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Y3 - Haem > Malignancy > Flashcards

Malignancy Flashcards

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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
How long is chemo given in acute lymphoblastic leukaemia?
Maintenance chemo is given for 2-3 years
26
What is the treatment for acute myeloid leukaemia?
Intense chemotherapy
27
What chemotherapy drugs can be given in acute myeloid leukaemia?
Danorubicin | Cytarabine
28
What is neutropenic fever?
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
29
What is the management of neutropenic fever?
Quick clinical assessment and culture of fluids | Start on broad spectrum antibiotics before cultures come back
30
What are the complications of chemotherapy?
``` Nausea and vomiting Hair loss Liver, renal dysfunction Tumour lysis syndrome Infection Late - loss of fertility, cardiomyopathy ```
31
What is tumour lysis syndrome?
Potentially deadly condition presenting in the first round of chemo hyperkalemia, hyperphosphatemia, hypocalcemia, and hyperuricemia
32
What is given as protection against tumour lysis syndrome?
IV allopurinol prior to and in the first days of chemo
33
What are the blood test results in chemotherapy?
High potassium High phosphate Low calcium
34
What are the complications of tumour lysis syndrome?
Cardiac arrhythmias Seizures Sudden death
35
When should you suspect a fungal infection in neutropenic fever?
Prolonged neutropenia and persisting fever unresponsive to antibiotics
36
Which protozoal infection can cause neutropenic fever, and which leukaemia is thus more common in?
Pneumocystis jiroveci pneumonia | Acute lymphoblastic anaemia
37
What is chronic lymphocytic leukaemia?
Expansion of a B cell population Proliferation of abnormal progenitors with no maturation block So too many lymphoid cells
38
What are the lymphoid cells?
T cells B cells Plasma cells NK cells
39
What are the myeloid cells?
Erythrocytes Platelets Granulocytes (basophils, neutrophils, eosinophils) Macrophages
40
What is the most common leukaemia?
Chronic lymphocytic leukaemia
41
What is the median age of presentation of chronic lymphocytic leukaemia?
70
42
What condition is chronic lymphocytic leukaemia associated with?
Autoimmune haemolytic anaemia | Idiopathic thrombocytic purpura
43
What is the presentation of chronic lymphocytic leukaemia?
``` Can be asymptomatic Symptoms of marrow failure (anaemia, bleeding, infections) B symptoms - night sweats - weight loss Lymphadenopathy ```
44
What do blood tests show in chronic lymphocytic leukaemia?
Normochromic normocytic anaemia Low neutrophils, low platelets High WCC - high lymphocytes
45
What does electrophoresis show in chronic lymphocytic leukaemia?
Hypogammaglobinaemia
46
What does a blood film show in chronic lymphocytic leukaemia?
Smear/smudge cells | No blasts
47
What is the progression of chronic lymphocytic leukaemia?
Rule of 3rds - 1/3 don’t progress - 1/3 will progress with time - 1/3 actively progressing at presentation
48
What is the management of chronic lymphocytic leukaemia that is not actively progressing?
Observation
49
What is the management of chronic lymphocytic leukaemia that IS actively progressing?
Chemotherapy - cyclophosphamide, fludarabine +/- stem cell transplant
50
When is chemotherapy indicated in chronic lymphocytic leukaemia?
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
What can chronic lymphocytic leukaemia progress to?
Lymphoma
52
If chronic lymphocytic leukaemia has progressed to another condition, what investigation is done?
Lymph node biopsy
53
What is chronic myeloid leukaemia?
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
What is pathognomonic for chronic myeloid leukaemia?
Philadelphia chromosome | 9;22
55
What is the philadelphia chromosome?
Pathognomonic for chronic myeloid leukaemia Resultant gene is BCR-ABL1 Produces abnormal tyrosine kinase that drives stem cell proliferation
56
What are the phases of chronic myeloid leukaemia?
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
Who does chronic myeloid leukaemia most commonly affect?
40-60 year olds
58
What is the presentation of chronic myeloid leukaemia?
``` Slow onset Weight loss Fatigue Fever Night sweats Gout Abdominal pain/bloating, early severity (splenomegaly) ```
59
Why does gout present in chronic myeloid leukaemia?
Increased rate due to increased cell turnover
60
What do blood show in chronic myeloid leukaemia?
High urate Normochromic, normocytic anaemia High WCC (neutrophils, basophils, eosinophils)
61
What does a bone marrow biopsy show in chronic myeloid leukaemia?
Hypercellular marrow
62
What is the management of chronic myeloid leukaemia?
Tyrosine kinase inhibitors - imatinib
63
What effect do tyrosine kinase inhibitors have on chronic myeloid leukaemia?
Not curative but completely control the disease
64
What is lymphoma?
Malignant proliferations of lymphocytes that accumulate in lymph nodes causing lymphadenopathy
65
What are risk factors for lymphoma?
``` Idiopathic Immunosuppression - HIV, inherited immunodeficiency Autoimmune disorders Infections - EBV, H pylori Genetic predisposition Environment ```
66
What is the presentation of lymphoma?
``` Lymphadenopathy B symptoms - fever - night sweats - weight loss Lethargy Itch without rash Alcohol-induced pain Compression, infiltration and extra-modal disease ```
67
What is the character of lymphadenopathy in lymphoma?
Painless, soft, rubbery, persistent
68
What is the degree of weight loss seen in lymphoma?
10% over a 6 month period
69
What are the symptoms of compression, infiltration and extra-modal disease in lymphoma?
``` Renal failure Superior vena cava obstruction - SOB - Dilated neck veins - Facial swelling Effusions Marrow failure ```
70
What is the diagnostic investigation for lymphoma?
Lymph node biopsy
71
What is the staging system for lymphoma?
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
What do blood tests show in lymphoma?
Raised lactate dehydrogenase (prognostic not diagnostic) | Lymphocytosis in CLL
73
What is Hodgkin's lymphoma?
Malignant proliferations of B cells
74
What is Hodgkin's lymphoma characterised by?
Reed-Steinberg cell
75
What do reed-steinberg cells look like?
2 nuclei | Owls (salad fingers)
76
What is the most common subtype of Hodgkin's lymphoma?
Nodular sclerosing
77
What is the spread of Hodgkin's lymphoma?
Orderly lymph node spread
78
What features of lymphoma presentation are specific to Hodgkin's lymphoma?
Lymph node pain on drinking alcohol
79
Who presents most commonly with Hodgkin's lymphoma?
``` Young adults (15-30) Or elderly ```
80
What is the management of Hodgkin's lymphoma?
Multi-agent chemotherapy | - Adriamycin, bleomycin, vincristine, dacarbazine
81
What are complications of Hodgkin's lymphoma treatment?
Bleomycin can cause pneumonitis | Associated with marrow suppression and risk of AML
82
What is the second line treatment for Hodgkin's lymphoma?
Immunotherapy/stem cell transplantation
83
What is non-Hodgkin's lymphoma?
Diverse group go lymphomas that are not Reed-Steinberg positive
84
Which is the more common non-Hodgkin's lymphoma - B or T cell lymphoma?
B cell
85
Are B cell lymphomas generally low or high grade?
Low grade
86
What is a common high grade non-Hodgkin's lymphoma?
Diffuse large B cell lymphoma
87
What are risk factors for non-Hodgkin's lymphoma?
Immunosuppression | Infection - HIV, EBV, malaria, hepatitis C, H pylori
88
What are some features of Burkitt lymphoma?
Fastest growing EBV and HIV association Beware of tumour lysis
89
What are symptoms that can be caused by extra-nodal involvements in non-Hodgkin's lymphoma?
``` CNS Spleen - splenomegaly Marrow - marrow failure Skin - papular rash, usually T cell Waldeyer's ring - difficulty breathing ```
90
Who usually presents with non-Hodgkin's lymphoma?
Usually older patients but can vary
91
What is the management of non-Hodgkin's lymphoma?
R-CHOP (Rituximab, Cyclophosphamide, Hydroxydaunorubicin, Vinicristine, Predisolone) Steroids
92
What is the role of steroids in management of non-Hodgkin's lymphoma?
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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