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Flashcards in haematological malignancies Deck (81):
1

what conditions consist of myeloproliferative disorders?

polycythaemia vera, CML, essential thrombocythaemia, myelofibrosis,

2

what causes myeloproliferative disorders?

In these disorders, there is uncontrolled clonal proliferation of one or more of the cell lines in the bone marrow, namely erythroid, myeloid and megakaryocyte lines.

3

what is the Ann Arbor staging system for hodgkin lymphoma?

1. Disease is confined to one lymph node group
2. Disease is in 2 adjacent lymph node groups
3. Present in lymphoid tissues on both sides of the diaphragm
4. Present in a non lymph node/extranodal organ (e.g. brain, bone marrow

4

what are the "B" symptoms?

night sweats and 10% weight loss and fever

5

what is the treatment of Hodgkin Lymphoma?

A- Adriamycin
B- Bleomycin
V- Vinblastince
D- Dacarbazine
All given IV
= ABVD treatment
+/- GM CSF subcutaneous injection

6

What is the treatment for Non Hodgkin lymphoma?

Cyclophosphamide IV
Hydroxydornorubison (adriomycin) IV
Oncovan (mitotic spindle inhibitor) IV
Prednisolone (PO)
= CHOP
+/- rituximab

7

what is the difference between a leukaemia and a lymphoma?

leukaemias affect the bone marrow as well. Lymphoma only affects a lymphoid tissue outside the bone marrow

8

what are the signs of bone marrow failure?

anaemia, bleeding and infection

9

what is the mutation associated with CML?

BCR-ABL mutation. This BCR ABL fusion gene codes for a 210 kDa protein with tyrosine kinase activity, which plays a causative role in the disease as an oncogene influencing cellular proliferation, differentiation and survival.

10

what is the most common leukaemia? What mutation is it associated with?

CLL. BCL2 dysregulation leading to impaired Caspase path towards apoptosis. This causes cell survival

11

which is more common- B or T cell lymphoma?

B cell lymphomas

12

why do we give G-CSF for CHOP-R treatment?

CHOP-R treatment is myelosuppressive

13

what is light chain restriction a marker of?

a marker of malignancy

14

What does MGUS stand for? what is it a risk factor for?

Monoclonal gammopathy of undermined significance. Higher chance of progressing to myeloma

15

how do we investigate multiple myeloma?

Bone marrow aspirate- film + flow cytometry, CRAB, urine test, FBE, U and E, B-2 microglobulin, serum albumin, LDH, serum and urine protein electrophoresis and measurement of free light chains. Do imaging for lytic bone lesions (a skeletal survey of spine, skull, long bones + spinal MRI).

CRAB
1. Hypercalcemia
2. Renal insufficiency
3. Anaemia
4. Lytic bone lesions

16

what do you think of a normal protein level with a low albumin?

multiple myeloma

17

Traditional chemotherapy for multiple myeloma?

Alkylating agents (cyclophosphamide)
Steroids
Vincristine

18

Supportive therapy for myeloma?

1. Plasmapheresis, dialysis, radiotherapy/surgery
2. Bisphosphonate- (prevent orthopaedic complications)
3. Infection control (bc they may be immune suppressed)
4. Pain management (crush fractures/plasmacytomas)
5. Psychological/supportive management

19

what protein do we look for in the urine for Multiple myeloma?

Bence Jones protein

20

does everyone with paraprotein have multiple myeloma?

no. Unless they demonstrate CRAB features

21

what is a lymphoma?

malignancy derived from lymphoid tissue

22

Clinical aggressiveness of Burkitt Lymphoma?

High grade. Need to come into hospital and need treatment ASAP

23

what is the clinical aggressiveness of follicular lymphoma?

low grade

24

what directs prognosis in Non hodgkin lymphoma?

the grade (histological aggressiveness)

25

what symptoms might make you think of a lymphoma as a ddx?

palpable pathological lymphadenopathy (firm, rubbery, non-tender ) – rate of growth depends on biological aggressiveness of subtype
• dyspnoea and cough with subsequent demonstration of a mediastinal mass
• abdominal swelling and/or progressive discomfort( although non-specific )
• PUO and night sweats

26

what determines the prognosis of a hodgkin lymphoma?

the stage or subtype determines the prognosis

27

in lymphoproliferative disorders like NHL what marker is always raised?

LDH

28

what kind of lymphoma causes a mediastinal mass? what might you see on blood film?

T cell lymphoma
-on blood film may see eosinophillia

29

what is something you want to ask yourself when you have a patient with leukaemia?

Acute or chronic?
Myeloid or lymphoid?

30

Secondary acute leukaemia is caused by what?

previous cytotoxic therapy is the most important reason
- exposure to ionising radiation/chemicals

31

Kids with down syndrome is more susceptible to which type of leukaemia

AML

32

clinical presentation of acute leukaemia

1. anaemia- lethargy, chest pain, pallor
2. neutropenia- fevers/rigors/infections
3. thrombocytopenia- bruising and bleeding
4. B symptoms- fevers, sweats, weight loss
5. Less commonly- lymphadenopathy, hepatosplenomegaly, hyperleucocytosis

33

key ix for acute leukaemia

1. FBE and FILM
2. Bone marrow biopsy- is this disease lymphoid or myeloid?
3. immunophenotyping- flow cytometry for cell surface markers
4. cytogenetics
5. molecular studies

34

how can we tell if the leukaemia is lymphoid or myeloid in origin?

bone marrow biopsy and flow cytometry

35

if you see blasts in the peripheral blood-

CALL A HAEMATOLOGIST

36

what defines acute leukaemia?

if you see infiltration of malignant cells= blasts greater than 20% nucleated cells on BONE marrow biopsy

37

auer rods indicate?

myeloid malignancy

38

implication of CD34 from flow cytometry

marker of immature cells- indicates leukaemia

39

What do we use immunophenotyping/flow cytometry for when we ix for suspected leukaemia?

helps to determine the type of acute leukaemia by the pattern of surface antigen expression

40

what indicates good response to standard chemo for AML?

cytogenetics and molecular findings, and whether there is residual disease after therapy

41

ALL prognosis child vs adult?

child ALL generally has better prognosis than adult ALL. mainly due to implementation of good intensive paediatric protocols

42

Key medical treatment for ALL

L-Asparaginase

43

main features of APML at presentation

bruising, bleeding
Signs of DIC, low fibrinogen, thrombocytopenia

44

key histological cell of APML from bone marrow biopsy

Faggot cells- cytoplasm full of auer cells (promyelocyte)

45

cytogenetics fo APML?

15:17 translocation-RARa and PML genes

46

APML acute management?

1. Correct coagulopathy, with platelets, cyroprecipitate, and fibrinogen as needed
2. Transfer to hospital who has ATRA (all trans retinoid acid) to induce promyelocytic differentiation
3. Add arsenic

47

Philadelphian 9:22 chromosomal translocation associated with which leukaemias?

ALL and CML

48

3 stages of chronic myeloid leukaemia?

1. chronic
2. accelerated (if remains untreated)
3. blast (untreated)--> acute leukaemia

49

what would you see under the microscope for a blood film of CML?

eosinophillia, basophilia,
full spectrum of myeloid development

50

management of CML?

Tyrosine kinase inhibitor
- imatinib
Selectively kill CML cells, and has eliminated the need for allograft.

51

how does imatinib work?

blocks ATP binding site for BCR ABL protein reducing signalling for cell proliferation

52

commonest adult leukaemia?

CLL

53

What symptoms constitute advanced CLL

lymphadenopathy, splenomegaly
B cells
autoimmune anaemia
B symptoms
hypogammaglobulinemia and recurrent infections

54

CLL morphology- blood and bone marrow?

small mature, lymphocytosis with smear cells

55

What are the cell markers we are looking for on flow cytometry for CLL?

CD5-CD19

56

what is the significance of 17 p deletion in CLL?

loss of TSG p53 indicates poor prognosis

57

how might we ix cytogenetics of CLL?

FISH (fluorescent in situ hybridisation)

58

when do we start treating CLL?

when the patient is symptomatic.

59

prognosis of CLL?

can either be indolent disease or an aggressive disease if p53 lost due to 17p deletion

60

what is the only curable option of CLL

allograft

61

how do we manage symptomatic CLL patients?

1. chemotherapy inc. rituximab
2. monoclonal antibodies
3. allograft

62

which leukaemia are associated with splenomegaly

any leukaemias with a monocytic or monoblastic origin

63

describe a reed steinberg cell. what is it associated with?

binuclear cells with prominent eosinophillic nucleolus

Diagnostic of hodgkin lymphoma

64

what is the most common type of monoclonal M protein in multiple myeloma?

IgG

65

define gammopathy

overproduction of one or more immunoglobulins

66

what is the clinical criteria for multiple myeloma

Diagnosis= increased malignant plasma cells in the bone marrow + detection of serum/urinary M protein + skeletal lytic lesions (only 2 needed)

67

where do we normally see malignant plasma cells for multiple myeloma?

in the bone marrow

68

what are bence jones proteins and where do we see them?

free light chains in the urine.
in light chain only multiple myeloma

69

what is a paraprotein?

monoclonal protein--> immunoglobulin.

70

why do we get lytic lesions in multiple myeloma?

malignant plasma cells produce cytokines --> stimulate OC and results in net bone resorption --> lytic lesions

71

why do we get renal failure from multiple myeloma?

paraprotein deposition, hypercalcaemia, NSAIDs, amyloid

72

COMPLICATIONS OF MULTIPLE MYELOMA

Renal impairment
Hypercalcemia
Spinal cord compression --> treated with dexamethasone
Hyperviscosity

73

symptoms of multiple myeloma

Bone pain
Symptoms of anaemia
Recurrent infections
Symptoms of hypercalcaemia (renal stones)
Symptoms of renal failure

OR asymptomatic.

74

treatment of symptomatic CLL?

FCR chemotherapy regime

Fludarabine
Cyclophosphamide
Rituximab

OR allograft

75

what is the chemotherapy drugs for AML in fit patients (i.e. the patients that are not going to be palliated)

Cytarabine
Idarubicin

76

what is a plasmacytoma

clonal proliferation of plasma cell

77

cell marker for myeloma

CD138

78

what can MGUS progress to?

myeloma, lymphoproliferative disorder, amyloidosis

79

difference between plasmacytoma and myeloma?

plasmacytoma- solitary
myeloma- multifocal

80

what is primary amyloidosis associated with?

associated with a plasma cell or lymphoplasmatcytic lesion including MGUS

81

60-year-old man with chronic myeloid leukaemia (CML) with 7 cm splenomegaly associated with good or poor prognosis?

poor prognosis due to splenomegaly involvement