Oncology

Question 1

Which medication is associated with hypertrichosis and gum hyperplasia?

A. Azathioprine.
B. Cyclosporin.
C. Methotrexate.
D. Mycophenolate.
E. Tacrolimus.

Answer 1

B. Cyclosporin. Causes hypertrichosis and gum hyperplasia in 5-19%

All other medications cause alopecia.

Question 2

What is the single most common malignancy of childhood?

Question 3

What is this a picture of?

Answer 3

Immature lymphocytes.

Note no granules in cytoplasm.

Question 4

What is pathogenesis of ALL?

Answer 4

Aetiology is unclear but must have

Maturation arrest and

uncontrolled proliferation

Question 5

What is peak age for ALL?

Answer 5

3 years then steadily decreases with another much smaller peak at approx 16 years.

Question 6

What is second most common leukaemia of childhood?

Answer 6

AML

Question 7

What is cure rate of AML?

Answer 7

60-65%

Question 8

What is treatment of AML?

Answer 8

Cytarabine base chemo for 6-9 months

Question 9

What are some presenting features of AML?

Answer 9

• Fever
• Malaise
• Wt loss
• Myalgia/arthralgia
• Lymphadenopathy
• Anemia, thrombocytopenia
• Hepatosplenomegally
• Leucopenia or leucocytosis
• Bleeding
• Masses
• gum hypertrophy

Question 10

What is this and what does diagnosis do you consider?

Answer 10

Auer Rod = AML (pathognomonic)

Question 11

In the FAB classification of AML, M7 is megakaryoblastic leukaemia. What population of people are more likely to have this diagnosis?

Answer 11

Downs Syndrome

Question 12

In the FAB classification of AML, M3 is APML (Acute Promyelocytic Leukaemia).

What do you need to be careful to look for on diagnosis of APML?

Answer 12

DIC.

Need full coag profile plus fibrinogen and then serial measurements.

Question 13

What is treatment of APML?

Answer 13

Anthracycline & ATRC - Al Trans Retinoic Acid

Question 14

Anthracycline and ATRA are used in treatment of APML. What does these drugs do, what is a significant side effect and what is survival?

Answer 14

Anthracycline helps mature blasts into healthier cells.

Beware ATRA syndrome - acute resp failure with pulmonary oedema.

Good outcomes if survive induction.

Question 15

What are prognostic features of AML?

Answer 15

• Age - worse with increasing age
• Presentation WCC count - higher wcc = higher risk
• CNS disease - poorer prognosis
• Cytogenetics
  
  • favourable 8:21, 15:17
  • bad = monosomy 7, complex karyotype

Question 16

When is Hodgkins Lymphoma more common?

Answer 16

2nd decade

Question 17

What is usual presentation of Hodgkins Lymphoma?

Answer 17

Slowly progressing lymphadenopathy - often in teenage girl.

Mediastinal mass

“B” symptoms

Paraneoplastic syndromes ie itch.

Question 18

Prognosis of Hodgkin Lymphoma?

Answer 18

Good. 80% at 5 years depending on B symptoms.

Question 19

5 lines on Burkitts Lymphoma

Answer 19

• most common NHL
• Very aggressive
• usually presents at high stage often with large abdominal mass
• frequently bone marrow involvement
• Cure in 85%
• Treatment very intensive

Question 20

Tumour Lysis Syndrome

Answer 20

• Seen in malignancies with high tumour burden and high mitotic rate
• might be present at diagnosis
• usually commences after starting chemo

Lysis of tumour cells leads to release of intracellular electrolytes incl K+, Ca2+, phosphate and uric acid.

Question 21

What usually causes fatality in tumour lysis syndrome?

Answer 21

Hyperkalaemia

Question 22

Why does renal impairment/failure occur in tumour lysis syndrome?

Answer 22

Precipitation of calcium phosphate and uric acid in the tubules.

Calcium phosphate most likely to precipitate but most damage done by uric acid crystals.

Question 23

What is management of tumour lysis syndrome?

Answer 23

• Hyperhydration -NEVER with potassium
• Allopurinol or rasburicase (uric oxidase) NOT both
• Treat hyperkalaemia (sodibic)
• Check electrolytes, phosphate and uric acid.

Question 24

Why should you avoid giving Allopurinol and rasburicase together in treatment of tumour lysis sydrome?

Answer 24

Allopurinol - prevents formation of uric acid

Rasburicase - breaks down uric acid

allopurinol with decrease substrate for rasburicase so waste of medication.

Question 25

Haemorrhagic cystitis

Answer 25

Acrolein produced from degradation of cyclophosphamide causing haemorrhagic cystitis. Mercaptoethane sulphonate (MESNA) used to prevent.

Question 26

Where should vincristine NEVER be given?

Answer 26

Intrathecal. Should only be via central IV - causes tissue chemical burns peripherally.

Question 27

Hemihypertrophy and hemihyperplasia are associated with what oncological condition?

Answer 27

Beckwith-Wiedeman Syndrome associated with * Wilms Tumour * Hepatoblastoma

Question 28

What cells are depleted to minimise graft vs host disease?

Answer 28

T cells

Question 29

This CT plus 2/12 progressive ataxia and headaches =?

Answer 29

Medulloblastoma 35-40% posterior fossa tumours 2-3 month hx of headache, vomiting and truncal ataxia

Question 30

Describe veno-occlusive disease of the liver.

Answer 30

* Occurs in first 3/52 after transplant * Fibrous obliteration of terminal hepatic venules and small lobular veins. Results in damage to surrounding hepatocytes and sinusoids. * Most common cause of hepatic vein obstructioin in children * Cause of VOD after bone marrow transplant not clear **Symptoms** * **Jaundice** * **painful hepatomegally** * **rapid weight gain** * **ascites**

Question 31

what is the major RF for fungal infection in a pt undergoing chemotherapy?

Answer 31

**Neutropenia** Two major fungal infection subtypes occur in this population:**Yeasts** (such as Candida species) + **moulds** (Aspergillus fumigatus being the most common) The longer the period of neutropenia, the greater the risk of fungal infection and after 5-7 days of febrile neutropenia, an antifungal agent is usually added empirically due to the high risk of systemic fungal infection

Question 32

11 y/o who has undergone T-cell depleted unrelated bone marrow transplant for relapsed acute lymphoblastic leukaemia. Recovery is complicated by slow neutrophil engraftment, grade 3 acute graft-versus-host disease requiring high dose methylprednisolone, and persistent fevers. The computerised tomography (CT) scan of her chest, performed at day +50 post- transplantation, is shown below. Most likely pathogen?

Answer 32

**Aspergillus fumigatus** * Invasive Aspergillosis is a significant cause ofinfectious morbidity and mortality in hamatopoietic stem cell transplant recipients. * Incidence is about 10% in HLA mismatched or unrelated donor transplant. * Most dx 40-180 days after transplant * Risk is influenced by duration of neutropaenia, GVHD, use of corticosteroids, post-transplant CMV, viral respiratory tract infections, older age and T cell depletion of the graft * There are several characteristic signs of invasive aspergillosis on CT scan, pulmonary nodules enlarge and the dense central core of infarcted tissue becomes surrounded by oedema or haemorrhage forming a hazy rim (below). In neutropaenic patients, when bone marrow function recovers, the infarcted central core cavitates creating the **crescent sign**

Question 33

Neuroblastoma

Answer 33

* Neuroblastomas are **extracranial solid tumours**, median age of presentation **22 months**. * Most common extracranial tumour * They may develop at any site of sympathetic nervous system tissue, with **half arising in the adrenal glands**. * Metastatic disease can lead to * fever, irritability, failure to thrive, bone pain, cytopenias, bluish subcutaneous nodules, orbital proptosis, and periorbital ecchymosis. * Children with neuroblastoma can present with neurologic signs and symptoms, including Horner syndrome if the superior cervical ganglion is involved. * Children with extensive tumours are at risk of DIC and tumour lysis syndrome.

Question 34

Lymphoblastic NHL usually originate from which cell line?

Answer 34

T cells

Question 35

A 6 year old girl receiving chemotherapy for acute myeloid leukaemia presents in the early hours of Sunday morning with fever, bruising, petechiae and oral mucosal bleeding. Her platelet count is 4 x 109/L [150-400] and her blood group is Group A Rh negative. Platelet transfusion is indicated. The transfusion service does not have irradiated Group A Rh negative platelets available. Which of the following products would be most appropriate to request as an alternative? A) Irradiated Group A Rh positive pooled donor platelets. B) Irradiated Group A Rh positive single donor platelets. C) Irradiated Group O Rh negative pooled donor platelets. D) Irradiated Group O Rh negative single donor platelets. E) Unirradiated Group A Rh negative single donor platelets.

Answer 35

E = Unirradiated Group A Rh negative single donor platelets ABO and Rh(D) identical platelet transfusion is ideal Platelets collected from Rh(D) positive donors should be avoided for Rh(D) negative recipients. Platelets do not express Rh antigens on their surface, but the quantity of RBCs in platelet preparations may be sufficient to induce Rh sensitization, even in immunosuppressed cancer patients. If transfusion of RhD positive product to RhD negative recipient is unavoidable, consider giving Rhesus immunoglobulin (anti-D) particularly in girls Avoid incompatible plasma. Transfusing platelets from group O donors to group A, B or AB recipients may result in haemolysis (from anti-A and anti- B in group O plasma). Children and infants are more at risk than adults due to their small blood volume. If ABO incompatible transfusion is unavoidable, the use of pooled platelets in additive solution (T-Sol) may reduce the risk of haemolysis Immunocompromised patients should have irradiated blood products to prevent graft-vs-host disease There is no difference between pooled and single donor platelets in studies