Haematological Malignancy 1 Flashcards

Question

What leukaemia has the worst prognosis

Answer 1

Acute lymphocytic leukaemia in adults

Answer 2

Chronic myeloid leukaemia ALL in children Chronic lymphocytic leukaemia

Answer 3

Without it, clinical trial results could not be compared between countries Research results could not be evaluated collectively Epidemiological studies would be impossible Its the basis of cancer science internationally

Answer 4

Morphology Genetic features Cytochemistry Immunophenotyping Immunohistochemistry Molecular features

Answer 5

Emphasis on therapeutically and/or prognostically actionable biomarkers Genetic basis is sought where possible, but classification strives to keep practical worldwide applicability in perspective Result is an enhanced, contemporary, evidence-based classification of myeloid and histiocytic/dendritic neoplasms, rooted in molecular biology and an organisational structure that permits future scalability as new discoveries continue to inexorably inform future efitions

Answer 6

Acute leukaemias Lymphoproliferative disorders Myeloproliferative disorders Lymphomas

Answer 7

Chronic lymphocytic leukaemia

Answer 8

Chronic myeloid leukaemia Essential thrombocythaemia, polycythemia vera, myelofibrosis

Answer 9

In normal bone marrow 4% of cells are myeloblasts They should not be apparent in peripheral blood They have a high nucleocytoplasmic ratio (really big nucleus) They have a fine open lacy chromatin pattern accompanied by prominent nucleoli Cytoplasm stains a light blue

Answer 10

They have a high nucleocytoplasmic ratio (really big nucleus) They have a fine open lacy chromatin pattern accompanied by prominent nucleoli Cytoplasm stains a light blue

Answer 11

Increasing numbers can suggest myeloid leukaemia

Answer 12

Promyelocytes

Answer 13

Promyelocytes are significantly larger than myeloblasts They demonstrate a lower nuclear cytoplasmic ratio and occasional nucleoli The cytoplasm has a deep blue colouration in comparison to the paler myeloblast, and contains primary granules Primary granules are retained in nuetrophils, eosinophils and basophils The nucleus of the promyelocyte is also slightly indented Promyelocytes should not be present in the peripheral blood

Answer 14

An increase in the number of cells in the absence of other intermediate stages of maturation is associated with a subtype of AML called acute promyelocytic leukaemia

Answer 15

Myelocytes are smaller than promyelocytes Show evidence of chromatin clumping and nucleoli are no longer visible Secondary granules are present throughout the cytoplasm Secondary granules are so called because they are produced after the primary granules and possess a cell-specific function The myeloblast, promyelocyte and myelocyte stages of maturation are all mitotic stages At each stage, daughter cells are produced, so the larger the number of cells entering the cell cycle at the myeloblast stage, the greater the number of cells will become effector (mature) cells

Answer 16

Contain distinctive primary and secondary granules The nucleocytoplasmic ratio is much lower in metamyelocytes and the nucleus is kidney shaped As the metamyelocyte matures, the nucleus becomes increasingly curved so that it almost resembles a horseshoe The cells are now called "band forms" Finally the nucleus becomes segmented and the mature features of the effector cell, the neutrophil, eosinophil or basophil, become apparent

Answer 17

Very high levels of ionising radiation can be caused by: - Atomic bomb explosions e.g. Horoshima - Nuclear power plant accidents e.g. Chernobyl - Medical treatment e.g. Xrays or CT scan - Radiation therapy for cancer (can increase risk of leukaemia e.g. AML) - receiving radioactive iodine therapy for hyperthyroidism or thyroid cancer -> risk of AML 80% higher, 3.5 times more likely to get CML - air and spacetravel -> the leukaemia risk from space travel due to galactic cosmic rays - radioactive materials -> uranium mining as an occupation increase risk of leukaemia - exposure to radioactive material in tobacco products, which pick up these materials in the soil where they are grown

Answer 18

Working with benzene and formaldehyde are at greater risk of developing leukaemia Increased incidence of malignancy in petrol pump attendants Smokers at higher risk of AML Herbicides and pesticides associated with MDS and AML Radon increases risk of CLL

Answer 19

Risk begins to increase two years after treatment and peaks between five and 10 years after treatment Commonly associated with monosomies (when one Ch lacks its homolog) or deletion of Ch7 and 5

Answer 20

MDS (up to 30% develop AML) Severe Aplastic, Anaemia (BM failure) associated with treatment with immunosuppressive agents (Cylosporin A, Cyclophosphamide and/or G-CSF therapy) have a 20% risk of developing MDS or AML PNH also risk of developing MDS or AML Inflammatory Bowel Disease Autoimmune disorders (RA, SLE, Coeliac, pernicious anaemia)

Answer 21

20% increase of AML M7 Approximately 10% have TAM at birth, 30% will develop AML M7 within 5 years Although TAM can closely mimic leukaemia, most neonates who survive 4 to 8 weeks show complete remission Trisomy 21? Role in leukamogenesis - but notably TAM and AML M7 share somatic mutations of GATA 1 transcription factor in almost all cases GATA1 is a lineage-restricted TF that is central to the correct differentiation, proliferation and apopotosis or erythroid and megakaryoctic cells - so mutation can assist in AML occurring However, studies have shown that truncating mutations in GATA1 are not sufficient to cause leukaemia in people, and have suggested that trisomy 21 may have an essential leukaemogenic function in cooperation with GATA1 mutation in DS

Answer 22

Infection with T-cell leukaemia virus (HTLV-1) increases the risk of leukaemia No direct evidence of viral genomic inclusions within leukaemia cells Maternal infection a risk factor Breast feeding and attendance at a creche during first year of life decreases risk No evidence of vaccinations causing an increased risk Seasonal variations noted - even in Ireland! September/October highest Prevalence of childhood leukaemia noted? Back to school Virus involvement in 15% of all cancer (notably Burkitt lymphoma, cervical cancer etc) Small peaks in ALL have been associated with outbreaks of influenza Increase in ALL incidence post strep throat -> possible bacterial link

Answer 23

ALL does not appear to run in families, with the exception being identical twins People who have a first-degree family member who has had CLL (parent, sibling or child) have more than twice the risk of developing the disease themselves Siblings of children with AML have up to a four times higher risk of developing the disease, with the risk in identical twins being around 20 percent. In contrast, children who have a parent who has adult-onset leukaemia do not appear to be at a higher risk Family history has no role in CML

Haematological Malignancy 1 Flashcards

(47 cards)