Haematological Malignancies

Question 1

what are haematological malignancies?

Answer 1

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

Question 2

what is leukaemia?

Answer 2

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

Question 3

what are lymphomas?

Answer 3

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

Question 4

what are the systemic symptoms of leukaemias?

Answer 4

weight loss, fever, frequent infections

Question 5

what are the lung related symptoms of leukaemia?

Answer 5

easy shortness of breath

Question 6

what are the muscular related symptoms of leukaemia?

Answer 6

muscle weakness

Question 7

what are the bone and joint related symptoms of leukaemia?

Answer 7

pain or tenderness

Question 8

what is the physiological symptoms of leukaemia?

Answer 8

fatigue or loss of appetite

Question 9

what are the skin related symptoms of leukaemia?

Answer 9

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

Question 10

what is the survival rate of leukaemia?

Answer 10

46%

Question 11

what is acute leukaemia?

Answer 11

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

Question 12

what is chronic leukaemia?

Answer 12

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

Question 13

what causes the build up of malignant cells?

Answer 13

failure of cells to undergo apoptosis

Question 14

what are the 2 types of acute leukaemia?

Answer 14

acute lymphoblastic leukaemia and acute myelogenous leukaemia

Question 15

what are the 2 types of chronic leukaemia?

Answer 15

chronic lymphocytic and chronic myelogenous

Question 16

what are the features of acute lymphoblastic leukaemia?

Answer 16

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

Question 17

what are the features of chronic lymphocytic leukaemia?

Answer 17

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

Question 18

what are the features of acute myelogenous leukaemia?

Answer 18

onogenic mutations, immature myeloid lineage cells, anaemia symptoms

Question 19

what are the features of chronic myelogenous leukaemia?

Answer 19

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

Question 20

what type of leukaemia is most common in children?

Answer 20

acute lymphoblastic

Question 21

what kind of leukaemia is twice as common in men?

Answer 21

chronic lymphocytic

Question 22

what kind of leukaemia is caused by the philadelphia chromosome?

Answer 22

chronic myelogenous

Question 23

what causes the symptoms of leukaemia?

Answer 23

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

Question 24

what are the risk factors of leukaemia?

Answer 24

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

Question 25

what gene forms on the philedelphia chromosome?

Answer 25

BCR-ABL1

Question 26

how does the philadelphia chromosome form?

Answer 26

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

Question 27

what does the philadelphia gene code for?

Answer 27

tyrosine kinase

Question 28

what does the tyrosine kinase gene cause?

Answer 28

myeloid white blood cell production

Question 29

how does a bone marrow transplant work?

Answer 29

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

Question 30

what is targeted therapy?

Answer 30

targeting the cancers specific genes or proteins

Question 31

what is biological therapy?

Answer 31

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

Question 32

what are myeloproliferative neoplasms?

Answer 32

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

Question 33

what are the types of MPN's?

Answer 33

ET, PV, myelofibrosis

Question 34

what is ET?

Answer 34

blood disorder causing a high number of platelets to form

Question 35

what are most symptoms of ET caused by?

Answer 35

blood clots forming due to bleeding problems

Question 36

why do people with ET have blood problems?

Answer 36

because the platelets are not fully mature and dont function properly

Question 37

what can blood clots in areas of the body cause?

Answer 37

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

Question 38

what is erythromelalgia?

Answer 38

redness, throbbing and burning in the hands and feet

Question 39

what is erythromelalgia a symptom of?

Answer 39

ET

Question 40

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

Answer 40

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

Question 41

what is polycythaemia vera?

Answer 41

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

Question 42

what is PV associated with?

Answer 42

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

Question 43

what mutation is also present in PV?

Answer 43

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

Question 44

what are the three groups of symptoms caused by PV?

Answer 44

cytokines. hyperviscosity and splenomegaly

Question 45

what are the symptoms of increased cytokines?

Answer 45

tiredness, itching, muscle ache, night sweats

Question 46

what are the symptoms of hyper viscosity of the blood?

Answer 46

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

Question 47

what are the symptoms of splenomegaly?

Answer 47

fullness, abdominal discomfort

Question 48

what is myelofibrosis?

Answer 48

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

Question 49

what is primary myelofibrosis also called?

Answer 49

chronic idiopathic myelofibrisis

Question 50

what is primary myelofibrosis?

Answer 50

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

Question 51

what is secondary myelofibrosis?

Answer 51

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

Question 52

what is a patient likely to develop when having myelofibrosis?

Answer 52

acute myeloid leukaemia

Question 53

how does primary myelofibrosis begin?

Answer 53

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

Question 54

what does over stimulation of fibroblasts cause in myelofibrosis?

Answer 54

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

Question 55

what happens due to myelofibrosis destroying healthy bone marrow?

Answer 55

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

Question 56

what are the symptoms of myelofibrosis?

Answer 56

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

Question 57

what is multiple myeloma?

Answer 57

cancer of the plasma cells

Question 58

what environmental exposures may cause MM?

Answer 58

herbicides, pesticides, benzene, hair dyes, radiation

Question 59

what happens to the plasma cells in MM?

Answer 59

undergo malignant transformation and on;y produce monoclonal antibodies

Question 60

what is plasmacytomas?

Answer 60

a build up in the bone marrow of the plasma proteins

Question 61

what do plasmacytomas do?

Answer 61

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

Question 62

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

Answer 62

causes bone pain, breaks or fractures

Question 63

what functions of MM causes the symptoms?

Answer 63

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

Question 64

how can MM be treated?

Answer 64

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

Question 65

what drugs modulate the immune system?

Answer 65

dexamethasone and lenalidomide

Question 66

what does pamidronic acid do?

Answer 66

inhibits bone resorption

Question 67

what does melphalan do?

Answer 67

is toxic to myeloma cells

Question 68

what is daratumumab?

Answer 68

antibody that kills cells with CD38 on their surface

Question 69

what does elotuzumab do?

Answer 69

activates NK cells to destroy MM cells