Haematological Malignancies Flashcards

1
Q

what are haematological malignancies?

A

cancers that begin in cells from the bone marrow and are then subdivided into which type of blood cell that is affected

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2
Q

what is leukaemia?

A

presence of malignant white blood cells within peripheral blood or bone marrow, causes suppression of production of normal blood cells in red bone marrow

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3
Q

what are lymphomas?

A

cancer of the lymphoid origin that spill out into the blood and bone marrow

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4
Q

what are the systemic symptoms of leukaemias?

A

weight loss, fever, frequent infections

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5
Q

what are the lung related symptoms of leukaemia?

A

easy shortness of breath

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6
Q

what are the muscular related symptoms of leukaemia?

A

muscle weakness

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7
Q

what are the bone and joint related symptoms of leukaemia?

A

pain or tenderness

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8
Q

what is the physiological symptoms of leukaemia?

A

fatigue or loss of appetite

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9
Q

what are the skin related symptoms of leukaemia?

A

night sweats, easy bleeding and bruising, purplish patches or spots

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10
Q

what is the survival rate of leukaemia?

A

46%

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11
Q

what is acute leukaemia?

A

increased numbers of immature immune cells called blasts which make up 20% of nucleated cells in the bone marrow

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12
Q

what is chronic leukaemia?

A

less aggressive and have blasts below 20% of nucleated cells in the bone marrow

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13
Q

what causes the build up of malignant cells?

A

failure of cells to undergo apoptosis

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14
Q

what are the 2 types of acute leukaemia?

A

acute lymphoblastic leukaemia and acute myelogenous leukaemia

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15
Q

what are the 2 types of chronic leukaemia?

A

chronic lymphocytic and chronic myelogenous

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16
Q

what are the features of acute lymphoblastic leukaemia?

A

short onset, derived from lymphoid stem cells, common in children, immature B and T cells

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17
Q

what are the features of chronic lymphocytic leukaemia?

A

common in adults 55+, caused by chromosomal deletion, twice as common in men

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18
Q

what are the features of acute myelogenous leukaemia?

A

onogenic mutations, immature myeloid lineage cells, anaemia symptoms

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19
Q

what are the features of chronic myelogenous leukaemia?

A

prolonged onset, from myeloid stem cells, in adults, philadelphia chromosome

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20
Q

what type of leukaemia is most common in children?

A

acute lymphoblastic

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21
Q

what kind of leukaemia is twice as common in men?

A

chronic lymphocytic

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22
Q

what kind of leukaemia is caused by the philadelphia chromosome?

A

chronic myelogenous

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23
Q

what causes the symptoms of leukaemia?

A

dysregulated stem cell production, so many WBC produced causing less healthy blood cells to be made which causes the symptoms

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24
Q

what are the risk factors of leukaemia?

A

radiation, smoking, benzene, genetic disorders, chemotherapy, some viruses

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25
Q

what gene forms on the philedelphia chromosome?

A

BCR-ABL1

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26
Q

how does the philadelphia chromosome form?

A

the gene ABL1 on chromosome 9 gets stuck on BCR gene on chromosome 22

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27
Q

what does the philadelphia gene code for?

A

tyrosine kinase

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28
Q

what does the tyrosine kinase gene cause?

A

myeloid white blood cell production

29
Q

how does a bone marrow transplant work?

A

patient RBM destroyed with radiation and transplant marrow migrates to red bone marrow cavities and multiplies

30
Q

what is targeted therapy?

A

targeting the cancers specific genes or proteins

31
Q

what is biological therapy?

A

the use of special medicines to improve the body’s natural defences against cancer e.g. CAR’s on T cells

32
Q

what are myeloproliferative neoplasms?

A

group of rare disorders of the bone marrow that cause an increase in the number of blood cells

33
Q

what are the types of MPN’s?

A

ET, PV, myelofibrosis

34
Q

what is ET?

A

blood disorder causing a high number of platelets to form

35
Q

what are most symptoms of ET caused by?

A

blood clots forming due to bleeding problems

36
Q

why do people with ET have blood problems?

A

because the platelets are not fully mature and dont function properly

37
Q

what can blood clots in areas of the body cause?

A

headaches, dizziness, chest pain, fainting and tingling in hands and feet

38
Q

what is erythromelalgia?

A

redness, throbbing and burning in the hands and feet

39
Q

what is erythromelalgia a symptom of?

A

ET

40
Q

what mutation can be caused by ET? what does it cause?

A

mutation in the JAK2 enzyme affecting the receptor to TPO so cells multiply without stimulation

41
Q

what is polycythaemia vera?

A

slow growing blood cancer that causes too many red blood cells to be made, thickening the blood

42
Q

what is PV associated with?

A

low levels of EPO due to negative feedback from the many red blood cells being produced

43
Q

what mutation is also present in PV?

A

JAK2 so changes the signalling pathway for EPO so cells proliferate independently

44
Q

what are the three groups of symptoms caused by PV?

A

cytokines. hyperviscosity and splenomegaly

45
Q

what are the symptoms of increased cytokines?

A

tiredness, itching, muscle ache, night sweats

46
Q

what are the symptoms of hyper viscosity of the blood?

A

headaches, dizziness, skin redness, vision issues, ear rining

47
Q

what are the symptoms of splenomegaly?

A

fullness, abdominal discomfort

48
Q

what is myelofibrosis?

A

fibrous scar tissue builds up inside the bone marrow and cells arent properly made

49
Q

what is primary myelofibrosis also called?

A

chronic idiopathic myelofibrisis

50
Q

what is primary myelofibrosis?

A

when it arises in patients with no history of bone marrow problems

51
Q

what is secondary myelofibrosis?

A

when the patient already has bone marrow conditions such as PV or ET

52
Q

what is a patient likely to develop when having myelofibrosis?

A

acute myeloid leukaemia

53
Q

how does primary myelofibrosis begin?

A

increased expression of cytokines from mutates HSC’s and megakaryocytes over stimulate fibroblasts

54
Q

what does over stimulation of fibroblasts cause in myelofibrosis?

A

overgrowth of thick, coarse fibres in the bone marrow which gradually replace the bone marrow tissue

55
Q

what happens due to myelofibrosis destroying healthy bone marrow?

A

prevention of production of adequate numbers of healthy blood cells, causing anaemia and production of blood cells in liver and spleen

56
Q

what are the symptoms of myelofibrosis?

A

fatigue, bone pain, fever, night sweats, early satiety

57
Q

what is multiple myeloma?

A

cancer of the plasma cells

58
Q

what environmental exposures may cause MM?

A

herbicides, pesticides, benzene, hair dyes, radiation

59
Q

what happens to the plasma cells in MM?

A

undergo malignant transformation and on;y produce monoclonal antibodies

60
Q

what is plasmacytomas?

A

a build up in the bone marrow of the plasma proteins

61
Q

what do plasmacytomas do?

A

erode the hard, outer shell or cortex of the bone surrounding the marrow, thinning them

62
Q

what can happen to the bone as a result of plasmacytomas?

A

causes bone pain, breaks or fractures

63
Q

what functions of MM causes the symptoms?

A

the M protein thickens the blood and gets deposited in organs and interfere with the kidneys, nerves and the immune system

64
Q

how can MM be treated?

A

chemotherapy, radiation, stem cell transplants, surgery, drugs that modulate the immune system

65
Q

what drugs modulate the immune system?

A

dexamethasone and lenalidomide

66
Q

what does pamidronic acid do?

A

inhibits bone resorption

67
Q

what does melphalan do?

A

is toxic to myeloma cells

68
Q

what is daratumumab?

A

antibody that kills cells with CD38 on their surface

69
Q

what does elotuzumab do?

A

activates NK cells to destroy MM cells