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Y3 - Haem > Malignancy > Flashcards

Malignancy Flashcards

1
Q

What is leukaemia?

A

Haematological malignancies where there is malignant monoclonoal expansion of a single cell line

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2
Q

What are risk factors for leukaemia?

A

Irradiation
Chromosomal abnormalities (Down syndrome)
Drugs (cytotoxic - cyclophosphamide, melphalan)

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3
Q

What are the types of leukaemia?

A

Acute lymphoblastic leukaemia
Acute myeloid leukaemia
Chronic lymphocytic anameia
Chronic myeloid leukaemia

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4
Q

What are features of aggressive disease in leukaemia?

A

Acute onset, rapid progression of symptoms
Large cells with high nuclear:cytoplasmic ratio
Prominent nucleoli
Rapid proliferation

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5
Q

What is acute leukaemia?

A

Malignant proliferation of abnormal progenitors (blasts) with arrested maturation
The abnormal cells replace the normal cells resulting in reduction in bone marrow function

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6
Q

In what circumstance can acute myeloid leukaemia arise secondary to something else?

A

Can arise secondary to treatment with chemotherapy fro other malignancies

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7
Q

What are the common sites of infiltration outside the bone marrow in acute lymphoblastic leukaemia?

A

CNS
Lymph nodes
Liver/spleen
Testicles

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8
Q

What are the common sites of infiltration outside the bone marrow in acute myeloid leukaemia?

A 
Skin
Gums
Lymph nodes
Liver/spleen
(no CNS)
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9
Q

What is the most common childhood cancer?

A

Acute lymphoblastic leukaemia

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10
Q

What age does acute lymphoblastic leukaemia most commonly occur?

A

2-8

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11
Q

What age does acute myeloid leukaemia most commonly occur?

A

Adults

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12
Q

What is the presentation of acute leukaemia?

A 
Symptoms of marrow failure 
- anaemia (fatigue)
- thrombocytopenia (braising, bleeding)
- leukopenia (infections)
Bone pain
Lymphadenopathy
Hepatosplenomegaly
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13
Q

Which infections are commonest in acute leukaemia?

A

Throat and ear

Mostly bacterial

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14
Q

What are the symptoms of extra-marrow infiltration in acute lymphoblastic leukaemia?

A

CNS - cranial nerve palsies, meningism
Liver/spleen - abdominal pain, swelling
Orchidomegaly

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15
Q

What are the symptoms of extra-marrow infiltration in acute myeloid leukaemia?

A

Gum hypertrophy

Liver/spleen - abdominal pain, swelling

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16
Q

What do blood tests show in acute leukaemia?

A

Normocytic, normochromic anaemia
Neutropenia
Thrombocytopenia in AML
Leucocytosis - high WCC (variable in AML)

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17
Q

What does a blood film show in acute lymphoblastic leukaemia?

A

> 20% blasts

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18
Q

What do blasts cells look like?

A

High nuclear:cytoplasm ratio
Prominent nucleus
Abnormal granulation

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19
Q

What does a blood film show in acute myeloid leukaemia?

A

> 20% blasts

Auer rods

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20
Q

What are Auer rods?

A

Cells seen in acute myeloid leukaemia - diagnostic

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21
Q

What is required for a definitive diagnosis of acute leukaemia?

A

Immunophenotyping

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22
Q

What investigation is used to look for infiltration in acute leukaemia?

A

CT CAP (chest, abdomen, pelvis) or CT head

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23
Q

What is the management for acute lymphoblastic leukaemia?

A

Chemotherapy

Long duration, curative intent

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24
Q

What is the cure rate for acute lymphoblastic leukaemia?

A

90% in children

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25
Q

How long is chemo given in acute lymphoblastic leukaemia?

A

Maintenance chemo is given for 2-3 years

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26
Q

What is the treatment for acute myeloid leukaemia?

A

Intense chemotherapy

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27
Q

What chemotherapy drugs can be given in acute myeloid leukaemia?

A

Danorubicin

Cytarabine

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28
Q

What is neutropenic fever?

A

Neutropenia increases severity and duration of infections so in neutropenia gram negative bacteria can cause life-threatening sepsis in neutropenic patients that comes on quickly and with great severity
Bacterial or fungal

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29
Q

What is the management of neutropenic fever?

A

Quick clinical assessment and culture of fluids

Start on broad spectrum antibiotics before cultures come back

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30
Q

What are the complications of chemotherapy?

A 
Nausea and vomiting
Hair loss
Liver, renal dysfunction
Tumour lysis syndrome
Infection
Late - loss of fertility, cardiomyopathy
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31
Q

What is tumour lysis syndrome?

A

Potentially deadly condition presenting in the first round of chemo
hyperkalemia, hyperphosphatemia, hypocalcemia, and hyperuricemia

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32
Q

What is given as protection against tumour lysis syndrome?

A

IV allopurinol prior to and in the first days of chemo

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33
Q

What are the blood test results in chemotherapy?

A

High potassium
High phosphate
Low calcium

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34
Q

What are the complications of tumour lysis syndrome?

A

Cardiac arrhythmias
Seizures
Sudden death

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35
Q

When should you suspect a fungal infection in neutropenic fever?

A

Prolonged neutropenia and persisting fever unresponsive to antibiotics

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36
Q

Which protozoal infection can cause neutropenic fever, and which leukaemia is thus more common in?

A

Pneumocystis jiroveci pneumonia

Acute lymphoblastic anaemia

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37
Q

What is chronic lymphocytic leukaemia?

A

Expansion of a B cell population
Proliferation of abnormal progenitors with no maturation block
So too many lymphoid cells

38
Q

What are the lymphoid cells?

A

T cells
B cells
Plasma cells
NK cells

39
Q

What are the myeloid cells?

A

Erythrocytes
Platelets
Granulocytes (basophils, neutrophils, eosinophils)
Macrophages

40
Q

What is the most common leukaemia?

A

Chronic lymphocytic leukaemia

41
Q

What is the median age of presentation of chronic lymphocytic leukaemia?

A

70

42
Q

What condition is chronic lymphocytic leukaemia associated with?

A

Autoimmune haemolytic anaemia

Idiopathic thrombocytic purpura

43
Q

What is the presentation of chronic lymphocytic leukaemia?

A 
Can be asymptomatic
Symptoms of marrow failure (anaemia, bleeding, infections)
B symptoms 
- night sweats
- weight loss
Lymphadenopathy
44
Q

What do blood tests show in chronic lymphocytic leukaemia?

A

Normochromic normocytic anaemia
Low neutrophils, low platelets
High WCC - high lymphocytes

45
Q

What does electrophoresis show in chronic lymphocytic leukaemia?

A

Hypogammaglobinaemia

46
Q

What does a blood film show in chronic lymphocytic leukaemia?

A

Smear/smudge cells

No blasts

47
Q

What is the progression of chronic lymphocytic leukaemia?

A

Rule of 3rds

  • 1/3 don’t progress
  • 1/3 will progress with time
  • 1/3 actively progressing at presentation
48
Q

What is the management of chronic lymphocytic leukaemia that is not actively progressing?

A

Observation

49
Q

What is the management of chronic lymphocytic leukaemia that IS actively progressing?

A

Chemotherapy
- cyclophosphamide, fludarabine
+/- stem cell transplant

50
Q

When is chemotherapy indicated in chronic lymphocytic leukaemia?

A

Marrow failure
Massive or progressive splenomegaly or lymphadenopathy
Progressive - doubling of lymphocyte count in less than 6 months
Systemic symptoms (fever, night sweats, weight loss)
Haemolysis

51
Q

What can chronic lymphocytic leukaemia progress to?

A

Lymphoma

52
Q

If chronic lymphocytic leukaemia has progressed to another condition, what investigation is done?

A

Lymph node biopsy

53
Q

What is chronic myeloid leukaemia?

A

Considered to be a myeloproliferative disorder where there is clonal expansion of a stem cell population producing myeloid cells
Proliferation of abnormal progenitors but not maturation block - so too many myeloid cells

54
Q

What is pathognomonic for chronic myeloid leukaemia?

A

Philadelphia chromosome

9;22

55
Q

What is the philadelphia chromosome?

A

Pathognomonic for chronic myeloid leukaemia
Resultant gene is BCR-ABL1
Produces abnormal tyrosine kinase that drives stem cell proliferation

56
Q

What are the phases of chronic myeloid leukaemia?

A

Chronic - 3-5 years of myeloproliferation with sparing of maturation
Accelerated - maturation begins to slow an symptoms worsen
Blast crisis - maturation fails and immature blasts accumulate (essentially becomes AML)

57
Q

Who does chronic myeloid leukaemia most commonly affect?

A

40-60 year olds

58
Q

What is the presentation of chronic myeloid leukaemia?

A 
Slow onset
Weight loss
Fatigue
Fever
Night sweats
Gout
Abdominal pain/bloating, early severity (splenomegaly)
59
Q

Why does gout present in chronic myeloid leukaemia?

A

Increased rate due to increased cell turnover

60
Q

What do blood show in chronic myeloid leukaemia?

A

High urate
Normochromic, normocytic anaemia
High WCC (neutrophils, basophils, eosinophils)

61
Q

What does a bone marrow biopsy show in chronic myeloid leukaemia?

A

Hypercellular marrow

62
Q

What is the management of chronic myeloid leukaemia?

A

Tyrosine kinase inhibitors - imatinib

63
Q

What effect do tyrosine kinase inhibitors have on chronic myeloid leukaemia?

A

Not curative but completely control the disease

64
Q

What is lymphoma?

A

Malignant proliferations of lymphocytes that accumulate in lymph nodes causing lymphadenopathy

65
Q

What are risk factors for lymphoma?

A 
Idiopathic
Immunosuppression - HIV, inherited immunodeficiency
Autoimmune disorders
Infections - EBV, H pylori
Genetic predisposition
Environment
66
Q

What is the presentation of lymphoma?

A 
Lymphadenopathy
B symptoms
- fever
- night sweats
- weight loss
Lethargy
Itch without rash
Alcohol-induced pain
Compression, infiltration and extra-modal disease
67
Q

What is the character of lymphadenopathy in lymphoma?

A

Painless, soft, rubbery, persistent

68
Q

What is the degree of weight loss seen in lymphoma?

A

10% over a 6 month period

69
Q

What are the symptoms of compression, infiltration and extra-modal disease in lymphoma?

A 
Renal failure
Superior vena cava obstruction
- SOB
- Dilated neck veins
- Facial swelling
Effusions
Marrow failure
70
Q

What is the diagnostic investigation for lymphoma?

A

Lymph node biopsy

71
Q

What is the staging system for lymphoma?

A

Ann Arbor staging
Stage 1 - one lymph node or extra-lymph node area
Stage 2 - 2 or more lymph nodes on one side of the diaphragm
Stage 3 - 2 or more lymph nodes on both sides of the diaphragm
Stage 4 - disseminated or multiple extra-nodal area involvement (including bone marrow)

72
Q

What do blood tests show in lymphoma?

A

Raised lactate dehydrogenase (prognostic not diagnostic)

Lymphocytosis in CLL

73
Q

What is Hodgkin’s lymphoma?

A

Malignant proliferations of B cells

74
Q

What is Hodgkin’s lymphoma characterised by?

A

Reed-Steinberg cell

75
Q

What do reed-steinberg cells look like?

A

2 nuclei

Owls (salad fingers)

76
Q

What is the most common subtype of Hodgkin’s lymphoma?

A

Nodular sclerosing

77
Q

What is the spread of Hodgkin’s lymphoma?

A

Orderly lymph node spread

78
Q

What features of lymphoma presentation are specific to Hodgkin’s lymphoma?

A

Lymph node pain on drinking alcohol

79
Q

Who presents most commonly with Hodgkin’s lymphoma?

A 
Young adults (15-30)
Or elderly
80
Q

What is the management of Hodgkin’s lymphoma?

A

Multi-agent chemotherapy

- Adriamycin, bleomycin, vincristine, dacarbazine

81
Q

What are complications of Hodgkin’s lymphoma treatment?

A

Bleomycin can cause pneumonitis

Associated with marrow suppression and risk of AML

82
Q

What is the second line treatment for Hodgkin’s lymphoma?

A

Immunotherapy/stem cell transplantation

83
Q

What is non-Hodgkin’s lymphoma?

A

Diverse group go lymphomas that are not Reed-Steinberg positive

84
Q

Which is the more common non-Hodgkin’s lymphoma - B or T cell lymphoma?

A

B cell

85
Q

Are B cell lymphomas generally low or high grade?

A

Low grade

86
Q

What is a common high grade non-Hodgkin’s lymphoma?

A

Diffuse large B cell lymphoma

87
Q

What are risk factors for non-Hodgkin’s lymphoma?

A

Immunosuppression

Infection - HIV, EBV, malaria, hepatitis C, H pylori

88
Q

What are some features of Burkitt lymphoma?

A

Fastest growing
EBV and HIV association
Beware of tumour lysis

89
Q

What are symptoms that can be caused by extra-nodal involvements in non-Hodgkin’s lymphoma?

A 
CNS
Spleen - splenomegaly
Marrow - marrow failure
Skin - papular rash, usually T cell
Waldeyer's ring - difficulty breathing
90
Q

Who usually presents with non-Hodgkin’s lymphoma?

A

Usually older patients but can vary

91
Q

What is the management of non-Hodgkin’s lymphoma?

A

R-CHOP
(Rituximab, Cyclophosphamide, Hydroxydaunorubicin, Vinicristine, Predisolone)
Steroids

92
Q

What is the role of steroids in management of non-Hodgkin’s lymphoma?

A

Can be used in emergency management of suspected lymphoma but starting steroid before biopsy can cause cell necrosis and distort cellular and tissue architecture to confuse the diagnosis - do biopsy before starting steroids

Y3 - Haem (9 decks)

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