Haematology

Question 1

what is acute myeloid leukemia (AML)

Answer 1

• heterogenous clonal stem cells malignancy in which immature hematopoietic cells proliferate and accumulate in bone marrow, peripheral
• results in inhinition of normal hematopoiesis, characterized by neutropenia, anemia and thrombocytopenia
• FAB subtype: M0-M7
• most common risk factor is previous exposure to radiation or chemotherapy
• inherited bone marrow failure
• genetic disorders: down syndrome

Question 2

test for AML

Answer 2

• blood smear
• cytology
• cytogenetics

Question 3

what are the 3 risk categories for AML

Answer 3

• favourable
• intermediate
• adverse

Question 4

treatment for AML

Answer 4

chemotherapy

• idarubicin/daunorubicin + cytarabine 3+7 regimen
• consolidation with HIDAC

allogenic stem cell transplantation for intermediate and poor risk disease

Question 5

what is leukostasis

Answer 5

high blast count -> hyperviscosity -> reduced tissue perfusion

Question 6

what is acute promyelocytic leukemia (APML)

Answer 6

• unique subtype of AML
• leukemia cells have a balanced reciprocal translocation between chromosomes 15 and 17
• exquisitely sensitive to ATRA, anthracyclines and arsenic trioxide

Question 7

what is acute lymphoblastic leukemia (ALL)

Answer 7

• ALL is a cnacer of lymphoid line of blood cells characterised by the development of large numbers of immature lymphocytes
• arise from a lymphoid progenitor cell that has sustained multiple genetic damage
• progresses rapidly and is typically fatal within weeks or months if left untreated

Question 8

types of ALL

Answer 8

precursor lymphoid neoplasm

• B lymphoblastic leukemia/lymphoma
• T lymphoblastic leukemia/lymphoma

mature lymphoid neoplasm
- burkitt leukemia/lymphoma

Question 9

treatment of ALL

Answer 9

multi-target chemotherapy

• induction
• consolidation-intensification
• maintenance

Question 10

what is chronic myeloid leukemia (CML)

Answer 10

• pluripotent hematopoietic stem cell neoplasm characterised by the BCR-ABL1 fusion gene, derived from balanced translocation between the long arms of chromosomes 9 and 22

Question 11

3 phases of CML

Answer 11

chronic
accelerated
blastic

Question 12

what is chronic lymphocytic leukemia (CLL)

Answer 12

• presents with lymphocytosis

- presents with symptoms referable to lymphadenopathy, splenomegaly, or anemia, fatigue, recurrent infection

Question 13

diagnosis of CLL

Answer 13

• presence in the peripheral blood of >5x10^9/L monoclonal B lymphocytes for >3months
• leukemia cells found in the blood smear are characteristically small, mature lymphocytes with a narrow border of cytoplasm and a dense nucleus lacking discernible nucleoli and having partially aggregated chromatin

Question 14

what is the Ann Arbor Staging for lymphoma

Answer 14

stage I - localised disease; single lymph node region or single organ

stage II - two or more lymph node regions on the same side of the diaphragm

stage III - two or more lymph node regions above and below the diaphragm

stage IV - widespread disease; multiple organs, with or without lymph node involvement

Question 15

treatment/management for lymphoma

Answer 15

chemotherapy

radiation

immunotherapy

• monoclonal antibodies
• cellular therapy - allogenic stem cell tx
• immunostimulants
• CAR (chimeric antigen receptor)

targeted therapy

• pathogenic molecules
• signalling pathway

Question 16

what is graft vs host disease

Answer 16

• chemotherapy and/or radiation causes tissue damage: toxins can enter blood
• pro-inflammatory proteins (cytokines) are produced in the remaining recipient cells and are present at increased level in the blood
• new cells enter the patient, T-cells in the graft proliferate and grow in response to the cytokines and caused damage to tissues

Question 17

explain CAR-T therapy

Answer 17

leukapheresis –> T-cell activation/transduction –> modified T-cell expansion –> chemotherapy –> modified T-cell infusion

Question 18

explain novel therapies

Answer 18

normally, bodies have immune checkpoints

• normal checkpoints of immune system
• role is to prevent immune response from being so strong that immune system destroys/attacks healthy cells in body
• when checkpoint protein and partner protein bind together, they send an OFF signal to prevent immune system from killing/attacking cancer cells

novel therapies:
- immunotherapy cells - block the checkpoint protein from binding to the partner protein –> prevents the OFF signal from being sent –> allowing T cells to irradicate tumour