Oncology

Question 1

1. ALL: what are the most likely cells mutated?

common features of presentation?

Answer 1

pre B cell - 70%, T cell 30%
Anaemia
Neutropaenia
Thrombocytopaenia
effect from leukaemic cells: mediastinal mass/HSM/arthritis/testicular enlarment

Question 2

diagnosis of ALL?

Answer 2

flow cytometry (leukaemic blasts on film, or in CSF)

Question 3

most common childhood cancer?

Answer 3

ALL

Question 4

treatment for ALL?

Answer 4

induction (3 drugs) , consolidation, maintanance and CNS prophylaxis
BMT if refractory

Question 5

AML distinguishing features?

Answer 5

CHLOROMAS - gums, bleeds. hyperleukocytosis, priapism

Question 6

AML risk stratificaiton

Answer 6

cytogenetics, response to treatment

Question 7

AML treatment?

Answer 7

anthracycline, cytarabine, fludorbine, Gemtuzumab (CD33)

Question 8

APML features and risk stratification

Answer 8

WCC>10 high risk
PML RARA (15-17 translocation)

Question 9

treatmenr for APML:

Answer 9

retinoic acid and arsenic

Question 10

specific compliactions of APML

Answer 10

1. differentiation: promyelocytes differentiate to mature T cells and cause a cytokine storm - treat with steroids
2. DIC

Question 11

4. JMML features?

Answer 11

Aggressive and rare
associated with KRAS, PTPN11, NRAS and NF1 (noonan or neurofibromatosis)
often associated with a transient myeloproliferative disorder that often resolved but may turn into JMML

Question 12

treatment for JML?

Answer 12

mild chemo if syndromic (6 mercaptopurine)

BMT if not

Question 13

what cancers is trisomy 21 assocated with?

Answer 13

Transient abnormal myelopoeisis

AML and ALL

Question 14

Hodgekin lymphoma

Answer 14

reen-sterberg, 40% teenagers
B symptoms (weight loss, night sweats, itch)

Question 15

Ann arbor staging

Answer 15

Stage I indicates that the cancer is located in a single region, usually one lymph node and the surrounding area. Stage I often will not have outward symptoms.
Stage II indicates that the cancer is located in two separate regions, an affected lymph node or lymphatic organ and a second affected area, and that both affected areas are confined to one side of the diaphragm—that is, both are above the diaphragm, or both are below the diaphragm.
Stage III indicates that the cancer has spread to both sides of the diaphragm, including one organ or area near the lymph nodes or the spleen.
Stage IV indicates diffuse or disseminated involvement of one or more extralymphatic organs, including any involvement of the liver, bone marrow, or nodular involvement of the lungs.

Question 16

non hodgekin lymphomas

Answer 16

Burkitts

T or B cell lymphoma

Question 17

Burkitts characteristic

Answer 17

starry sky (sky is tumour cell, stars are the fat laden macrhophages)
sporadic

Question 18

risk stratification for Burkitts lymphoma?

Answer 18

CNS involvement

LDH level

Question 19

particular risk for Burkitts lymphoma?

Answer 19

at risk of tumour lysis syndrome if high grade tumours

Question 20

what is tumour lysis syndrome?

Answer 20

When cancer cells break down quickly in the body, levels of uric acid, potassium, and phosphorus rise faster than the kidneys can remove them. This causes TLS. Excess phosphorus can “sop up” calcium, leading to low levels of calcium in the blood

Question 21

who is at risk of tumour lysis syndrome?

Answer 21

Not all cancer patients are at equal risk of developing TLS. Patients with a large “tumor burden” of cancer cells and/or tumors that typically have rapidly dividing cells, such as acute leukemia or high-grade lymphoma, as well as tumors that are highly responsive to therapy, are at greatest risk of developing TLS. TLS can occur spontaneously (before cancer treatment) but is more common within a week of starting treatment.

Question 22

how is TLS treated?

Answer 22

In general, treatment of TLS consists of intensive hydration, stimulation of diuresis, and, more specifically, in the use of allopurinol and rasburicase. Rasburicase, a recombinant urate oxidase, rapidly and effectively reduces hyperuricemia, which subsequently significantly decreases the risk of acute renal failure and other clinical manifestations of TLS

Question 23

how is AKI caused in tumour lysis syndrome?

Answer 23

formation of uric acid crystals in the renal collecting system

Question 24

symtoms and signs of TLS?

Answer 24

This leads to series of metabolic manifestations, especially hyperuricemia, hyperkalemia, hyperphosphatemia, and hypocalcaemia. Besides seizures and cardiac arrhythmias, acute kidney injury (AKI) is the hallmark of TLS, which determines the clinical outcome

Question 25

treatment for Burkitt’s lymphoma?

Answer 25

Rituximab - CD20

Question 26

T and B cell lymphoblastic lymphoma?

Answer 26

same ass for ALL

Question 27

Tyrosine Kinase inhibitors?

Answer 27

JAK-STAT pathway

Question 28

Blentumumab

Answer 28

monoclonal ab for CD19 b cell (lymphoma cell) then attaches to CD3 cytotoxic t well and killed.

Question 29

CAR t cell treatmen?

Answer 29

autologous CD19 genetically modified t cells to attach to CD19 B wells and cause increased proliferation of them

Question 30

some side effects of Car t cell treatment:

Answer 30

cytokine release/cytokine storm

can use steroids to treat but this also decreases efficacy of the Car T cells

Question 31

ICANS

Answer 31

immune effector cell associated neurological syndrome

Question 32

clinical effects of ICANS?

Answer 32

fever, anaphylaxis, b cell aplasia, HLH