Hematology / Oncology

Question 1

What factors leads to high risk classification of ALL?

Answer 1

• Age <1 or >10
• WBC >50 at diagnosis
• Cytogenetics
• Minimal residual disease* *most important!
• CNS Status
• Testicular involvement

Question 2

What are red flags for a diagnosis other than ITP?

Answer 2

• Age < 12 months
• FHx of “ITP”
• Congenital anomalies
• Consanguinity
• Constitutional symptoms
• Significant lymphadenopathy +/- hepatosplenomegaly
• Lack of response to first-line therapies?
• HistoryPhysical Exam*Ix**
• Constitutional symptoms (fevers, weight loss, night sweats)
• Bone pain
• Recurrent thrombocytopenia
• Poor treatment response
• Lymphadenopathy
• Hepatomegaly
• Splenomegaly
• Child is “unwell”
• Signs of chronic disease
• ​​Low Hgb (unless mildly low and explained by bleeding)

High MCV

• Abnormal WBC and/or neutrophil count
• Abnormal cellular morphology on smear

Question 3

What are possible triggers for G6PD?

Answer 3

Moth balls, fava beans, sulfa drugs, certain antimalarials, anti-pyretics? ASA

Question 4

What are possible triggers / predisposing factors to Hodgkin and non-Hodgkin lymphoma?

Answer 4

HL → Potential Triggers: EBV, CMV, HHV6

NHL → Predisposing Factors:

• Genetic → ataxia-telangiectasia, Bloom syndrome
• Immune deficiencies → SCID, Wiskott-Aldrich syndrome
• Viruses → HIV, EBV

Question 5

When should a biopsy be considered for an enlarged lymph node?

Answer 5

• Persistent or unexplained fever, weight loss, night sweats
• Supraclavicular location
• Mediastinal mass on CXR
• Hepatosplenomegaly on examination
• Hard nodes, or fixation of the nodes to surrounding tissues, or >2cm in size
• Increase in size over baseline in 2 weeks, no decrease in size in 4-6 weeks, or no regression to “normal” in 8-12 weeks

Question 6

What are the disorders that have a higher risk of neuroblastoma?

Answer 6

• Hirschsprung disease
• Central hypoventilation syndrome
• NF type 1
• Beckwith-Wiedmann & hemihypertrophy
• Turner, Noonan
• Pheochromocytoma

Question 7

What are associated syndromes with Wilm’s tumours?

Answer 7

• Familial cases are AD (earlier age of dx, increased f of bilateral disease)
• FW1 gene associated, FW2, WT1 (best characterized)
• Horseshoe kidney is RF
• Associated with hemihypertrophy, aniridia, GU anomalies, associated with Beckwith Wideman and Denys Dash Syndrome

Question 8

What are predisposing factors to leukemia?

Answer 8

• Genetic → Down syndrome, Bloom syndrome, ataxia-telangiectasia, Fanconi anemia, Diamond-Blackfan anemia, Shwachman-Diamond syndrome, SCID, Neurofibromatosis Type 1, Li-Fraumeni syndrome
• Environmental → radiation exposure in utero/childhood, drugs

Question 9

What are the main side effects of each of the following: MTX, Cisplatin/Carboplatin, Doxorubicin, VNC, Bleomycin, Cyclophosphamide, 5SFU/6MP

Answer 9

• MTX = Mucositis, Myelosuppression
• Cisplatin/Carboplatin = acoustic nerve damage, nephrotoxic
• Doxorubicin = Cardiotoxic
• VCN = peripheral neurotoxicity
• Bleomycin = pulmonary fibrosis
• Cyclophosphamide = hemorrhagic cystitis, fertility
• 5SFU/6MP = myelosuppression