Malignancy

Question 1

what is the best way to identify mature non-lymphoid cells?

Answer 1

morphology

Question 2

malignant haemopoiesis is characterised by what?

Answer 2

inc numbers of abnorml and dysfunctional cells

Question 3

what malignancy is characterised by proliferation of abnormal proegnitors with block in differentiation/maturation?

Answer 3

acute luekaemia

Question 4

what malignancy is characterised by proliferation of abnormal progentiors but NO block in differentiation/maturation?

Answer 4

chronic myeloproliferative disorders

Question 5

what is meant by ‘clone’ with regards to driver mutations?

Answer 5

population of cells dervied from one ‘parent cell’- the daughter cells share genetic marker of parent (they can diversify but will always share genetic backbone)

Question 6

normal haemopoeisis is poly/monoclonal?

Answer 6

normal- polyclonal

malignant- usually monoclonal

Question 7

what are the two types of haematological malignancy?

Answer 7

lineage (myeloid or lymphoid)

anatomical site (blood/lymph)

Question 8

blood involvement is which kind of malignancy?

Answer 8

blood involvment = leukaemia

Question 9

lymph node involvemnt is which kind of malignancy?

Answer 9

lymphoma

Question 10

which malignancy involved the marrow?

Answer 10

myeloma → plasma cell malignancy in marrow

Question 11

which malignancy can involve blood and lymph nodes?

Answer 11

Chronic Lymphocytic Leukaemia (CLL)

Question 12

if somone presents with lymph nodes what should you always check?

Answer 12

bloods

Question 13

define acute leukaemia

Answer 13

excess of ‘blasts’ >20% in either the peripheral blood or bone marrow → decrease of normal haemopoetic reserve i.e. low platelts, low neutrophils

Question 14

who is acute myeloid leukaemia most common in?

Answer 14

more common in elderly

Question 15

how is AML investigated?

Answer 15

blood count

coag screen

bone marrow aspirate

Question 16

auer rods are typically seen in which malignancy?

Answer 16

Acute Myeloid Leukaemia (AML)

Question 17

how is AML treated?

Answer 17

intensive chemo for multiple cycles

Question 18

during chemo for AML risk of infection increases. if neutropenic fever suspected what should be done?

Answer 18

cover with broad spectrum antibiotic particulary gram -ve

Question 19

what acute leukaemia tends to occur mainly in children?

Answer 19

acute lymphoblastic leukaemia ALL

Question 20

ALL is a disease of which cells?

Answer 20

disease of primitive lymphoid cells (lymphoblasts)

Question 21

ALL has a tendancy to involve which body system?

Answer 21

CNS

(in males can get into testes)

Question 22

why is bone pain common in ALL?

Answer 22

rapid marrow expansion causes pain

Question 23

how is ALL treated?

Answer 23

chemotherapy followed by maintainence therapy

Question 24

what are the problems of marrow suppression?

Answer 24

anaemia

neutropenia

thrombocytopenia

Question 25

which gram stain is higher risk in neutropenic patients?

Answer 25

gram -ve higher risk (can cause life threatening spesis within hours)

Question 26

which responds better to treatment- low or high grade Non-hodgkins lymphoma?

Answer 26

high grade (grows more rapidly but repsonds better to treatment than slow growing low grade tumours)

Question 27

how is a diagnosis of Non-Hodgkins Lymphoma confirmed?

Answer 27

biopsy

Question 28

what protein is found on Hodgkins lymphoma?

Answer 28

CD30

Question 29

what is the treatment for Hodgkins?

Answer 29

brentuximab vedotin

Question 30

who tends to get hodgkins and who gets NHL?

Answer 30

young- Hodgkins older- Non-Hodgkins

Question 31

which is more common Hodgkins or Non-Hodgkins?

Answer 31

Non-Hodgkins

Question 32

what is the abnormal cell found in Hodgkins Lymphoma?

Answer 32

Reed-sternberg cells (abnormal type of B lymphocytes)

Question 33

which has a better prognosis, hodgkins or non-hodgkins?

Answer 33

Hodgkins- generally easier to treat

Question 34

what is a malignancy of plasma cells?

Answer 34

myeloma

Question 35

what are the direct tumour cell effects of myeloma on the body?

Answer 35

bone lesions bone pain inc in calcium marrwo failure

Question 36

what are the paraprotein mediated effects of myeloma on the body?

Answer 36

renal failure immune suppression amyloid

Question 37

how is myeloma classified?

Answer 37

type of antibody produced

Question 38

what is the benign form of myeloma?

Answer 38

MGUS (premalignant) (monoclonal gammopathy of unknown significance)

Question 39

which steps in the cell cycle are involved in differentiation and in proliferation?

Answer 39

differentiate- G0 and G1 proliferate- S, G2, M

Question 40

what the two types of treatments for tumours with regards to the cell cycle?

Answer 40

cell cycle specific (tumour specific) non-cell cycle specific (non-tumour specific)

Question 41

duration of dose rather than dose more important in specific or non-specific treamtent?

Answer 41

specific In non-secific cumalitive dose more importnant rather than duration

Question 42

tyrosine kinase inhibitors can be used in the treatment of which malignancy?

Answer 42

chronic myeloid leukamia

Question 43

give an example of a tyrosine kinase inhibitor

Answer 43

imatinib

Question 44

in what disease is philadelphia chromosome seen?

Answer 44

Chronic myeloid leukaemia

Question 45

what are the two subtypes of Chronic Myeloproliferative DIsorders?

Answer 45

BRC-ABL1 positive BRC-ABL1 negative

Question 46

what is seen in CML?

Answer 46

proliferation if myeloid cells- granulocytes

Question 47

other than myeloproliferative disorders what else can cause a high Hb?

Answer 47

chronic hypoxia i.e. COPD/sleep apnoea is a common reason for eleavted Hb

Question 48

bloods can look identical in myeloproliferative disorders vs reactive but what differentiates the two?

Answer 48

eoisinophilia and basophilia in MPD reactive will have an explanation i.e. empyema

Question 49

what can be used to treat CML?

Answer 49

tyrosine kinase inhibitors i.e. imatinib

Question 50

what are the three forms of BRC-ABL1 negative myeloproliferative disorders?

Answer 50

Polycythameia Rubra Vera Idiopathic myelofibrosis essential Thrombocytopenia

Question 51

what are the two forms of Polycythaemia Rubra Vera?

Answer 51

primary or secondary

Question 52

what are some signs of polycythaemia?

Answer 52

headache, fatigue itchy- especially when in bath/shower plethroic (redness)

Question 53

what mutation is seen in \>95% of people with Polycythaemia?

Answer 53

JKA2 mutation

Question 54

what is the treamtent for polycythaemia rubra vera?

Answer 54

venesect to Hc\<0.45 aspirin cytotoxic oral chemo i.e. hydroxycarbamide

Question 55

what is essential thrombocytopenia?

Answer 55

uncontrolled production of abnormal platelets?

Question 56

what does essential thrombocytopenia result in?

Answer 56

thrombosis at very high levels can cause bleeding \>1500 (platelts can bind to VWF so circulatory levels decrease)

Question 57

why is platelet count following surgery less realible in diagnosing throbocytopenia?

Answer 57

inc in platelets following surgery is normal- way of dealign with massive haemostatic challenge

Question 58

the JAK 2 gene is found in 50% patients with essential thrombovytopenia. in those that dont what other gene mutation will be present?

Answer 58

CALR- calreticulin MPL mutation also

Question 59

how is essential thrombocytopenia treated?

Answer 59

aspirin cytoreductive therapy to control proliferation i.e. hydroxycarbamide

Question 60

tear drop shaped RBC's can be seen in which mhyeloproliferatve disorder?

Answer 60

myelofibrosis

Question 61

in additon to tear drop RBC's what else is seen on blood film in myelofibrosis?

Answer 61

leucoerythroblastic | (nucelated red cells and myelocytes)

Question 62

how is myelofibrosis treated?

Answer 62

supportive: blood transfusion, platelets allogenic stem cell transplantation in select few JAK2 inhibitors