Which medication is associated with hypertrichosis and gum hyperplasia?
B. Cyclosporin. Causes hypertrichosis and gum hyperplasia in 5-19%
All other medications cause alopecia.
What is the single most common malignancy of childhood?
What is this a picture of?
Note no granules in cytoplasm.
What is pathogenesis of ALL?
Aetiology is unclear but must have
Maturation arrest and
What is peak age for ALL?
3 years then steadily decreases with another much smaller peak at approx 16 years.
What is second most common leukaemia of childhood?
What is cure rate of AML?
What is treatment of AML?
Cytarabine base chemo for 6-9 months
What are some presenting features of AML?
Leucopenia or leucocytosis
What is this and what does diagnosis do you consider?
Auer Rod = AML (pathognomonic)
In the FAB classification of AML, M7 is megakaryoblastic leukaemia. What population of people are more likely to have this diagnosis?
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?
Need full coag profile plus fibrinogen and then serial measurements.
What is treatment of APML?
Anthracycline & ATRC - Al Trans Retinoic Acid
Anthracycline and ATRA are used in treatment of APML. What does these drugs do, what is a significant side effect and what is survival?
Anthracycline helps mature blasts into healthier cells.
Beware ATRA syndrome - acute resp failure with pulmonary oedema.
Good outcomes if survive induction.
What are prognostic features of AML?
Age - worse with increasing age
Presentation WCC count - higher wcc = higher risk
CNS disease - poorer prognosis
favourable 8:21, 15:17
bad = monosomy 7, complex karyotype
- favourable 8:21, 15:17
- bad = monosomy 7, complex karyotype
When is Hodgkins Lymphoma more common?
What is usual presentation of Hodgkins Lymphoma?
Slowly progressing lymphadenopathy - often in teenage girl.
Paraneoplastic syndromes ie itch.
Prognosis of Hodgkin Lymphoma?
Good. 80% at 5 years depending on B symptoms.
5 lines on Burkitts Lymphoma
most common NHL
usually presents at high stage often with large abdominal mass
frequently bone marrow involvement
Cure in 85%
Treatment very intensive
Tumour Lysis Syndrome
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.
What usually causes fatality in tumour lysis syndrome?
Why does renal impairment/failure occur in tumour lysis syndrome?
Precipitation of calcium phosphate and uric acid in the tubules.
Calcium phosphate most likely to precipitate but most damage done by uric acid crystals.
What is management of tumour lysis syndrome?
Hyperhydration -NEVER with potassium
Allopurinol or rasburicase (uric oxidase) NOT both
Treat hyperkalaemia (sodibic)
Check electrolytes, phosphate and uric acid.
Why should you avoid giving Allopurinol and rasburicase together in treatment of tumour lysis sydrome?
Allopurinol - prevents formation of uric acid
Rasburicase - breaks down uric acid
allopurinol with decrease substrate for rasburicase so waste of medication.
Acrolein produced from degradation of cyclophosphamide causing haemorrhagic cystitis.
Mercaptoethane sulphonate (MESNA) used to prevent.
Where should vincristine NEVER be given?
Should only be via central IV - causes tissue chemical burns peripherally.
Hemihypertrophy and hemihyperplasia are associated with what oncological condition?
Beckwith-Wiedeman Syndrome associated with
- Wilms Tumour
What cells are depleted to minimise graft vs host disease?
This CT plus 2/12 progressive ataxia and headaches =?
35-40% posterior fossa tumours
2-3 month hx of headache, vomiting and truncal ataxia
Describe veno-occlusive disease of the liver.
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
- painful hepatomegally
- rapid weight gain
what is the major RF for fungal infection in a pt undergoing chemotherapy?
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
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?
- 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
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.
- fever, irritability, failure to thrive, bone pain, cytopenias, bluish subcutaneous nodules, orbital proptosis, and periorbital ecchymosis.
Lymphoblastic NHL usually originate from which cell line?
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.
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