizing these children, it is important to obtain a full blood count (FBC) to
determine their neutrophil count and check that they are not signi cantly anaemic or
thrombocytopenic. Perioperatively, it is sensible to carefully consider the risk/bene t of
using regional anaesthetic techniques, especially central neuraxial block, as these may carry
an increased risk of infection and also avoid i.m. injections which can be associated with
localized abscess formation. It is also important to maintain skin integrity, take care when
instrumenting the airway, avoid rectal trauma when inserting temperature probes, and
possibly refrain from using rectally administered medication.

Care of central venous lines

Many children with a haematological malignancy have an indwelling central venous catheter
(CVC) for administration of chemotherapy, blood sampling, and hydration therapy. These
lines can be a common source of infection, and care must be taken when using them.
2
Infection of these lines can come from four sources: the skin, direct contact of the catheter
or hub by an infected source (i.e. hand/ uid), haematological seeding from another source,
or infection of the infusion uid or drug solution.

2
Factors to reduce catheter-related bloodstream infection include: education (sta , carers,
and where appropriate the child), aseptic insertion using chlorhexidine skin preparation,
disinfection of catheter hubs, removal of super uous connectors, replacement of
administration sets within 72–96 h, assessment of entrance/exit sites, use of transparent
semi-permeable dressings, and prompt replacement of damp/soiled catheter site dressings.
Some anaesthetists avoid the use of indwelling central venous lines all together and opt for
an inhalation induction to decrease the infection risk.
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When using an indwelling line for induction, there are several steps the anaesthetist should
follow to reduce infection risk. Drugs drawn up in advance should be kept in a clean tray, and
 the hubs of the syringes covered. When accessing the line, gloves should be worn and the
access port should be cleaned with a chlorhexidine containing wipe. Post-procedure and
before transfer to the recovery room, it is important to ush the line with a su cient volume
of normal saline (and document this has occurred) to ensure that there is no residual drug in
the cannula or associated connectors.

Anaesthesia for common procedures

Bone marrow biopsy/harvest

Bone marrow biopsy is used to diagnose leukaemia, and also to monitor response to
treatment. Cells obtained from aspirates are analysed using microscopic morphological
evaluation. Samples obtained from the trephine biopsy are used to evaluate the cellularity of
the marrow and to exclude marrow involvement by lymphoma.

Bone marrow harvest is performed as a treatment for leukaemia. Harvested cells can later be
re-infused into the child to re-establish cell production after high-dose chemotherapy. It
can be either autologous (cells frozen) or allogenic. The ethical issues surrounding this are
complex. Children who are deemed Gillick competent are able to consent to donate. In cases
where the donor child is unable to give consent, someone with parental responsibility can
provide consent on their behalf. Before this, however, at least in the UK, there must be
involvement with an accredited assessor/patient advocate from the Human Tissue
3
Authority.

The preferred site for obtaining bone marrow in children is the posterior superior iliac crests.
They contain the most accessible marrow, are non-weight bearing, and performing the
procedure at this site has a low potential for damaging other important structures. The child
is normally placed in the prone or lateral decubitus position. While bone marrow biopsy is a
short procedure, harvests can take between 60 and 90 min.

As part of the preoperative assessment for bone marrow biopsy, it is important to know the
underlying (or suspected) diagnosis, whether the child is undergoing active treatment and
the results of a recent FBC. Even in cases of severe thrombocytopenia, a bone marrow biopsy
can be undertaken, provided su cient local pressure is applied afterwards to achieve
haemostasis. A spontaneously breathing anaesthetic technique supplemented by local
anaesthetic in ltration of the wound with the addition of simple analgesia is usually
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acceptable. It is sensible to avoid the use of NSAIDs if the platelet count is particularly low.
 For autologous bone marrow harvest, in addition to the preoperative assessment above, it is
important to have obtained a group-and-save sample. As it is a prolonged procedure in the
prone position, most anaesthetists will intubate and ventilate the child. Up to 10% of the
patients' bone marrow may be harvested, potentially resulting in anaemia and
hypovolaemia. If this is the case, uid resuscitation will be required. However, blood
transfusion should be delayed until after the procedure as the transfused red blood cells can
contaminate the harvested marrow.

Long-term venous access

Many children with malignancy have long-term venous access for regular blood tests and
administration of chemotherapy. The requesting oncologist makes the choice of line.
Subcutaneously placed port devices are more convenient for short intermittent use, while
tunnelled lines (e.g. Hickman lines) are preferable if the child needs venous access very
frequently. These lines are at risk of infection, thrombosis, leakage, and dislodgement, so
children often require more than one line during a prolonged course of treatment. Hence,
central veins should be preserved and not used indiscriminately for routine perioperative
access.

The preoperative state of the child needs to be assessed thoroughly before insertion.
Coagulation status and platelet count should also be reviewed. The vascular anatomy and
patency of vessels should be checked by ultrasound imaging before the procedure,
particularly if the child has had multiple lines in the past. I.V. or gaseous induction may be
used, depending on the preference of the child, parents, and the anaesthetist. In smaller
children, a tracheal tube is often preferred due to both the position of the neck and the
proximity of the operator to the airway. Laryngeal masks may be suitable in the older child,
but the in ated cu may distort the vascular anatomy and so are not favoured by some
operating teams for that reason. The use of paracetamol and local anaesthetic in ltration at
the insertion point reduces postoperative pain, although a small dose of short-acting opioid
perioperatively is usually required.

Chemotherapy and radiotherapy

Chemotherapy regimens for children normally use a combination of at least two cytotoxic
drugs. Many children with leukaemia and non-Hodgkin lymphoma (NHL) require intrathecal
chemotherapy and for most cases, a general anaesthetic is required. The three most
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commonly used drugs are cytarabine, methotrexate in conjunction with hydrocortisone.
These have numerous side-e ects ranging from nausea and vomiting through to organ
toxicity and carry a risk of secondary malignancy. It is important to note that anaesthetists
 should never be involved in performing the lumbar puncture or the administration of
intrathecal chemotherapy. Before 2001, there were at least 14 cases of doctors, including
anaesthetists, incorrectly administering the i.v. chemotherapy drug vincristine via the
intrathecal route, resulting in death or paralysis of the patients. Subsequent to this, the UK
Department of Health issued guidance stating that only personnel who have received the
4
correct training and are on a register may administer intrathecal chemotherapy.

Before commencing the induction, it is important to establish whether the oncologist or

nursing sta wish to carry out any other procedures such as blood tests, bone marrow
biopsy, placement of a nasogastric tube, or dressing changes at the same time. Many of these
children already have long-term venous access, so that i.v. induction is preferable. When
using indwelling lines for induction, it is important to use an aseptic technique.

With an experienced operator, performing a lumbar puncture and administration of

intrathecal chemotherapy is a brief procedure. While anaesthesia can be provided via a mask
using an inhalation spontaneously breathing technique, an alternative is to use propofol and
remifentanil. The technique used at Great Ormond Street, and described by Glaisyer and
5
Sury, has a faster recovery time than sevo urane and is reported to be preferred by parents.

Radiotherapy is used as both a curative and palliative measure for children with
haematological malignancy. Curative regimes use a low daily dose of radiotherapy over 5–6
weeks compared with the single high-dose therapy used for palliation. Although an
important part of the treatment strategy for many children with haematological malignancy,
radiotherapy does have its drawbacks. Tumour lysis (see below) may be provoked and there is
the long-term risk of a secondary malignancy developing. It is imperative that children
receiving radiotherapy remain completely immobile to ensure correct localization of the
treatment. Therefore, a general anaesthetic is often required, especially in the younger child.
Older children may tolerate the treatment awake with suitable preparation from the play
therapist. For children receiving radiotherapy to the head and neck, custom-made plastic
shells for the patient to lie in are produced before treatment. Similar moulds can be made for
6
other parts of the body. The treatment itself usually takes no more than 10–15 min.

For anaesthetists, working in the radiotherapy suite is often an unfamiliar and remote
environment. Access to piped gases and monitoring is often limited, as is the ready
availability of emergency assistance. The child is remotely monitored during the procedure to
prevent exposure of the anaesthetic team to radiation with conventional minimal monitoring
supplemented by camera surveillance.
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The decision to sedate or fully anaesthetize the child is a decision for the anaesthetist. Since
these children usually go home between therapies, it is important to utilize methods
 associated with minimal hangover e ects. Most anaesthetists use a laryngeal mask airway to
reduce the risk of supraglottic swelling associated with repeated daily intubations. However,
if the child needs to be in the prone position for their radiotherapy, a tracheal tube is usually
preferred for increased airway security. Antiemetics are recommended as nausea is a well-
recognized side-e ect of radiotherapy; however, due to the risk of tumour lysis syndrome
(TLS) (see below), dexamethasone should be avoided. To minimize stress for the child and
parents, radiotherapy lists tend to follow a set structure and are carried out in the morning to
allow the child to recover, eat, and go home as soon as possible.

7
Oral mucositis is a common complication of chemoradiotherapy, occurring in 40–70% of
children receiving treatment. It is manifested by in ammation, ulceration, and in severe
cases, there may be bleeding of the mucous membranes. It is important to establish the
presence of mucositis before anaesthesia as airway management may be di cult and
instrumentation may lead to bleeding which may be troublesome in the presence of a co-
existing coagulopathy.

The child with an anterior mediastinal mass

They are commonly seen in children with NHL, Hodgkin's, and acute lymphoblastic
leukaemia (ALL) of the T-cell variety. Almost 50% of children with Hodgkin's disease have a
mediastinal mass at the time of diagnosis. Although tumours may also present in the
posterior compartment, it is the anterior mediastinal masses that cause the most signi cant
problems in the perioperative period.

Masses located in the anterior and superior compartment present the greatest risk due to the
anatomical proximity to the heart, major vessels (in particular the superior vena cava), and
airways. Signs and symptoms can be divided into respiratory and cardiovascular (Table 2),
8
although nearly 50% of masses are asymptomatic at presentation.

Table 2
Symptoms and signs of mediastinal masses

  Cardiovascular  Respiratory 

Symptoms  Chest pain  Wheeze 

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Syncope  Cough 

Nausea  Breathlessness 
   Cardiovascular  Respiratory 

Headache   

Signs  Superior vena cava obstruction  Stridor 

Cyanosis  Orthopnoea 

Facial swelling   

Dilated upper body veins   

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Respiratory symptoms are more common with anterior mediastinal masses. The heart and
great vessels may be involved with the mass, potentially leading to pericardial e usion and
even tamponade. The aorta is relatively spared due to its anterior position and high
intravascular pressure.

The severity of symptoms does not necessarily correlate well with the degree of compression.
It is known that as a useful guide, children with >50% compression of the trachea are at risk
9
of complications during anaesthesia. However, signi cant carinal and major bronchial
compromise may also occur without evidence of tracheal compression. It is important to note
that airway compression may only be evident in the supine position, and that syncope from
compression of the great vessels may only manifest during a Valsalva manoeuvre.

Ideally, in the child presenting with severe symptoms or superior vena cava obstruction, it is
preferable to commence tumour shrinkage therapy before anaesthesia. The alternatives are
high-dose steroid therapy (dexamethasone and prednisolone most commonly used) and
radiotherapy. Both treatments may cause di culty as they may compromise obtaining a
complete histological diagnosis, although limiting the period of pre-diagnostic treatment
10
will usually limit the extent of this problem.

In children with severe symptoms or adverse radiological features, consideration should be

given to obtaining a diagnosis without recourse to general anaesthesia. This may be possible
from peripheral blood lm analyses or obtaining a tissue sample using local anaesthesia (e.g.
bone marrow aspirate, pleural uid, or even peripheral lymph node biopsy). However, such
techniques are not without risk particularly if supplemented by i.v. sedation.

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Before Content
anaesthetizing these children, it is important to undertake a full history and
examination with a low threshold of suspicion for compressive symptoms. In addition to
blood tests, diagnostic imaging is important in establishing the extent of the mass. These
 include chest X-ray, CT/MRI, echocardiography, and possibly pulmonary function tests (in
the older child) to look for dynamic airway compression. These children may require a
exible breoptic bronchoscopy to detect evidence of tracheomalacia. Performing this
procedure under local anaesthetic is preferable, as the complication rate for these children
10
undergoing general anaesthesia may be as high as 19.6%.

The main aim of anaesthesia is to minimize complications. Strategies to reduce the risk of
airway compromise, particularly in severe cases, include the use of anti-sialagogue agents,
gas induction in either the head-up or lateral position, maintenance of spontaneous
ventilation, and awake extubation. Initiation of positive pressure ventilation can cause
cardiorespiratory collapse due to the loss of negative intrapleural pressure, resulting in a
drastic reduction in venous return.

If there is airway compromise or reduced cardiac output, moving the child into the lateral or
prone position may improve the situation and aid oxygenation. It is important that breoptic
equipment is available, and given that the obstruction may be in the distal airways, the use of
a rigid bronchoscope may need to be considered.

Tumour lysis syndrome

TLS is the manifestation of the metabolic derangement caused by tumour breakdown,

leading to the rapid release of intracellular cations, anions, nucleic acid metabolites, and
proteins into the bloodstream. It may occur spontaneously or after the commencement of
cytotoxic therapy, steroid treatment, or radiotherapy. It is more common when the burden of
tumour is high, with tumours that are highly sensitive to treatments and those that
proliferate rapidly. It can occur with all forms of haematological malignancy but is most
11
common in Burkitt's lymphoma, with a reported incidence of up to 42%.

Biochemically, hyperuricaemia occurs due to purine breakdown; hyperphosphataemia

because malignant cells contain up to four times the level of organic and inorganic
phosphate; hyperkalaemia secondary to cell lysis and hypocalcaemia as a result of the
hyperphosphataemia and the precipitation of calcium phosphate in the tissues.

Uric acid is poorly soluble; however, under normal physiological conditions, it is 99% ionized
and therefore excreted. In TLS, as the uric acid concentration increases, it becomes less
ionized and crystals begin to precipitate in the renal tubules leading to renal failure. This in
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combination with the hyperphosphataemia leads to precipitation of calcium phosphate in the
tubules increasing the risk of renal failure. The clinical symptoms and signs normally
manifest 48–72 h after the commencement of treatment and are summarized in Table 3.
 Table 3
Symptoms and signs of TLS

Symptoms  Nausea and vomiting 

Lethargy 

Headache 

Signs  Oedema 

Heart failure 

Arrhythmias 

Seizures 

Tetany 

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The best way to minimize the onset and severity of the syndrome is to delay the initiation of
chemotherapy until prophylactic measures are possible and avoid trigger factors such as
surgical manipulation of the tumour and the use of dexamethasone. The rst published
12
report of TLS developing during a general anaesthetic was in 2001. Dexamethasone is used
in the treatment of haematological malignancy as it induces apoptosis of B cells, ALL cells,
and monoclonal lymphoma cells. The higher the dose of steroid, the more intense the
−2
response. Treatment doses of dexamethasone are in the order of 3 mg m per 12 h, which
−1 13
approximates to a 0.1 mg kg dose, the same as the antiemetic dose.

The biochemical disturbances associated with TLS may occur rapidly, especially
hyperkalaemia, thus vigilance and prompt management are required. The mainstay of
treatment is aggressive hyperhydration, diuresis, and drugs to control the hyperuricaemia.
−2 −1
To achieve e ective hydration, a child requires 3 litre m day of uid aiming for a diuresis
−2 −1 11
in the order of ≥100 ml m h . In children who are profoundly hypoalbuminaemic or have
poor cardiac function, this volume of uid may lead to pulmonary oedema. Hence,
hyperhydration therapy may need to be initiated and monitored in a high-dependency
environment. Hyperphosphataemia can be treated with aluminium hydroxide, while
hyperuricaemia can be controlled with either allopurinol or rasburicase. Urinary
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alkalinization with bicarbonate can be considered; however, this can lead to calcium
phosphate deposition in the presence of hyperphosphataemia and urinary xanthine crystals
if there is concomitant treatment with allopurinol. Urgent transfer to paediatric intensive
 care unit (PICU) for renal replacement therapy may be required for children who have
hyperkalaemia or worsening renal failure which does not respond to rst-line treatment.

Blood product administration

As a consequence of their condition, many children with haematological malignancy will

require blood products during their treatment. It is therefore important to know which
products to administer and when as shown in Tables 4 and 5.

Table 4
Indications for blood products

16
Blood product  Recommended administration  

CMV deplete Immunodeficient children 

blood 
Post-stem cell transplant 

Irradiated blood  Children with Hodgkins disease during treatment and for life 

During treatment with purine analogues and for at least 2 yr a erwards 

Children receiving HLA-matched platelets or donations from first- or second-degree

relatives 

Platelets  Absolute count <10 

<20 at risk of bleeding, DIC, severe mucositis 

Count 20–40 for CVC insertion or lumbar puncture 

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Table 5
Doses of blood products

Blood product  Dose 

−1 −1
Packed cells  4 ml kg will raise Hb by 1 g dl (desired Hb−actual Hb)×weight (kg)×3 
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−1
Platelets  Children <15 kg 10–20 ml kg ; children >15 kg one apheresis unit (single
donor) 
 Blood product  Dose 

−1
Fresh-frozen plasma 10–20 ml kg  
(FFP) 
−1
Cryoprecipitate  5 ml kg  

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Common PICU problems

Respiratory failure
Children with haematological malignancy can develop respiratory failure as a result of
infection or from the side-e ects of treatment. Cytotoxic agents such as bleomycin are
associated with pulmonary injuries including lung brosis, pneumonitis, and predisposition
to oxygen-induced lung injury, while lung damage secondary to radiotherapy is related to
the total dose of radiation received.

Pleural e usions, pulmonary in ltrates, and infective pneumonitis are frequently the cause
of respiratory failure. Pleural e usions may occur due to in ammation from infective causes,
from cancer cells, or from obstruction of lymphatic drainage as the result of a mediastinal
masses.

Infection is commonly caused by gram-negative bacteria, although immunocompromised

children are also at risk from parasites, fungi, and viruses such as CMV. These children are
also at risk from pneumonitis occurring from graft vs host disease (GVHD) either as a result
of receiving non-irradiated blood or post-bone marrow transplant. This can make diagnosis
di cult and occasionally a lung biopsy may be required to make the diagnosis.

Clinical symptoms and appropriate investigations are the same as those for respiratory
failure due to other causes. Management of these children is largely supportive and aimed at
treating the underlying cause. The mainstay includes broad-spectrum antibiotics,
supplemental oxygen therapy, and ventilation. Although overall treatment is similar to that
for any child with other causes of respiratory failure, there are important di erences. These
children are at risk of enhanced oxygen toxicity due to previous exposure to cytotoxic
14
agents. Thrombocytopenia means that great care must be taken when instrumenting the
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airway. They are at greater risk of ventilator-acquired pneumonia. Ongoing chemotherapy
may need to be stopped while the child recovers.
 Cardiovascular failure
Children may present with cardiovascular compromise secondary to tumour compression,
particularly in the mediastinum (see above), sepsis, and the e ects of chemotherapeutic
agents such as anthracyclines which have known cardiotoxic potential (e.g. doxorubicin,
daunorubicin). The cardiac toxicity of these agents is related to the cumulative dose
administered; monitoring of patients receiving these agents is recommended with regular
echocardiography. Slower infusion rates, liposomal formulations of the drug, and the use of
cardioprotective agents such as dexrazoxane may all minimize the potential for cardiac
impairment with these drugs.

Sepsis
Sepsis remains a leading cause of morbidity and mortality in children with haematological
malignancy. As previously stated, these children are at risk of immunocompromise due to
their underlying condition and the treatment used. Other factors which can increase the risk
of infection are summarized in Table 6.

Table 6
Factors that predispose to immunocompromise. GIT, gastrointestinal tract

9 −1
Neutropenia  Duration and absolute count <0.5×10 litre for ≥1 week increases risk 

Lymphopenia  Prolonged lymphopenia increases susceptibility to nosocomial infections 

Malnutrition  Impairs phagocytes and depresses lymphocyte function

Nutritional imbalance due to decreased appetite and vomiting 

Antibiotics  Broad-spectrum use a ects GIT lining

Prolonged use can cause colonization with resistant organisms 

Damage to physical barriers  Mucositis and GIT damage secondary to chemotherapy

Presence of indwelling lines and catheters 
15
Renal/hepatic dysfunction  Hepatic failure increases risk of infection with gram-negative bacilli  

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Treatment
Skip is with empiric antibiotics while awaiting microbiological analyses. Most
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hospitals will initiate treatment using a broad-spectrum penicillin and an aminoglycoside
with additional cover for gram-positive organisms if the child has an indwelling CVC. If the
 child presents with septic shock, then uid resuscitation regimes should be guided by cardiac
output monitoring, since many cytotoxic agents cause cardiomyopathy and impaired cardiac
function.

Hyperviscosity
9 −1
Hyperleucocytosis is de ned as a white cell count >100×10 litre and tends to occur at
presentation or during a relapse. There is a high risk of morbidity and mortality at counts
9 −1
>300×10 litre . The point when symptoms develop depends on the patient and the
underlying disease. In acute myeloid leukaemia, it occurs at a lower white cell count due to
the larger size of the myeloblasts.

The high white count causes increased blood viscosity, leading to endothelial damage,
capillary obstruction, micro-infarction, and organ dysfunction. The organs most at risk are
the brain and the lungs. In the brain, this manifests as cerebral haemorrhage or infarction,
while intravascular leucostasis in the pulmonary capillaries causes alveoli damage, hypoxia,
and respiratory failure. The blood supply to the kidneys is also at risk, and a syndrome
similar to TLS may manifest.

Early commencement of cyto-reductive chemotherapy is the basis of treatment. In severe

cases, leucopheresis or exchange transfusion may be required. Before therapy, hyper-
hydration and treatments to reduce the risk of TLS must be started. Many of these children
have a compensatory anaemia o setting the high white count. It is important not to
transfuse these children before treatment, as this will increase viscosity and worsen the
problem. Platelet transfusion does not signi cantly increase viscosity and may be required to
9 −1
reduce the risk of cerebral haemorrhage if the count is <20×10 litre .

Post-haemopoietic stem cell transplant

Haemopoietic stem cell transplant (HSCT), rst used successfully in 1968, is now used to
treat a range of childhood conditions including acute leukaemias and NHL. The two main
forms are autologous, where the patient's own stem cells are used, and allogenic, which uses
donor cells. Mortality post-HSCT remains high, and is due to a combination of sepsis,
pulmonary complications, and GVHD. Therefore, many of these children require PICU
management.

The pulmonary complications of HSCT are multifactoral and occur in up to 15% of patients.
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There may be direct damage and scarring of the pulmonary interstitium as a consequence of
the pre-transplant conditioning regimes. Post-transplant, these children are at risk of
 pulmonary oedema caused by endothelial damage from chemotherapy, in addition to a wide
variety of infections. Critical care management of these problems is similar to those
described above. However, survival rates in children ventilated for HSCT-related pulmonary
complications are <20%.

GVHD is a common complication post-HSCT, which carries signi cant risk of morbidity and
mortality. It is subdivided into acute and chronic forms. The acute form occurs within the
rst 100 days post-transplant, although most commonly in the rst 5 weeks. Acute GVHD
occurs in up to 80% of those receiving an allogenic transplant and arises due to donor
cytogenic T-cells recognizing host cells as foreign and rejecting them.

The disease predominantly a ects the skin, gastrointestinal tract, and the liver. The severity
of involvement is graded. Mild GVHD is manifested by a maculopapular rash, nausea,
diarrhoea, and mild derangement of liver function. In severe disease, there can be skin
desquamation, ileus, and fulminant hepatic failure.

The mainstay of critical care treatment is supportive, concentrating on scrupulous skin care,
preventing superimposed infection, and maintaining appropriate uid balance in the face of
high GI losses. Speci c therapy depends on the grade of disease and includes high-dose i.v.
methylprednisolone, addition of further immunosuppressive therapy such as mycophenolate
mofetil (MMF), or the use of serotherapy (e.g. alemtuzumab).

Further reading

A more comprehensive discussion of the issues surrounding the anaesthetic and critical care
management of children with haematological malignant disease is beyond the scope of this
article. Further useful information may be found in the articles listed below:

1. Abrams AN, Hazen EP, Penson RT. Psychosocial issues in adolescents with cancer. Cancer
Treat Rev 2007; 33(7): 622–30

2. Latham GJ, Greenberg RS. Anesthetic considerations for the pediatric oncology patient.
Paediatr Anaesth 2010; 20: 295–304, 20: 396–420, 20: 479–48 (series).

Declaration of interest
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None declared.
 Acknowledgement

We are grateful to Dr Henrik Hack, consultant paediatric anaesthetist, Manchester Children's

Hospital, UK, for his helpful comments.

References

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 Subject: Management of Intercurrent Disease
Issue Section: Articles

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