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Year 3 > Week 20 - Leukaemia, Lymphoma, Myeloma > Flashcards

Week 20 - Leukaemia, Lymphoma, Myeloma Flashcards

1
Q

what is leukaemia

A

a cancer of a particular line of stem cells in the bone marrow, causing unregulated production of a specific type of blood cell

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2
Q

what are the four main types of leukaemia

A
  • acute myeloid leukaemia
  • acute lymphoblastic leukaemia
  • chronic myeloid leukaemia
  • chronic lymphocytic leukaemia
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3
Q

how are the types of leukaemia classed

A

depending on how rapidly they progress and the cell line that is affected

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4
Q

what is a more rare, less likely to appear in exams form of leukaemia

A

acute promyelocytic leukaemia

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5
Q

what leukaemia most commonly affects children under 5 years old

A

acute lymphoblastic leukaemia

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6
Q

which leukaemia is associated with Down’s syndrome and children

A

ALL

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7
Q

what leukaemia is associated with warm haemolytic anaemia, Richter’s transformation and smudge cells

A

CLL

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8
Q

how many phases does CML have and what is it associated with

A

has three phases, including a long chronic phase, and is associated with the Philidelphia chromosome

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9
Q

what may AML result in

A

a transformation from a myeloproliferative disorder and is associated with Auer rods

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10
Q

what does a genetic mutation in one of the precursor cells in the bone marrow lead to

A

excessive production of a single type of abnormal white blood cell

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11
Q

what can the excessive production of a single type of cells do to other cell lines

A

can suppress the other cell lines, causing the underproduction of different cell types.

this results in pancytopenia, which is a combination of low red blood cells , white blood cells and platelets

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12
Q

what are the potential presenting features for leukaemia

A

Fatigue
Fever
Pallor due to anaemia
Petechiae or bruising due to thrombocytopenia
Abnormal bleeding
Lymphadenopathy
Hepatosplenomegaly
Failure to thrive (children)

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13
Q

what is one key presenting feature of leukaemia

A

bleeding under the skin due to thrombocytopenia

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14
Q

what does bleeding under the skin cause

A

. Bleeding under the skin causes non-blanching lesions.

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15
Q

what are lesions of bleeding under the skin called; there are three names according to the different sizes

A

Petechiae are less than 3 and caused by burst capillaries
Purpura are 3 – 10mm
Ecchymosis is larger than 1cm

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16
Q

what are the top differentials for a non-blanching rash caused by bleeding under the skin

A

Leukaemia
Meningococcal septicaemia
Vasculitis
Henoch-Schönlein purpura (HSP)
Immune thrombocytopenic purpura (ITP)
Thrombotic thrombocytopenic purpura (TTP)
Traumatic or mechanical (e.g., severe vomiting)
Non-accidental injury

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17
Q

what do NICE recommend within 48 hours for patients with suspected leukaemia

A

a full blood count within 48 hours

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18
Q

what is used to look for abnormal cells and inclusions

A

a blood film

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19
Q

what is a very non-specific marker of tissue damage that is often raised in leukaemia

A

Lactate dehydrogenase (LDH)

it can be raised in literal heavy exercise too so it is not helpful for screeing

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20
Q

what is used to analyse the cells in the bone marrow to establish a definitive diagnosis of leukaemia

A

bone marrow biopsy

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21
Q

what are used to help stage the condition of leukaemia

A

CT and PET scans

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22
Q

where is a bone marrow biopsy usually taken from and what does it involve

A

the iliac crest

it involves a local anaesthetic and a specialist needle.

the options are aspiration or trephine. bone marrow aspiration involves taking a liquid sample of cells from within the bone marrow

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23
Q

what does bone marrow trephine involve

A

taking a solid core sample of the bone marrow and provides a better assessment of the cells and structure

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24
Q

what does ALL (acute lymphoblastic leukaemia) affect

A

ALL affects one of the lymphocyte precursor cells causing acute proliferation of a single type of lymphocyte, usually B lymphocyte

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25
Q

what does excessive accumulation of these cells lead to

A

replaces other cell types in the bone marrow leading to pancytopenia

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26
Q

what is chronic lymphocytic leukaemia

A

where there is a slow proliferation of a single type of well-differentiated lymphocyte, yet again usually the B lymphocyte

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27
Q

who does CLL usually affect

A

adults over 60 years of age

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28
Q

what does Richter’s transformation refer to

A

the rare transformation of CLL into high-grade B cell lymphoma

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29
Q

what are smear or smudge cells and what are they particularly associated with

A

Smear or smudge cells are ruptured white blood cells that occur while preparing the blood film when the cells are aged or fragile. They are particularly associated with chronic lymphocytic leukaemia.

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30
Q

what are the three phases of chronic myeloid leukaemia

A

chronic phase
accelerated phase
blast phase

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31
Q

what is the chronic phase of CML

A

often asymptomatic and patients are diagnosed after an incidental finding of a raised white cell count

this phase can last for several years before progressing

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32
Q

what is the accelerated phase of CML

A

occurs when the abnormal blast cells take up a high proportion of the bone marrow and blood cells

in the accelerated phase, patients are more symptomatic and devlop anaemia, thrombocytopenia and immunodeficiency

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33
Q

what phase does the blast phase come after

A

comes after the accelerated phase

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34
Q

what does the blast phase involve

A

an even higher proportion of blast cells in the blood
the blast phase has severe symptoms and pancytopenia,

it is often fatal

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35
Q

CML is associated with which chromosome

A

with the Philadelphia chromosome

this refers to an abnormal chromosome 22 caused by a reciprocal translocation of genetic material between a section of chromosome 9 and chromosome 22

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36
Q

what does this translocation in CML create

A

an abnormal gene sequence called BCR-ABL1 which codes for an abnormal tyrosine kinase enzyme that drives the proliferation of the abnormal cells

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37
Q

what is acute myeloid lukaemia presenation

A

normally presents form middle age onwards

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38
Q

what is AML a result of

A

transformation from a myeloproliferative disorder such as polycythaemia ruby vera or myelofiborisis

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39
Q

what will a blood film and bone marrow biopsy show in AML

A

show a high proportion of blast cells

auer rods in the cytoplasm of blast cells are a characteristc finding in AML

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40
Q

what is leukaemia mainly treated with

A

chemotherapy and targeted therapies, depending on the type and individual features

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41
Q

what are two examples of targeted therapies used in CLL

A

Tyrosine kinase inhibitors (e.g., ibrutinib)

Monoclonal antibodies (e.g., rituximab, which targets B-cells)

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42
Q

what are the other treatment options for leukaemia

A

radiotherapy
bone marrow transplant
surgery

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43
Q

what is tumour lysis syndrome and what does it result from

A

results from chemicals released when cells are destroyed by chemotherapy, resulting in;
- high uric acid
- hyperkalaemia
-high phosphate
- low calcium

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44
Q

what happens when there is high uric acid in the kidneys

A

uric acid can form crystals in the interstitial space and tubules of the kidneys, causing acute kidney injury.

45
Q

what is required in patients before chemotherapy to prevent tumour lysis syndrome

A

Very good hydration and urine output before chemotherapy is required in patients at risk of tumour lysis syndrome. Allopurinol or rasburicase may be used to suppress the uric acid levels.

46
Q

what type of cancer is lymphoma

A

type of cancer that affects the lymphocytes inside the lymphatic system

47
Q

where do cancerous cells proliferate in lymphoma

A

inside the lymph nodes causing the lymph nodes to be abnormally large

48
Q

what are the main two categories of lymphoma

A

Hodgkin’s lymphoma (a specific disease)

Non-Hodgkin’s lymphoma (which includes all other types)

49
Q

what is the most common specific type of lymphoma

A

Hodgkin’s lymphoma

50
Q

what kind of age distribution does Hodgkin’s have and what are the peaks

A

It has a bimodal age distribution with peaks around 20-25 and 80 years.

51
Q

what are the risk factors for Hodkin’s lymphoma

A

HIV
Epstein-Barr virus
Autoimmune conditions, such as rheumatoid arthritis and sarcoidosis
Family history

52
Q

what are the three notable types of non-Hodkin’s lymphoma

A

Diffuse large B cell lymphoma typically presents as a rapidly growing painless mass in older patients

Burkitt lymphoma is particularly associated with Epstein-Barr virus and HIV

MALT lymphoma affects the mucosa-associated lymphoid tissue, usually around the stomach

53
Q

what are the risk factors for non-Hodkin’s lymphoma

A

HIV
Epstein-Barr virus
Helicobacter pylori (H. pylori) infection is associated with MALT lymphoma
Hepatitis B or C infection
Exposure to pesticides
Exposure to trichloroethylene (a chemical with a variety of industrial uses)
Family history

54
Q

what is the key presenting symptom in lymphoma

A

lymphadenopathy

the enlarged lymph node or nodes might be in the neck, axilla, or inguinal region

55
Q

what are the characterisitics of the enlarged lymph nodes

A

non-tender and feel firm or rubbery

56
Q

patient’s with Hodgkin’s lymphoma may experience lymph node pain after what

A

Patients with Hodgkin’s lymphoma may experience lymph node pain after drinking alcohol.

57
Q

what do B symptoms refer to

A

systemic symptoms of lymphoma

58
Q

what are the B symptoms of lymphoma

A

fever
weight loss
night sweats

59
Q

what are the additional non-specific symptoms

A

Fatigue
Itching
Cough
Shortness of breath
Abdominal pain
Recurrent infections

60
Q

what is a critical diagnostic investigation for lymphoma

A

lymph node biopsy

61
Q

what is the characteristic finding on a biopsy for Hodkin’s lymphoma

A

Reed-Sternberg cells

62
Q

what are Reed-sternberg cells

A

large cancerous B lymphocytes with 2 nuclei and prominent nucleoli, giving them a cartoonish appearance of an owl face with large eyes

63
Q

what classification system is used for Hodgkin’s and non-Hodgkin’s

A

the Lugano classification system

64
Q

what does the Lugano classification emphasise and describe the simple version of it

A

It emphasises whether the affected nodes are above or below the diaphragm. A simplified version is:

Stage 1: Confined to one node or group of nodes
Stage 2: In more than one group of nodes but on the same side of the diaphragm (either above or below)
Stage 3: Affects lymph nodes both above and below the diaphragm
Stage 4: Widespread involvement, including non-lymphatic organs, such as the lungs or liver

65
Q

what are the critical treatments for Hodgkin’s lymphoma

A

chemotherapy and radiotherapy

66
Q

what may chemotherapy result in

A

infections, cognitive impairment, secondary cancers, and infertility

67
Q

what does management of non-Hodgkin’s lymphoma involve

A

watchful waiting
chemotherapy
monoclonal antibodies
stem cell transplantation
radiotherapy

68
Q

what type of cancer is myeloma

A

cancer affecting the plasma cells in the bone marrow

69
Q

what are plasma cells

A

B lymphocytes, that produce antibodies

70
Q

what does cancer in a specific type of plasma cell result in the production of

A

large quantities of a specific paraprotein or M protein which is an abnormal antibody or part of an antibody

71
Q

what is multiple myeloma

A

where the myeloma affects multiple bone marrow areas in the body

72
Q

what is MGUS

A

monoclonal gammopathy of undetermined significance

73
Q

what does MGUS involve

A

production of a specific paraprotein without other features of myeloma or cancer

74
Q

what does monoclonal refer to

A

identical copies or clones originating from a single cell.

75
Q

what is MGUS

A

often an incidental finding in an otherwise healthy personb

76
Q

what does smouldering myeloma involve

A

abnormal plasma cells and paraproteins but no organ damage or symptoms

it has a greater risk of progression to myeloma

77
Q

what are B lymphocytes made up of and how are they arranged

A

complex molecules made up of heavy and light chains arranged in a Y shape

78
Q

what are the five types of antibodies produced by B lymphocytes

A

A,G,M,D,E

79
Q

what is myeloma

A

a cancer of a single type of plasma cell, with a genetic mutation that causes them to rapudly and uncontrollably multiply

80
Q

what do plasma cells with a genetic mutation in myeloma produce

A

They produce a specific paraprotein (or M protein), which is an abnormal antibody (immunoglobulin) or part of an antibody (often the light chain). There is an abnormally high level of this paraprotein (paraproteinaemia).

81
Q

what does the Bence Jones protein refer to

A

free light chains in the urine

82
Q

what is the pneumonic used to remember the four key features of myeloma

A

CRAB

83
Q

what does CRAB stand for

A

C – Calcium (elevated)
R – Renal failure
A – Anaemia
B – Bone lesions and bone pain

84
Q

what is the most common complication of myeloma

A

anaemia

the cancerous plasma cells invade the bone marrow, resulting in suppression of the other blood cell lines, leading to anaemia, leukopenia, and thrombocytopenia

85
Q

what type of anaemia is seen in myeloma

A

normocytic and normochromic

86
Q

what does myeloma bone disease result from

A

increased osteoclast activity and suppressed osteoblast activity

87
Q

what do osteoblasts and osteoclasts do

A

osteoblasts deposit bone and osteoclasts absorb bone

88
Q

where are the common sites of myeloma bone disease

A

skull, spine, long bones and ribs

89
Q

what are patches of thin bone described as

A

osteolytic lesions

90
Q

what do the weak points in the bone lead to

A

pathological features for example a vertebral body in the spine may collapse or a long bone such as the femur may break under minimal force

91
Q

what does increased osteoclast activity cause

A

calcium rebasoption from the bone into the blood resulting in hypercalcaemia

92
Q

what are plasmacytomas

A

individual tumours formed by cancerous plasma cells

they can occur in the bones, replacing normal bone tissue or in the soft tissue

93
Q

what are the various causes of renal impairment in patients with myeloma

A

Paraproteins deposited in the kidneys
Hypercalcaemia affecting kidney function
Dehydration
Glomerulonephritis (inflammation around the glomerulus and nephron)
Medications used to treat the condition

94
Q

what is the normal plasma viscocity or internal friction in blood flow

A

between 1.3 and 1.7 times that of water

95
Q

when does plasma viscocity increase

A

when there are more proteins in the bloof, such as the paraproteins found in myeloma

96
Q

what are the issues that hyperviscocity syndrome may cause

A

Bleeding (e.g., nosebleeds and bleeding gums)
Visual symptoms and eye changes (e.g., retinal haemorrhages)
Neurological complications (e.g., stroke)
Heart failure

97
Q

what presenting features that should raise suspicion of myeloma include

A

Persistent bone pain (e.g., spinal pain)
Pathological fractures
Unexplained fatigue
Unexplained weight loss
Fever of unknown origin
Hypercalcaemia
Anaemia
Renal impairment

98
Q

what are the laboratory results seen in myeloma

A

FBC (anaemia or leukopenia in myeloma)
Calcium (raised in myeloma)
ESR (increased in myeloma)
Plasma viscosity (increased in myeloma)
U&E (for renal impairment)
Serum protein electrophoresis (to detect paraproteinaemia)
Serum-free light-chain assay (to detect abnormally abundant light chains)
Urine protein electrophoresis (to detect the Bence-Jones protein)

99
Q

what is used to confirm the diagnosis of myeloma

A

bone marrow biopsy

100
Q

what are the typical x ray findings seen in patients with myeloma

A

Well-defined lytic lesions (described as looking “punched-out”)
Diffuse osteopenia
Abnormal fractures

101
Q

what is the name of the condition that refers to multiple lytic lesions seen in the skull on X ray

A

raindrop skull

102
Q

is myeloma ever fully cured

A

myeloma takes a relapsing-remitting course and is never fully cured

103
Q

what does treatment for myeloma usually involve (chemotherapy)

A

Bortezomib (a proteasome inhibitor)
Thalidomide
Dexamethasone

104
Q

what is a treatment option given to fitter patients and may achieve a more extended period of remission

A

High-dose chemotherapy followed by a stem cell transplant is an option for fitter patients and may achieve a more extended period of remission

105
Q

what are the two types of stem cell transplantation

A

Autologous (using the person’s own stem cells)

Allogeneic (using stem cells from a healthy donor)

106
Q

what does management of myeloma bone disease involve

A

Bisphosphonates to suppress osteoclast activity
Radiotherapy for bone lesions can improve bone pain
Orthopaedic surgery to stabilise bones (e.g., by inserting a prophylactic intramedullary rod) or treat fractures
Cement augmentation (injecting cement into vertebral fractures or lesions) to improve spine stability and pain

107
Q

what is pancytopenia

A

a condition in which there is a lower-than-normal number of red and white blood cells and platelets in the blood

108
Q

what are the main causes of pancytopenia

A

megaloblastic anaemia, AML and aplastic anaemia

109
Q
A

Year 3 (63 decks)

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