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2004 FRACP paper one

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Question 1 top

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Question 3 top Download PDF

A 68yo man scheduled for hemiorrhaphy presents for pre-anaesthetic assessment and is found to have non-tender cervical lymphadenopathy.

Full blood examination shows:

                Haemoglobin          125 g/L                                                  [128-175]
                MCV                         96 fL                                                       [80-97]
                White cell count      19.5 x 109/L                                         [3.9-12.7]
                                Differential:
                                                Neutrophils           2.5 x 109/L          [1.9-8.0]
                                                Lymphocytes        16.2 x 109/L        [0.9-3.3]
                                                Monocytes            0.3 x 109/L           [0.3-1.1]
                                                Eosinophils            0.4 x 109/L          [0-0.5]
                                                Basophils               0.1 x 109/L          [0-0.1]
                Platelet count                                       213 x 109/L         [150-396]

The blood film is shown below.

 

With respect to the peripheral blood lymphoid cell immunophenotype, which of the following surface antigens is most likely to be co-expressed with CD19 (cluster of differentiation 19)?

  1. CD3
  2. CD5
  3. CD8
  4. CD10
  5. CD34

 

 

 

 

The blood film shows smear cells and mature-looking lymphocytes.
This, along with the elevated lymphocyte count indicates a diagnosis of CLL.

CHRONIC LYMPHOCYTIC LEUKAEMIA

  1. B-cell CLL is considered to be identical to the mature B-cell small lymphocytic lymphoma
  2. Characterised by progressive accumulation of functionally incompetent lymphocytes which are monoclonal in origin

 

Clinical Presentation

  1. B symptoms – weight loss, fever, night sweats, fatigue
  2. Lymphadenopathy
  3. Splenomegaly
  4. Hepatomegaly
  5. Elevated WCC
  6. Anaemia
  7. Thrombocytopaenia
  8. Many people asymptomatic

 

Laboratory Findings

  1. Lymphocytosis in peripheral blood and bone marrow
  2. Smudge cells on blood smear – reflects fragility of B-CLL cells – characteristic of CLL
  3. Mature-appearing small lymphocytes with large nucleoli and only a thin rim of surrounding cytoplasm
  4. Mature-appearing but functionally and developmentally immature
  5. 3 major sets of phenotypic findings of B-CLL lymphocytes:
    1. Extremely low levels of surface Ig
    2. Expression of one or more B-cell associated antigens – CD19, CD20 (weak), CD21, CD23 and CD24 monoclonal antibodies
    3. Expression of CD5, a T-cell associated antigen
  6. In addition, CLL cells are usually negative for cyclin D1 and CD10. FMC7, CD22 and CD79b are usually negative or weakly expressed
  7. Scoring system – one point for each cellular characteristic:
    1. Staining for surface Ig weakly positive
    2. CD5 positive
    3. CD23 positive
    4. CD79b or CD22 weakly positive
    5. FMC7 negative
  8. 4-5 points = 97% accuracy for Dx of CLL
  9. Most non-CLL B-cell lymphoproliferative diseases had scores 0-2
  10. Higher risk of autoimmune haemolytic anaemia and thrombocytopaenia and hypogammaglobulinaemia

 

BMAT

    • Increased percentage of mature-appearing lymphocytes
    • Nodular, interstitial or diffuse infiltrative patterns

     

    From UTD – bone marrow aspirate
    Note lymphocytosis

    Lymph Node Biopsy

    1. Diffusely effaced nodal architecture with occasional residual naked germinal centres
    2. Infiltrate of mature-appearing small lymphocytes
    3. Low mitotic activity

     

    Chromosomal Changes

    1. No changes that are specifc for CLL but certain patterns are common:
      1. Trisomy of chromosome 12, 3, 16
      2. Del (13q14)
      3. Del (11q)
      4. Del (17p)

     

    Minimum Diagnostic Requirements

    1. Absolute lymphocyte count in peripheral blood >10 with predominantly mature-appearing small lymphocytes
    2. Normocellular to hypercellular bone marrow with lymphocytes accounting for >30% of all nucleated cells
    3. If lymphocyte count 5-10 then need to do lymphocyte phenotyping to show low surface Ig, one or more B cell associated antigens (CD19, CD20, CD21, CD23, CD24) and T cell associated antigen CD5

     

    Differential Diagnosis

    1. Lymphoma (generally has abundant surface Ig)
    2. Other forms of leukaemia – can differentiate on film/BMAT/ immunohistochemistry
    3. Infectious mononucleosis, pertussis, toxoplasmosis – transiently elevated lymphocyte count

     

    Answer: B

    Prognosis

    1. Usually an initial asymptomatic phase which can last a few months to many years
    2. Survival 2 to 20 years
    3. Often transforms into another lymphoproliferative disorder which can be related to the original B-CLL cells or derived from a separate cell of origin
    4. Most common transformations are:
      1. Prolymphocytic leukaemia
      2. Aggressive lymphoma (Richter’s transformation)
      3. Hodgkin’s disease
      4. Multiple myeloma
    5. Higher risk of second malignancy (lung, GIT, skin)
    6. Prognosis based on staging (Rai):
      1. Lymphocytosis only – 150 month survival
      2. Lymphadenopathy, organomegaly – 70-100 month survival
      3. Anaemia, thrombocytopaenia – 9 month survival

     

    Treatment

      • Not felt to be a curable disease
      • Best treatment regime not known
      • Chlorambucil or cyclophosphamide with or without corticosteroids
      • Second line are nucleoside analogs (eg: fludarabine)
      • Rituximab has also be used as first line
      • Autologous haematopoietic cell transplantation

       

      Topic

      Haematology: Leukaemia

      Question 4 top Download PDF

      A hospitalised patient with a rash on her face and a lace-like rash on her extremities has a confirmed diagnosis of erythema infectiosum (parvovirus B19).

      Which of the following infection controls would be the most appropriate?
      A. Standard.
      B. Contact.
      C. Respiratory.
      D. Single room.
      E. Negative pressure room.

      Infection with parvovirus B19 can lead to a variety of clinical manifestations depending on the patient infected.

      Most commonly affects children causing erythema infectiosum, a mild febrile illness with a rash.

      In adults 25% will be asymptomatic, 50% will have a flu-like illness and 25% will develop rash or arthralgia.

      Parvovirus infects erythroid cells.

      Infection with parvovirus leads to a Th-1-mediated cellular immune response with production of IgM antibodies and subsequent formation of immune complexes.

      Can occur at any age but primarily affects children aged 3 to 15yrs.

      Incubation period is to 14 days. Spread primarily via aerosol respiratory droplets. In hosts who are immunocompetent, the patient is viraemic and capable of spreading the infection only during the incubation period. Once the patient has developed clinical signs such as a rash or arthralgias they are no longer infective.
      Tends to spread easily among families (but less so in schools).

      Physical Findings:
      • Slapped-cheek appearance (2-4 days)
      • Macular rash mainly on extremities then develops (1-4 days)
      • Followed by lacy, reticular pattern on proximal extremities (3 days-3 weeks)
      • Polyarthropathy (more common than skin findings in adults) – symmetrical, worsens over the day, hands, wrists, knees, ankles

      Complications:

      • Aplastic crisis - high risk in patients with underlying haematological disorders (eg: sickle cell, thalassaemia, G6PD deficiency)
      • Chronic bone marrow failure - immunocompromised hosts are at risk
      • Congenital infection - PV-B19 crosses placenta and can lead to:
        • Severe fetal anaemia
        • Congestive heart failure
        • Hydrops fetalis
        • Intrauterine death (in 3 to 10% of mothers who are infected)
      • Variety of other disorders that may be linked to or triggered by PV-B19:
        • Hepatitis
        • Myocarditis
        • Viral-associated haemocytophagia
        • Vasculitis
        • Nephrotic syndrome
      So the correct answer is A - no precautions required as the patient has already developed the rash so is no longer infective.

      If the patient was known to be infected but had not yet developed any signs or symptoms (even though very unlikely to ever know this) then respiratory precautions would be advisable to prevent spread.

      Topic

      Infectious Diseases - Simple principles of infection control in hospital and notification of infectious diseases.

       

      Question 5 top Download PDF

      Knee pain experience principally when walking downstairs is most likely to reflect pathology in which of the following knee structures?

        A. Medial compartment
        B. Lateral compartment
        C. Retropatellar compartment
        D. Suprapatellar bursa
        E. Lateral meniscus

         

        Three Compartments:

        1. Medial tibiofemoral
        2. Lateral tibiofemoral
        3. Patellofemoral

         

        Knee pain can be due to one of the following:

        1. Intraarticular process such as meniscal or ligamentous injury or fracture
        2. Patellar malalignment or dysfunction
        3. Cartilage loss due to OA or synovitis
        4. Periarticular bursitis or tendonitis
        5. Referred pain from hip, femur or spine
        6. Inflammatory arthritides

         

        Pain Patterns:

        1. Medial knee pain refers to medial compartment OA, bursitis, medial ligament strain, medial meniscal tear
        2. Anterior knee pain refers to problems with quadriceps muscle or tendon, patella or patellar tendon or large knee effusion
        3. Lateral knee pain refers to lateral compartment OA, lateral collateral ligament strain, lateral meniscal tear
        4. Popliteal pain refers to can accompany any of above. May be caused by effusion

         

        I think this can be worked out by exclusion... B and E are both sort of the same. There is no reason why walking down stairs will put more strain on one side of the knee than the other so A and B can be excluded as well as E. We are left with retropatellar compartment problems (ie. patellofemoral) or suprapatellar bursitis. Generally a bursitis will be sore ALL the time, even at rest. It will be painful to move at all, not just on walking down stairs. So the answer is C.

        Topic

        Rheumatology:soft tissue rheumatism

        Question 6 top Download PDF

        During pregnancy, many cardiovascular conditions are associated with an increased risk of maternal mortality.

        Which one of the following cardiovascular conditions is associated with the highest rate of maternal mortality?

        Moderate to severe:

        A. Pulmonary hypertension
        B. Hypertrophic cardiomyopathy
        C. Coarctation
        D. Aortic regurgitation
        E. Peripartum cardiomyopathy

        While all the conditions are associated with an increased risk pulmonary HT carries the greatest risk to the mother so answer is A.

        NORMAL PHYSIOLOGY OF PREGNANCY
        Cardiac Output
        • rise 30 – 50%
        • influenced by posture (increased in L lateral, decreased when supine)
        • preload increased due to increased blood volume
        • afterload reduced due to decreased systemic resistance
        • increased HR
        • Can cause decompensation in pts with previously asymptomatic heart disease
        Blood Pressure
        • Lower due to reduced systemic vascular resistance
        • Resistance reduced due to vasodilation and low-resistance circuit of uteroplacental circulation
        Physical Examination
        • Distended neck veins, basal creps, left and right ventricular heaves, exaggerated heart sounds and flow murmur are all normal in pregnancy
        • Physiological S3 gallop
        • Peripheral oedema in 3rd trimester
        ECG
        • Shortened PR and QT intervals, increased HR
        • Non-specific ST and T wave changes in 4-14% of pregnancies
        Labor and Delivery
        • Increased cardiac output due to increased blood volume from intramyometrial blood and increased BP and HR associated with uterine contractions

        Predictors of cardiac events during pregnancy
        • NYHA class II to IV or cyanosis
        • Previous cardiac event
        • Left heart obstruction (MV area < 2cm2, AV area <1.5cm2, peak LV outflow gradient >30mmHg)
        • Left ventricular systolic dysfunction (LVEF <40%)
        • 0 points: 5% risk of event
        • 1 point: 27% risk of event
        • 2 or more points: 75% risk of events
        • Correlates with neonatal risk


        VALVULAR HEART DISEASE

        High risk:
        • Severe AS with or without symptoms

        Severity of aortic stenosis in adults

          Aortic jet velocity, m/sec Mean gradient, mmHg Valve area, cm2

        Normal
        1.5 less than 5 3.0-4.0
        Mild 1.5 - 3.0 5 - 25 1.5 - 3.0
        Moderate 3.0-4.0 25-40 1.0-1.5
        Severe* greater than 4.0 greater than 40 less than 1.0
        • Symptomatic MS (NYHA class II to IV)
        • Aortic or mitral regurgitation with NYHA class III to IV
        • Aortic or mitral valve disease with severe LV dysfunction (EF <40%) or severe pulmonary HT (PAP >75% of systemic pressure)
        • Marfan syndrome with or without AR
        • Mechanical prosthetic valve requiring anticoagulation
        Low risk:
        • Asymptomatic AS with LVEF >50% and mean gradient less than 25mmHg
        • AR or MR with no or mild symptoms (NYHA class I to II)
        • MV prolapse with either no MR or mild-mod MR with LVEF >50%
        • Mild MS (valve area >1.5cm2, mean gradient less than 5mmHg) without severe pulmonary HT
        • Mild to mod pulmonary valve stenosis

        CARDIOMYOPATHY

        • Heart failure, arrhythmia and stroke more common in women whose LVEF <40%
        • Pregnancy not recommended if LVEF <40%
        • Pregnancy generally well tolerated in HCM (low mortality)
          CONGENITAL HEART DISEASE
        Major risk factors for complications
        • Pulmonary HT
        • Maternal cyanosis
        • Poor maternal functional class
        • History of arrhythmia
        • Maternal anticoagulants
        Pulmonary HT
        • Pulmonary HT is the most serious risk to the mother, especially Eisenmenger syndrome
        • Limits appropriate adaptive responses to circulatory changes
        • Maternal mortality with Eisenmenger syndrome is 30 – 50%
        • Reversed right to left shunt is worsened with decreased systemic vascular resistance ? cyanosis
        • During labor increased systemic resistance can cause abrupt reduction in CO and fatal syncope
        Cyanosis
        • Arterial oxygen saturation prior to pregnancy is one of the most important predictors of poor fetal and maternal outcomes
        • Likelihood of live birth significantly reduced when sats < 85%
        Coarctation
        • Increased risk of rupture or dissection
        • Independent predictor of preeclampsia
        Left to Right Shunts
        • ASD generally not a problem in young, otherwise well women
        • ASDs associated with increased risk of AF, flutter in pts >30yrs
        • Risk of paradoxical embolisation
        • VSD ok if mild-mod
        • Large VSD (>1cm2) usually have Eisenmenger syndrome and are at high risk
        • PDA usually ok, most often repaired

        So while all the conditions are associated with an increased risk pulmonary HT carries the greatest risk to the mother so answer is A.

        Topic

        Cardiology - Cardiac changes and problems in pregnancy

        Question 7 top

        Question 8 top

        Question 9 top Download PDF

        Diagnosis of a number of autoimmune diseases involves the detection of serum autoantibodies. Which of the following criteria is most indicative of a pathogenic role for a disease-associated autoantibody?

        A. Demonstration of immunoglobulin at site of lesion
        B. Elution of immune complexes from site of lesion
        C. Reproduction of disease by passive transfer of patient immunoglobulin
        D. Clinical response to plasmapheresis
        E. Elution of complement from site of lesion

        CRITERIA FOR AUTOIMMUNE DISEASE

        1. Naturally occurring antibodies are common
        2. Presence of autoantibodies does not establish autoimmunity as cause of illness
        3. Direct evidence of causality implies that autoimmune response can be shown to produce the disease
        4. This can only be done by the transfer of autoantibody from a patient to a healthy recipient (human or animal)
        5. For example, pemphigus was reproduced in a mouse after injection of patient serum
        6. Other example is in pregnancy – transplacental transmission of disease can occur (eg: complete heart block associated with lupus) and resolves after birth
        7. This is the best evidence possible
        8. Can also have indirect evidence (eg: disease occurring in animal after immunisation with appropriate antigen) or circumstantial evidence (eg: presence of autoantibodies, clustering of disease)

         

        Answer: C

        Topic

        Rheumatology:epidemiology

         

        Question 10 top Download PDF

        A patient presents 12 hours after a thunderclap headache. A contrast cranial CT scan is normal.

        To determine if there is aneurysmal bleeding, the most appropriate next investigation is:

        1. Cranial MRI with diffusion weighted imaging
        2. CSF spectroscopy for bilirubin
        3. CSF spectroscopy for crenated red blood cells
        4. Four vessel cerebral angiogram
        5. Cranial magnetic resonance imaging/angiography (MRI/MRA)

         

        SAH

        • 80% causes by ruptured aneurysms
        • Other causes include trauma, AV malformations, vasculitis, dissection, amyloid, bleeding diathesis and illicit drug use (especially cocaine and amphetamines)

         

        Risk Factors:

        • Smoking
        • HT
        • Alcohol
        • Family history
        • Oestrogen deficiency
        • ?Antithrombotic therapy

         

        Manifestations:

        • Ruptured aneurysm --> blood in CSF --> increased intracranial pressure --> thunderclap headache (97%)
        • May be brief LOC, SZ, nausea or vomiting
        • Meningism and lower back pain can occur after several hours
        • Warning leak (sentinel headache) in 30 to 50%
        • Physical exertion may be trigger
        • Lack of lateralising signs
        • Terson’s syndrome = vitreous haemorrhages
        • Retinal subhyaloid haemorrhages

         

        Hunt and Hess grading system for patients with subarachnoid haemorrhage

        GRADE

            NEUROLOGICAL STATUS

        1

        Asymptomatic or mild headache and slight nuchal rigidity

        2

        Severe headache, stiff neck, no neurologic deficit except cranial nerve palsy

        3

        Drowsy or confused, mild focal neurologic deficit

        4

        Stuporous, moderate or severe hemiparesis

        5

        Coma, decerebrate posturing

        Based upon initial neurologic examination; adapted from Hunt, W, Hess, R, J Neurosurg 1968; 28:14.
        From UTD

        Complications:

        • Death (average 51%)
        • Vasospasm
          • Due to release of vasoactive substances from clot lysis
          • Uncommon prior to day 3, peaks day 7-8
          • More likely with large clot, GCS < 14
          • Can lead to infarction
          • Early vasospasm is rare but can occur (likely different mechanism)
        • Hydrocephalus (acute or chronic)
        • Increased ICP
        • Seizures
        • Hyponatraemia
          • Hypothalamic injury --> ADH secretion --> water retention
          • Or could be due to cerebral salt wasting
        • Cardiac abnormalities
          • STD, TWI, prolonged QT, arrhythmias
          • Hypoperfusion of posterior hypothalamus --> centrally mediated release of catecholamines within myocardium --> LV ischaemia
          • Other factors likely involved
        • Rebleeding (highest risk in first 24/24)
            • Higher risk if large aneurysm, high Hunt-Hess grade, sentinel headache

         

        Diagnosis:

        • CT and LP
        • CT within 12 hours has high sensitivity (close to 100%)
        • LP should always be performed if high clinical suspicion but CT normal
        • Classic findings are elevated opening pressure and RBCs in all tubes
        • Xanthrochromia represents haemoglobin degradation products (means blood in CSF for > 2hours)
        • Spectrophotometry detects blood breakdown products (oxyHb --> metHb --> bilirubin)
        • Highly sensitive when done after 12 hours
        • Negative CT and CSF effectively eliminates the diagnosis of SAH
        • Could consider angiography/MRA if diagnosis still in doubt
        • FLAIR and T2 sequences on MRI have high sensitivity for subacute SAH (ie. Few days after bleed)

        Identifying Aetiology:

        • Cerebral angiography
        • CTA and MRA are useful for screening and presurgical planning but not as good resolution as angiogram – miss aneurysms < 3mm

        Answer is B

        THUNDERCLAP HEADACHE

        Causes:

        • SAH
        • Sentinel headache
        • Cerebral venous thrombosis
          • Usually also have papilloedema, SZ, bilateral focal deficits and/or decreased GCS
          • Usually gradual onset of headache
          • Usually history of thromboembolic disease
          • CT can be normal
          • LP will have elevated opening pressure, can be elevated lymphocytes, RBCs and protein in 30-50%
          • MRV for diagnosis
        • Cervical artery dissection
          • Headache common presentation
          • Usually gradual and unilateral +/- neck pain
          • Neurological signs
          • CT, LP normal – need U/S or angiography or MRA or CTA
        • Spontaneous intracranial hypotension
          • Orthostatic headaches
          • Nausea/vomiting, dizziness, auditory changes, visual blurring, interscapular pain, radicular pain of ULs
          • Most common cause is CSF leak
          • Thunderclap headache uncommon presentation
          • Dx with MRI (CT, LP normal)
        • Pituitary apoplexy
          • TCH may be predominant feature
          • Other features include ophthalmoplegia, decreased visual acuity, change in mental status
          • Adrenal crisis can occur
          • CT, LP normal
          • Need MRI to diagnose
        • Retroclival haematoma
          • Rare manifestation of severe head and neck injuries where there is atlantoaxial dislocation
          • Spontaneous haemorrhage can also occur
        • Ischaemic stroke
          • Rarely
        • Acute hypertensive crisis
          • Headaches more commonly throbbing and indistinct
          • MRI more sensitive than CT at showing white matter changes consistent with reversible posterior leukoencephalopathy syndrome
        • Colloid cyst of third ventricle
          • Cyst can suddenly impede flow of CSF results in obstructive hydrocephalus
          • Headaches usually of sudden onset and resolution lasting seconds to 1 day
          • Associated nausea, vomiting, changes in mental state, SZ, coma and death can occur
          • Dx by CT or MRI
        • Infections
          • Usually gradual onset, rarely TCH with meningitis
          • Acute sinusitis can rarely cause TCH
        • Reversible cerebral vasoconstriction syndrome
          • Group of disorders characterised by reversible vasospasm of cerebral arteries
          • Dx by angiography/MRA
          • Usually self-limiting
        • Primary TCH
          • Benign, potentially recurrent headache disorder
          • No organic pathology
          • Diagnosis of exclusion

        Topic

        Neurology: subarachnoid haemorrhage | headache