Malignancy Flashcards

1
Q

Haematological malignancy can be classed based on:

A

lineage
developmental stage within lineage
anatomical site involved

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2
Q

Lineage of malignancy can be

A

myeloid or lymphoid

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3
Q

Developmental stage of malignancy can be

A

acute myeloblastic/ lymphoblastic (EARLY DEVELOPMENTAL STAGE)

chronic myeloid/ lymphocytic (LATE DEVELOPMENTAL STAGE)

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4
Q

Anatomical site of malignancy can be

A

blood involvement- LEUKAEMIA

lymph node involvement with lymphoid malignancy - LYMPHOMA

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5
Q

____________ and ____________ are histologically and usually clinically more aggressive than chronic leukaemia and low grade lymphomas

A

acute leukaemia and high grade lymphomas

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6
Q

Features of histological aggression?

A

large cells with high nuclear-cytoplasmic ratio
prominent nucleoli
rapid proliferation

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7
Q

Feature of clinical aggression?

A

rapid progression of symptoms

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8
Q

Acute leukaemia present with ________

A

failure of normal marrow function

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9
Q

Explain what leukaemia is?

A

primary malignant tumours of haemopoietic cells
neoplastic haemopoietic cells fill the marrow including long bones and there is spill over of abnormal cells into the blood

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10
Q

What is the marrow cellularity like in leukaemia?

A

high marrow cellularity

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11
Q

Explain the clinical presentation of leukaemia?

A

blast cells are taking over marrow so get ineffective haemopoiesis and deficiency in other parts of the blood

deficiency of red cells > anaemia
deficiency of platelets > thrombocytopenia
deficiency of white cells > infection

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12
Q

Describe the spread of leukaemia?

A

leukaemia cells circulate in the blood and/ or lymph and can therefore spread anywhere in the body
nodular deposits are however uncommon
after initially successful treatment, leukaemia infiltration may recur especially in the CNS (meninges) and the testis

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13
Q

What are the four main leukaemias?

A

acute myeloblastic leukaemia
acute lymphoblastic leukaemia
chronic myeloid leukaemia
chronic lymphocytic leukaemia

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14
Q

What is the aetiology of leukaemia?

A

in most cases the cause is unknown but risk factors include:
radiation
chemicals e.g. chemo, benzene
genetics
virus e.g. human T cell leukaemia virus 1

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15
Q

Describe the features of acute leukaemia?

A

rapidly progressive clonal malignancy of the marrow/ blood with maturation defects
defined as an excess of blasts ( > 20%) in either peripheral blood or bone marrow
decrease/ loss of haemopoietic reserve

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16
Q

What is the most common childhood cancer?

A

acute lymphoblastic leukaemia

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17
Q

Which acute leukaemia is mainly adults and which is mainly children?

A

children: acute lymphoblastic leukaemia
adults: acute myeloid leukaemia

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18
Q

What is ALL?

A

malignant disease of primitive lymphoid cells (lymphoblasts)

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19
Q

Clinical presentation of ALL?

A

symptoms of marrow failure:
anaemia - pallor, fatigue, breathlessness, faintness
infections
bleeding- failure of primary haemostasis due to thrombocytopenia, mucosal bleeding, easy bruising

bone pain

more common in ALL than other leukaemia to get CNS and testis involvement

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20
Q

Marrow is packed with blast cells and blood shows presence of blast cells?

A

acute leukaemia

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21
Q

What is required for definitive diagnosis of leukaemia?

A

immunophenotyping

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22
Q

What may FBC show in acute leukaemia?

A

low Hb, neutrophils and platelets

WCC may be raised because there are lots of blast cells

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23
Q

What is AML?

A

malignant disease of primitive myeloid cells (myeloblasts)

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24
Q

Who is AML more common in?

A

elderly (> 60 yo)

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25
Q

AML can occur de novo or?

A

secondary due to treatment of other cancer

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26
Q

Presentation of AML?

A

similar to ALL

there are certain subgroups though with characteristic presentations

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27
Q

Auer rods?

A

AML

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28
Q

What is chronic lymphocytic leukaemia?

A

cancer with monoclonal proliferation of small lymphocytes (the leukaemia form of lymphocytic lymphoma)
most cases are B cell type

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29
Q

Presentation of chronic lymphocytic leukaemia?

A

presents more slowly

anaemia, recurrent infections and can also get swollen lymph nodes

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30
Q

Treatment of chronic vs acute leukaemia?

A

acute tends to be more treatable

but chronic is less treatable but people live for years with it and people can die with the cancer not of the cancer

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31
Q

Curative treatment of acute leukaemia involves?

A

multi-agent chemotherapy

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32
Q

If someone getting chemo for leukaemia has neutropenic fever ______

A

they need empirical treatment with broad spectrum antibiotics

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33
Q

When should you suspect fungal infection in someone on chemo for leukaemia?

A

prolonged neutropenia and persisting fever unresponsive to anti-bacterial agents

34
Q

Prognosis of acute leukaemia?

A

many patients will go into remission (< 5% blasts with recovery of normal haemopoiesis)
unfortunately remissions may not be durable depending on the type of acute leukaemia and many patients will relapse
some patient will die of treatment related toxicity
childhood leukaemia has higher cure rates (ALL)

35
Q

2 main categories of lymphadenopathy?

A
reactive hyperplasia (should cease when stimulus stops)
neoplastic (no stimulus)
36
Q

What are the causes of reactive hyperplasia lymphadenopathy?

A

infection by bacteria or virus
immune stimulation e.g. RA
drugs
particulate material e.g. carbon breathed into the lungs causing a reaction

37
Q

What are the two causes of neoplastic lymphadenopathy?

A

primary lymphoid tumours

secondary metastatic cancer spread

38
Q

What types of lymphadenopathy would cause tender lymph nodes?

A

viral and bacterial

39
Q

What types of lymphadenopathy would cause non-tender lymph nodes?

A

lymphoma and metastatic cancer

40
Q

What will the consistency of lymph nodes in bacterial or viral infection be like?

A

firm

41
Q

What will the consistency of lymph nodes in lymphoma be like?

A

rubbery/ soft

42
Q

What will the consistency of lymph nodes in metastatic cancer be like?

A

hard

43
Q

What types of lymphadenopathy will have smooth lymph nodes?

A

bacterial and viral infection

lymphoma

44
Q

What types of lymphadenopathy will have irregular lymph nodes?

A

metastatic cancer

45
Q

What type of lymphadenopathy will have skin inflammation?

A

bacterial

46
Q

Are lymph nodes tethered in viral infection?

A

no

47
Q

Are lymph nodes tethered in bacterial infection?

A

sometimes

48
Q

Are lymph nodes tethered in lymphoma?

A

no

49
Q

Are lymph nodes tethered in metastatic cancer?

A

yes

50
Q

What is lymphoma?

A

malignant tumours derived from lymphoid cells, may arise within the lymph nodes or at other sites (extra nodal)

51
Q

Describe what 2 groups are lymphomas classically divided into?

A

Hodgkin 20-25%

  • almost always lymph node origin
  • characterised by presence of Reed-Sternberg cells

Non-Hodgkins 75-80%

  • 3/4 arise in lymph nodes
  • 90% are B cell and B cell is usually low grade and T cell high grade
52
Q

Risk factors for lymphoma?

A

none may be present
immunosuppression e.g. HIV, inherited immunodeficiency states
auto-immune disorders e.g. Sjrogens, coeliacs
infections e.g. EBV, H. pylori, HHV-8, HTLV-1
genetic predisposition
environmental factors

53
Q

Reed Sternberg cell?

A

hodgkin lymphoma

54
Q

Peak incidence in hodgkin lymphoma is in?

A

3rd decade

55
Q

Spread to lymph nodes in hodgkin lymphoma is____

A

orderly

56
Q

Treatment of hodgkin lymphoma is?

A

multi agent chemo +/- radio

57
Q

Bleomycin used in treatment of hodgkin lymphoma can cause

A

pneumonitis

58
Q

What is the cure rate of hodgkin lymphoma like?

A

good cure rates, particularly in younger patients

59
Q

What are the risks of intensive treatment in hodgkin lymphoma?

A

long term toxicity, including secondary cancers, CVS disease, infertility

60
Q

What is an option for patients with hodgkin lymphoma not responding to chemo?

A

immunotherapy/ stem cell transplantation

61
Q

What is a common high grade non hodgkin lymphoma?

A

diffuse large B cell lymphoma

62
Q

Is extra nodal disease common in non hodgkin lymphoma?

A

yes more so that hodgkins

particularly in T cell NHL, Burkitt lymphoma

63
Q

Treatment of non-hodgkin lymphoma?

A

multi agent chemo +/- radio
can also do monoclonal Ab therapy + chemo
e.g. ritixumab for B cell and brentuximab for T cell

64
Q

Is non hodgkin lymphoma curable?

A

high grade NHL is potentially curable

low grade is incurable but many patients may not need treatment

65
Q

What is an option for non hodgkin lymphoma patients failing chemo?

A

high dose therapy with autologous stem cell rescue or CART therapy

66
Q

What is the fastest growing human tumour?

A

Burkitt lymphoma (a type of non hodgkin lymphoma)

67
Q

What does Burkitt lymphoma have a relationship with?

A

EBV

68
Q

Where is Burkitt lymphoma more common?

A

equatorial africa, new guinea and other malaria endemic areas

69
Q

Chromosomal translocations involving c-myc gene?

A

Burkitt lymphoma

70
Q

Cure rate of Burkitt lymphoma?

A

has a high cure rate in high income countries

71
Q

What is meant by B symptoms?

A

Used in Ann Arbor staging
if someone has lymphadenopathy and absence of symptoms write A
if someone has some of the systemic symptoms listed it is denoted with a B

72
Q

List the B symptoms of lymphoma?

A

fever or
night sweats or
weight loss (unintentional 10% over 6 month period)

73
Q

What are some other symptoms of lymphoma that are not B symptoms?

A

itch without rash
alcohol induced pain of lymph nodes
symptoms relevant to compression > renal failure, SVC obstruction, effusions, marrow failure

74
Q

What is often increased in lymphoma?

A

LDH

75
Q

What type of biopsy is best for lymphoma?

A

FNA and core biopsy are often insufficient

excision biopsy is best

76
Q

What is the role of steroids in treatment of lymphoma?

A

lymphocytes are normally sensitive to steroid therapy and some lymphomas are exquisitely sensitive
steroids are an integral part of multi-agent chemo regimens for routine treatment of lymphoma
steroids can be used in emergency management of suspected lymphoma but starting steroid before biopsy can cause cell necrosis and distort cellular and tissue architecture confusing the diagnosis

77
Q

What do smudge cells suggest?

A

chronic lymphocytic leukaemia

the lymphocytes are fragile so break more easily when being prepared for the blood film

78
Q

What can cause atypical lymphocytes?

A

generally viral infections
EBV is the main one
(COVID can cause it too)

79
Q

Offer an urgent FBC (within 48 hours) for? (7)

A

Anyone presenting with unexplained:
- pallor
-fatigue
- generalised lympahdenopathy
- persistent infections
-bone pain
-bruising
-bleeding

80
Q

Children with what should be sent for immediate specialist referral due to chance of leukaemia?

A

unexplained petechiae or hepatosplenomegaly