SAQ Answers10th June 2015

CGH Regional training

SAQ 1This 65 year old lady was sent from radiology after an x-ray requested by her GP showed this. She has had recent weight loss and is an ex-smoker.

1. Describe the changes on this x-ray (1)

Right upper lobe (1/2) collapse/consolidation (1/2)

2. What are the top two differential diagnoses? (1)

Tumour, infection,

3. Give 4 extra-pulmonary effects of an apical lung mass. (4)i) Horner syndrome: ptosis, miosis, enophtalmos and anhydrosis.ii) Brachial plexus infiltration (Thoracic outlet syndrome)iii) Hoarseness of the voice (recurrent laryngeal nerve)iv) Stridor (tracheal compression)v) Paraneoplastic phenomenonvi) Hypercalcaemia vii) SVCO

4. The patient reports that in the last few days she has experienced swelling to the face and arms. You decide that Superior Vena Cava Obstruction is the most likely cause. Other than facial and upper limb swelling name 4 other symptoms or signs of SVCO (4)

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This is obstruction to the superior vena cava (SVC) blood flow by external compression, thrombosis or direct invasion of the SVC. It may present acutely or more insidiously as chronic dyspnoea.

Compression causes a reduction in blood flow from the head, neck and upper extremities to the heart. Low intravascular pressure in the SVC can also permit thrombus formation.

This condition may be the first presentation of malignancy or can occur in those with known malignancy. The most common cause will be carcinoma of the lung 65 to 80%, lymphoma 2 to 10%, other cancers 3 to 13%. Benign causes are rare.

Symptoms are those of venous hypertension Breathlessness Visual changes Dizziness Headache – worse on stooping Signs include Conjunctival and peri-orbital

oedema

Papilloedema – late Dilated neck veins – non-pulsatile Dilated collateral veins – arms and

anterior chest wall Stridor Cyanosis Increased respiratory rate

5. A large apical tumour causing compression of the SVC is seen on CT. Name 3 steps in the initial management of this lady

i) Oxygen / reduce tight clothingii) Consider corticosteroids (Dexamethasone 8-16mg)iii) Oncology input – consideration stent, chemo or radiotherapyiv) Respiratory or interventional radiology input – consideration tissue biopsyv) Discussion with patient / family regarding CT findings.

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Treatment is dependent on the cause of the obstruction, the severity of the symptoms and the patient’s prognosis. While the diagnosis of SVCO is often made on clinical grounds in patients with a history of thoracic malignancy, a chest X ray and CT scan may confirm the diagnosis and inform treatment.

Current management guidelines stress the importance of accurate histologic diagnosis prior to starting therapy and the upfront use of endovenous stents in severely symptomatic patients to provide more rapid symptom relief than can be achieved using RT.

SAQ 2A 72 year old gentleman attends the emergency department. He has experienced recent weight loss and has severe right hip and groin pain. This is his pelvix x-ray

1. What does it show (1)

Lytic lesions (multiple) including Right subtrochanteric region

He has blood tests which reveal the following results Na 131 K 4.5 Urea 7.0 Creatinine 95 Corrected Calcium 3.89

2. Given the above biochemical profile name 6 other symptoms and signs the patient may display (6)

CNS and PNS Confusion/delerium Coma Hypotonia & Hyporeflexia Seizures

Renal findings Renal stones Dehydration Renal failure Polyuria

GI findings Constipation and fecal impaction Anorexia Thirst Nausea &/or vomiting

Cardiac findings Arrhythmias Hypotension

Signs related to underlying malignancy

3. Name 2 ECG changes might you expect (1) Shortened QT interval Prolonged PR

Wide QRS Osbourne (or J) wave

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4. Name 3 treatments which might be started urgently? (3) Analgesia (Pain can be precipitated or exacerbated by hypercalcaemia) IV fluids Frusemide (consider – in textbooks so maybe in exam) Calcitonin Haemodialysis Biphosphonate

5. Give 2

causes of hypercalcaemia other than malignancy. (2)

Hyperparathyroidism (primary and tertiary)

Milk-alkali syndrome Sarcoidosis Endocrine (Addison’s,

Phaeochromocytoma,

Hyperthyroidism) Dehydration Respiratory (Sarcoidosis, TB) Drugs (Lithium, thiazides, Vitamin

D) Fictitious - Prolonged cuff time

The diagnosis and management of hypercalcaemia http://www.bmj.com/content/350/bmj.h2723

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Hypercalcaemia is a raised level of corrected calcium in the blood. It is the commonest life-threatening metabolic disorder in cancer patients, most frequently occurring in myeloma, breast, renal, lung and thyroid cancers however, 20% of patients with hypercalcaemia do not have bone metastases.

disodium pamidronate

Intravenous (IV) dose 30 to 90mg

Onset of effect <3 days

Maximum effect 5 to 7 days

Duration of effect 2.5 weeks

SAQ 3This 72 yr old gentleman presents with non–traumatic back pain. The pain has been getting worse for nearly a month.

1. Name 6 symptoms or elements from the history which indicate the need for back imaging (3)

Weight loss Night sweats History of cancer Weakness lower limbs Altered sensation in the lower

limbs

Bowel or bladder symptoms Night pain Thoracic pain Young <20 or older >55 patient Pyrexia

Other sensible suggestion (1/2 each)http://www.bmj.com/content/347/bmj.f3148

This is his x-ray

2. What does it show (1)

Wedge compression fracture L2 and L4

He has been suffering from recent night sweats and weight loss. As well as the pain in his lower back, he also has thoracic pain and has struggled to walk for the last day. Examination reveals normal power but a slightly unsteady gait.

3. What is the most likely cause for his inability to walk?

Metastatic spinal cord compression (1)

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Malignant spinal cord compression (MSCC) occurs when the dural sac and its contents are compressed at the level of the cord or cauda equina. This may be as a result of direct pressure, vertebral collapse or instability caused by metastatic spread or by direct extension of malignancy. It affects about 5 to 10% of patients with cancer. Myeloma and lung, breast and prostate cancers are the commonest malignancies involved, but MSCC should be considered in any malignancy especially with bone involvement.

Cord compression can be the initial presentation of cancer. One in 5 patients presenting with MSCC are not previously known to have malignancy.

Late diagnosis is common causing permanent loss of function and significant morbidity.

A rapid assessment, investigation and treatment may prevent or limit irreversible neurological damage.

4. Name 4 elements of management which should be instigated in the emergency department (4)

MRI whole spine (within 24hrs) Referral to oncology specialist - regional metastatic spinal cord compression

coordinator Discussion with patient regarding diagnosis Analgesia Bed rest with ‘spinal care’ High dose dexamethasone

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Assessment

Spinal cord compression Consider cord compression in any patient with cancer. Thoracic cord compression is most common but any part of the spine or multiple

sites can be affected. Sites of pain and level of compression do not always correlate; X-rays and bone

scans can be misleading. A full neurological examination should be done but may be normal initially. Magnetic resonance imaging (MRI) of the whole spine is the correct investigation if

MSCC is suspected.

Key signs and symptoms New, progressively severe back pain (particularly thoracic). New spinal nerve root pain (burning, shooting, numbness); may radiate down

anterior or posterior thigh (like sciatica), or like a band around the chest or abdomen.

Coughing, straining or lying flat may aggravate pain. New difficulty walking or climbing stairs; reduced power (motor weakness), Sensory impairment or altered sensation in limbs. Bowel or bladder disturbance; loss of sphincter control is a late sign with a

poor prognosis.

Note: normal neurological examination does not exclude evolving spinal cord compression

Cauda equina syndrome. Compression of lumbosacral nerve roots below the level of the cord itself results in a different clinical picture

Metastatic spinal cord compression

BMJ 2011; 342 doi: http://dx.doi.org/10.1136/bmj.d2402 (Published 27 April 2011)Cite this as: BMJ 2011;342:d2402

Nasir A Quraishi, consultant spine surgeon, Claire Esler, consultant oncologist

Metastatic spinal cord compression is defined radiographically as an epidural metastatic lesion causing true displacement of the spinal cord from its normal position in the spinal canal.1 It is an important source of morbidity (including paralysis and bowel and bladder disorders) in patients with systemic cancer.Case scenarioA 58 year old woman presents to her general practitioner with a two month history of middle to low back pain. She has a history of breast carcinoma, diagnosed eight years previously and which was treated with a mastectomy and chemotherapy. She also describes pins and needles in both legs for the past three days. On examination, she has tenderness over the area of the T11 and T12 vertebrae and reduced power distally (T4 and T5) but intact bowel and bladder function. She is referred urgently to the metastatic cord compression coordinator, who organises magnetic resonance imaging, which shows cord compression by a mass at the T11 and T12 levels, thought to be caused by metastases.How common is metastatic spinal cord compression?

o Skeletal system metastases are the third most common metastases, after those of the pulmonary and hepatic systems2

o Within the skeletal system, the spinal column is the most common site of metastases2

o Metastatic cord compression is estimated to occur in 5-10% of patients with cancer (most commonly those with breast, prostate, and lung cancers) and in about 40% of patients who have pre-existing, non-spinal bone metastases3

o Symptomatic metastatic spinal disease is expected to become more prevalent as survival rates for many common cancers improve

Why is metastatic spinal cord compression missed?Low back pain is one of the most common complaints in primary care, with most cases being benign, self limiting, and not needing a specific diagnosis. The challenge in primary care is therefore to identify those cases where low back pain is caused by a serious spinal disease, such as malignancy. Low back pain may be the first presentation of malignancy, and about 23% of patients with spinal metastases are thought to have had no previous diagnosis of cancer.4In a prospective observational study of 319 patients with metastatic cord compression, a median of two months passed from the onset of pain as reported to their primary care providers until the diagnosis of metastatic cord compression.5 Therefore, new onset back or neck pain in a patient with known cancer must be considered to be spinal metastatic disease until proved otherwise.

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Why does it matter?Although the rate of clinical progression is variable, patients with motor dysfunction inevitably progress to complete paralysis in the absence of intervention.6 Almost half of patients cannot walk by the time of diagnosis.7 However, neurological status at the time of diagnosis, particularly motor function, has been shown to correlate with prognosis from metastatic cord compression,8 thus reinforcing the concept that diagnosis before the development of a neurological deficit is of paramount importance. Furthermore, treatment before paralysis is clinically and cost effective.9

How is it diagnosed?

Clinical featuresAll segments of the spine can be affected by metastatic cord compression, but the

thoracic spine is the most commonly affected site (about 70% of cases), followed by

the lumbar spine (20%), cervical spine, and sacrum.6 As thoracic pain is less common in the general population than pain originating from the mobile cervical and lumbar regions, pain in the thorax should increase suspicion of the likelihood of cancer.10

Indeed pain is the most common presenting symptom of spinal metastases, occurring in 83-95% of cases.7 Three classic pain syndromes affect patients with spinal metastases: (a) local pain, with pain at rest (resulting from periosteal stretching from tumour growth and/or local inflammatory processes); (b) mechanical pain, with pain on movement and improved by rest (owing to instability); and (c) radicular pain (owing to irritation of a nerve root).7 Patients will often present with a combination of these, and they may have both myelopathic (with long tract signs such as upper motor neurone signs) and radicular abnormalities. They may have lower extremity weakness and hyper-reflexia below the level of compression (hyporeflexia if the cauda equina is compressed).

Sensory changes such as paraesthesia or anaesthesia typically occur in correlation

with motor weakness. Patients may therefore complain of sensory abnormalities in the

same dermatomal distribution as their motor dysfunction, and patients with

myelopathy may describe a sensory change across the chest or abdomen. Patients may

also have some degree of dysfunction of the bladder, bowel, and sexual organs as a

result of metastatic cord compression. Of these autonomic findings (present in 40-

64% of patients with metastatic cord compression),11 bladder dysfunction is the most common and often correlates with the degree of motor dysfunction.10 Sensory and autonomic symptoms and signs present late in these patients, and clinicians must therefore have a low suspicion threshold if patients with known malignancy have back pain. In the United Kingdom the guidelines from the National Institute for Health and Clinical Excellence (NICE) recommend education of patients at risk of developing

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metastatic spinal cord compression so that they know what symptoms to look out for.12

InvestigationsA general practitioner who suspects a patient of having metastatic spinal cord compression must immediately contact a specialist spine or oncology team for consideration of urgent imaging and further management. The NICE guidelines recommend that centres treating patients with this condition have a coordinator who advises clinicians, coordinates care, and organises urgent magnetic resonance imaging (fig 1⇓ and fig 2⇓).12 Such imaging remains the optimal imaging modality for assessing spinal metastatic disease (sensitivity 44-100%, specificity 90-93%).13 14

Fig 1 T2 weighted sagittal magnetic resonance scan showing metastatic spinal cord compression by a mass at T7-T8 (arrow)

Fig 2 Axial magnetic resonance scan at the T7-T8 level showing extensive tumour infiltration (arrow) with almost complete compression of the spinal cord

How is it managed?Metastatic spinal cord compression is an oncological emergency and, once it has been radiologically confirmed, definitive treatment should ideally start within 24 hours of diagnosis. Patients may have considerable pain and should receive analgesia in

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accordance with the World Health Organization’s “pain ladder”. NICE guidelines showed, on the basis of a systematic review of low quality randomised controlled trials and observational studies, that corticosteroids (dexamethasone 16 mg daily with gastric protection) may result in rapid improvement of neurological function.12 15 16

Primary treatment depends on a patient’s performance status, prognosis, preference, and tumour histological type. In very frail, terminally ill patients, active treatment may not be appropriate. Most patients are not suitable for surgery and should receive urgent external beam radiotherapy, although systematic reviews give no clear consensus on the best radiotherapy dose and fractionation.17 Patients with paraplegia are unlikely to regain any function, and treatment is mainly intended to help with pain.On the basis of a systematic review from the NICE guidelines of moderate to low quality evidence from retrospective studies,18 19 20 21 one prospective non-comparative study,22 one randomised controlled study,23 and an indirect comparative meta-analysis,24 surgery may provide better patient outcomes (including pain relief, a better chance of neurological recovery, and maintenance of ambulation) than radiotherapy in carefully selected patients.12 NICE therefore recommends surgery (decompression and stabilisation) plus radiotherapy for patients who are fit enough for surgery, have a prognosis of at least three months, and have:

o Spinal cord compression and have not had paraplegia for more than 48 hours, or

o An unstable spine, oro Deteriorating neurological function, oro Pain despite previous radiotherapy.12

Chemotherapy may occasionally be used as a primary treatment for metastatic cord compression that results from chemosensitive disease such as small cell lung cancer and lymphomas. Whatever treatment a patient receives, ongoing multidisciplinary care is crucial, attending to the patient’s medical, social, and psychological needs. Although rehabilitation is important for some patients, palliative care is crucial, as only about a fifth of patients with metastatic cord compression will survive for more than one year.12

Key pointso New onset back or neck pain in a patient with known cancer must be

considered to be spinal metastatic disease until proved otherwiseo If metastatic cord compression is suspected, urgent specialist referral is

critical as early diagnosis and treatment improves quality of life and functional outcome, such as the prevention of paraplegia

o Magnetic resonance imaging is the optimal imaging modality for assessing spinal metastatic disease

o Initial treatment includes corticosteroid use, with urgent definitive treatment comprising surgery or radiotherapy

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SAQ 4A 45yr old lady attends the emergency department with a nosebleed. It is resistant to initial management requires the placement of a nasal tampon to stop. Bloods are taken and show the following:

Hb 12.1WCC 7.3

Platelets 11

1. Her platelets are low. Name 4 ways, other than nosebleed, in which a patient with low platelets may present to the emergency department? (2)

spontaneous bruising mucous membrane bleeding heavy menstruation heavy post-partum bleeding retinal or subconjunctinal haemorrhage gastrointestinal bleeding intracranial bleeding

2. This is a new finding from her last blood test 2 years ago. List 6 causes of thrombocytopaenia. (3) Pregnancy HELLP syndrome Chronic liver disease Splenomegaly Immune thrombocytopenia (ITP) Infection (HIV, viral, bacterial,

parasites) Disseminated Intravascular

Coagulopathy (DIC) Drug-induced Heparin

Malignancy TTP – HUS Chemotherapy SLE Antiphospholipid syndrome Bone marrow disorder Vitamin deficiency Alcohol Pseudo-thrombocytopenia

(analysis error)

½ point per answer

Pregnancy – Most pregnant women have normal platelet counts. Approximately 5 percent develop incidental thrombocytopenia during pregnancy, also termed gestational thrombocytopenia. Gestational thrombocytopenia is mild, asymptomatic, occurs during late gestation, and resolves spontaneously after delivery. There is no association with fetal thrombocytopenia. Any change from routine obstetrical care is discouraged.

Severe thrombocytopenia, or thrombocytopenia accompanied by other findings during pregnancy (eg, renal insufficiency, hypertension, microangiopathic hemolytic anemia) should prompt an evaluation for more serious disorders such as the hemolysis, elevated liver enzymes, low platelet count (HELLP) syndrome.

Chronic liver disease or hypersplenism – Isolated thrombocytopenia may be the initial manifestation of chronic liver disease with portal hypertension and congestive splenomegaly (hypersplenism). Thrombocytopenia is usually mild to moderate (eg, in

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the range of 60,000 to 100,000/microL), and the spleen is often palpably enlarged. In these conditions, the total number of platelets is normal; the low platelet count in the peripheral blood merely represents redistribution of a greater proportion of platelets into the congested spleen. Up to 90 percent of platelets may be pooled within the splenic circulation in patients with chronic liver disease and hypersplenism, compared with approximately one-third of platelets in individuals without hypersplenism.

Immune thrombocytopenia – Immune thrombocytopenia (ITP) is a common cause of moderate to severe thrombocytopenia in an otherwise asymptomatic adult. Other cell lines are unaffected (ie, ITP does not cause anemia or leukopenia). ITP is caused by antibody-mediated platelet destruction. However, anti-platelet antibodies are not always detected, and their testing is not clinically useful. A presumptive diagnosis of ITP is made when the history, physical examination, and laboratory data do not suggest an alternative diagnosis. Only a complete blood count (CBC) and review of the peripheral blood smear are required; however, other testing may be useful (eg, HIV and hepatitis C testing, liver enzymes for possible hepatic impairment).

Congenital platelet disorders – Some congenital platelet disorders associated with thrombocytopenia are discovered incidentally. Several may be associated with very large platelets on the peripheral blood smear (eg, May-Hegglin anomaly, Bernard-Soulier syndrome, Gray platelet syndrome). Although these are often diagnosed in childhood, an adult presentation is possible; some individuals carry a mistaken diagnosis of ITP.

Infection – Thrombocytopenia can occur with a variety of infections. The mechanism(s) may include immune-mediated platelet destruction, bone marrow suppression, or platelet consumption.

Viral – Thrombocytopenia may occur after a number of viral infections (eg, rubella, mumps, varicella, parvovirus, hepatitis C, and Epstein-Barr virus). Thrombocytopenia may be an incidental finding that resolves spontaneously as the patient recovers; however, in some persistent infections, such as hepatitis C, thrombocytopenia may also persist.

HIV – Human immunodeficiency virus (HIV) infection is a rare etiology of newly discovered thrombocytopenia. However, since thrombocytopenia may be the initial manifestation of HIV infection, it is appropriate to test for HIV as part of the evaluation of thrombocytopenia

Bacterial infection/sepsis – Sepsis can cause thrombocytopenia by direct bone marrow suppression, which is usually accompanied by other cytopenias; as a component of disseminated intravascular coagulation (DIC), in which it is accompanied by coagulation abnormalities (eg, prolonged PT, aPTT, low fibrinogen); or from platelet consumption independent of DIC. Patients are generally acutely ill, and treatment is directed at the underlying infection. Infection with helicobacter pylori has also been associated with immune thrombocytopenia. This may be suspected in patients with dyspepsia or symptoms of peptic ulcer disease.Thrombocytopenia is a common finding with many other infections including leptospirosis and brucellosis.

Intracellular parasites – Malaria and babesiosis can cause thrombocytopenia, typically accompanied by fever and hemolytic anemia; patients may present with mild illness or be acutely ill. The causative organisms may be seen on review of the peripheral blood smear.

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Drug-induced immune thrombocytopenia – Virtually any medication can cause thrombocytopenia by the occurrence of drug-dependent, platelet-reactive antibodies, but certain agents are more commonly implicated. Examples include antibiotics (eg, sulfonamides, ampicillin, piperacillin, vancomycin, rifampin); antiepileptic agents (eg, carbamazepine, phenytoin), and quinine Typically, thrombocytopenia develops within hours of drug exposure if the patient has been previously exposed to the drug, or within one to two weeks of daily exposure to a new drug, and resolves within five to seven days of drug discontinuation. Specific treatment is rarely required.

Heparin-induced thrombocytopenia – Heparin-induced thrombocytopenia is a special case of drug-induced thrombocytopenia in which anti-platelet antibodies cause platelet activation, resulting in an increased risk of venous and arterial thrombosis.

Cytotoxic chemotherapy or radiation therapy – Cytotoxic chemotherapy agents and radiation therapy induce a predictable, dose-dependent myelosuppression.

Alcohol – Alcohol can cause thrombocytopenia by direct toxicity to the bone marrow, nutrient deficiencies, or hypersplenism associated with alcoholic liver disease.

Malignancy – Cancer can cause thrombocytopenia as a component of chronic DIC, bone marrow infiltration, or thrombotic microangiopathy.

Nutrient deficiencies – Deficiency of nutrients required for hematopoiesis (eg, folate, vitamin B12, copper) typically causes mild pancytopenia, but isolated thrombocytopenia may be seen.

TTP-hemolytic uremic syndromes (TTP-HUS) – TTP-HUS is rare in adults, but must be considered in any adult with microangiopathic hemolytic anaemia and thrombocytopenia.

Patients with TTP-HUS rarely present with the full pentad of clinical features (microangiopathic hemolytic anemia, thrombocytopenia, neurologic findings, renal insufficiency, fever) that were common in the era before effective treatment. Many patients have only thrombocytopenia and microangiopathic hemolytic anemia. Acute kidney injury supports the diagnosis of HUS rather than TTP.

Bone marrow disorders – Several primary hematologic disorders cause thrombocytopenia; however, these disorders typically cause other abnormalities of the CBC (eg, pancytopenia, leukocytosis). Suspicion of any of these conditions should prompt hematologist involvement and bone marrow evaluation (see 'Additional evaluation' below).

Rheumatologic/autoimmune disorders – Systemic lupus erythematosus (SLE) is associated with secondary ITP in one-quarter to one-half of individuals. The antiphospholipid syndrome (APS) is an autoantibody-mediated syndrome characterized by venous or arterial thrombosis and/or pregnancy morbidity; stroke and other neurologic complications may occur. Mild to moderate thrombocytopenia is often present .Thrombocytopenia may be seen in rheumatoid arthritis and may be accompanied by splenomegaly (eg, Felty's syndrome

Other causes – Additional rare causes of thrombocytopenia include vascular conditions associated with platelet destruction (eg, giant capillary hemangioma, large aortic aneurysms, cardiopulmonary bypass)

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The history and examination is normal. The blood film shows only reduced platelets. The patient is discussed with the haematology team and agreed that they will follow up the next day.

3. What is the most likely diagnosis (1)ITP

4. Name three pieces of thrombocytopenia specific discharge advice would you give: (3)

Activity restrictions – Patients who are otherwise healthy and have no manifestations of petechiae or purpura may not require activity restrictions. Individual considerations apply to participation in certain activities. As an example, individuals with moderate to severe thrombocytopenia (<50,000/microL) should not participate in sports such as boxing, rugby, and martial arts. However, no restrictions are necessary for usual activities. Excessive mandated restrictions are more likely to decrease the information the patient provides to the physician than to reduce the patient’s activities.

Anti-platelet medications and over-the-counter remedies – Patients should be educated about which medications and non-prescription remedies interfere with platelet function (eg, aspirin non-steroidal antiinflammatory drugs). In general, these agents are avoided unless there is a specific indication for which equivalent alternatives are lacking.

However, appropriate use of thromboprophylaxis or anticoagulants should not be withheld from a patient with mild to moderate thrombocytopenia (eg, >50,000/microL) if it is indicated (eg, postoperatively). For patients with more severe thrombocytopenia, decisions are made on a case-by-case basis regarding the risks of bleeding and benefits of anticoagulation.

Safe platelet count for invasive procedures – Most platelet count thresholds for invasive procedures are based on weak observational evidence at best. In general, procedures with a greater risk of bleeding are performed at higher platelet counts. While there is some flexibility in individual circumstances, anesthesiologists and surgeons performing these procedures will have the last word.

Emergency management of bleeding – Urgent management of critical bleeding in the setting of severe thrombocytopenia requires immediate platelet transfusion, regardless of the underlying cause.

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SAQ 5

A 4 yr old girl presents to the department at 0400 with a high temperature, cervical lymphadenopathy and right hip pain. She is an only child and her parents are both concerned about her.

1. What other information would you like to know? (3). Appropriate history for leg pain. Appropriate history for pyrexia. Paediatric history. Baseline observations

Her mother states that it feels like she has had a ‘cold forever’ and has noticed gradually that she thinks her daughter is becoming more tired over the last few months.

2. Formulate an early differential diagnosis based on history? (3). Septic hip. Transient tenosynovitis. Irritable hip. Osteomyelitis. Juvenille arthritis. Discitis. ALL. Other haematological malignancy. Systemic infection

3. You are worried that there might be a ‘sinister’ cause for these symptoms. What 6 important examination features might you find? (3)

. Generalized weakness

. Dizziness

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. Pallor of anaemia

. Evidence of weight loss

. Excessive and unexplained bruising

. Bone pain, joint pain (caused by the spread of "blast" cells to the surface of the bone or into the joint from the marrow cavity)

. Breathlessness

. Enlarged lymph nodes, liver and/or spleen

. Pitting edema (swelling) in the lower limbs and/or abdomen

. Petechiae, which are tiny red spots or lines in the skin due to low platelet levels

. Signs CNS involvement

4. You are keen to admit the child to Paediatrics – but after the antipyretics and analgesics she has improved, appears well and would like to go home – outline your argument for admitting the child (1)

Acute lymphoblastic leukaemia (ALL) is the most common malignancy diagnosed in children, representing nearly one third of all paediatric cancers. Peak incidence in children aged 2-5 years. Although a few cases are associated with inherited genetic syndromes, the cause of ALL remains largely unknown.

ALL is generally thought to arise in the bone marrow, but leukemic blasts may be systemically present at the time of presentation. They may be present in the bone marrow, thymus, liver, spleen, lymph nodes, testes, and CNS.

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Children with ALL generally present with signs and symptoms that reflect bone marrow infiltration and extramedullary disease. Because leukemic blasts replace the bone marrow, patients present with signs of bone marrow failure, including anaemia, thrombocytopenia, and neutropenia. Clinical manifestations include fatigue and pallor, petechiae and bleeding, and fever. In addition, leukemic spread may manifest as lymphadenopathy and hepatosplenomegaly. Other signs and symptoms of leukaemia include weight loss, bone pain, and dyspnoea.

Signs or symptoms of CNS involvement, even when it occurs, are rarely observed at the time of the initial diagnosis. The signs and symptoms include headache, nausea and vomiting, lethargy, irritability, nuchal rigidity, papilledema. Cranial nerve involvement, which most frequently involves the seventh, third, fourth, and sixth cranial nerves, may occur. Also, leukaemia can involve as intracranial or spinal mass, which causes numerous neurologic symptoms, most of which are due to nerve compression.

Testicular involvement at diagnosis is rare. However, if present, it appears as painless testicular enlargement and is most often unilateral.

Physical: Physical findings in children with ALL reflect bone marrow infiltration and extramedullary disease. Patients present with pallor caused by anaemia, petechiae, and bruising secondary to thrombocytopenia. They also have signs of infection because of neutropenia. In addition, leukemic spread may be seen as lymphadenopathy and hepatosplenomegaly.

The incidence of ALL peaks in children aged 2-5 years.

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Lab Studies: Basic laboratory tests

o FBC (lymphoblasts) An elevated leukocyte count of >10 X 109/L (>10 X 103/µL) occurs in one half of patients with ALL. The degree of leukocytic elevation (blasts) at diagnosis remains the most important predictor of the patient's prognosis. Neutropenia, anemia, and thrombocytopenia may be observed secondary to inhibition of normal hematopoiesis by leukemic infiltration.

o Increased serum levels of uric acid, K, PO4, Ca, LDH. The degree of abnormality reflects the leukemic cell burden and destruction (lysis). PT, aPTT, fibrinogen level,

Children with ALL generally present with signs and symptoms that reflect bone marrow infiltration and extramedullary disease. Because leukemic blasts replace the bone marrow, patients present with signs of bone marrow failure, including anaemia, thrombocytopenia, and neutropenia. Clinical manifestations include fatigue and pallor, petechiae and bleeding, and fever. In addition, leukemic spread may manifest as lymphadenopathy and hepatosplenomegaly. Other signs and symptoms of leukaemia include weight loss, bone pain, and dyspnoea.

Signs or symptoms of CNS involvement, even when it occurs, are rarely observed at the time of the initial diagnosis. The signs and symptoms include headache, nausea and vomiting, lethargy, irritability, nuchal rigidity, papilledema. Cranial nerve involvement, which most frequently involves the seventh, third, fourth, and sixth cranial nerves, may occur. Also, leukaemia can involve as intracranial or spinal mass, which causes numerous neurologic symptoms, most of which are due to nerve compression.

Testicular involvement at diagnosis is rare. However, if present, it appears as painless testicular enlargement and is most often unilateral.

Physical: Physical findings in children with ALL reflect bone marrow infiltration and extramedullary disease. Patients present with pallor caused by anaemia, petechiae, and bruising secondary to thrombocytopenia. They also have signs of infection because of neutropenia. In addition, leukemic spread may be seen as lymphadenopathy and hepatosplenomegaly.

SAQ 6

You are asked to see a 6 year old child who has presented to the ED with a purpuric rash over the extensor surfaces of the limbs (mainly lower) and buttocks. They are also complaining of some mild abdominal pain and a sore knee. They are afebrile with a CRT<2 seconds and a heart rate of 100bpm.

1. What is the most likely diagnosis in this child? (1/2 mark)

HSP (Henoch-Scholein purpura)

2. What is the differential diagnosis for purpuric lesions in a child? (4 marks, ½ for each correct answer)

Meningococcal diseaseHSPSome viral illnessesTraumaForceful coughing or vomiting may cause petechiae of the faceThrombocytopenia

- ITP- Leukaemia- Septic shock- Aplastic anaemia

3. What investigations would you like to do on this child in the ED? (2 marks, ½ for each correct answer)

BPFBCUEUrinalysis

4. What complications might occur in this child? (3 marks, ½ for each correct answer)

Haematuria (in 90% of cases) Spontaneous bowel perforationNephritis PancreatitisRenal failure HaematemesisNephrotic syndrome HTNBloody stools IntussusceptionSubcutaneous oedema (hands, feet, scrotum)Rarely, pulmonary and CNS involvement

5. What treatment might this child require? (1/2 mark)

AnalgesiaPrednisolone (1mg/kg for 2 weeks) - Some data exists to support the use of prednisolone. It probably helps prevent the

development of long term renal complications. It can also reduce the duration of abdominal pain and joint pain and may reduce the risk of recurrent episodes.

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Lab Studies: Basic laboratory tests

o FBC (lymphoblasts) An elevated leukocyte count of >10 X 109/L (>10 X 103/µL) occurs in one half of patients with ALL. The degree of leukocytic elevation (blasts) at diagnosis remains the most important predictor of the patient's prognosis. Neutropenia, anemia, and thrombocytopenia may be observed secondary to inhibition of normal hematopoiesis by leukemic infiltration.

o Increased serum levels of uric acid, K, PO4, Ca, LDH. The degree of abnormality reflects the leukemic cell burden and destruction (lysis). PT, aPTT, fibrinogen level,