Haematology Flashcards

1
Q

What is Myeloma?

A

Malignant disease of bone-marrow plasma cells

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2
Q

What happens pathophysiologically in Myeloma?

A

Clonal Expansion of abnormal proliferating plasma cells lead to a monoclonal paraprotein. This produces paraproteinaemia

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3
Q

What may be found in the urine of a patient with Myeloma?

A

Bence-Jones Protein - Excretion of light chains in the urine

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4
Q

What are some clinical features of Myeloma?

A

Bone Destruction
Bone Marrow infiltration
Kidney Injury
Recurrent infections

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5
Q

What causes Bone Destruction in Myeloma?

A

Increased osteoclastic activity in the absence of osteoblast activity

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6
Q

What can bone marrow infiltration in Myeloma lead to?

A

Anaemia
Neutropenia
Thrombocytopenia

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7
Q

What causes Kidney Injury in Myeloma?

A

Light chain deposition in the renal tubules
Hypercalcaemia
Hyperuricaemia
NSAID use

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8
Q

Why does Myeloma lead to recurrent infections?

A

Reduced levels of usual immunoglobulins

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9
Q

What are some symptoms of Myeloma?

A

Bone pain - Often backache due to vertebral involvement
Symptoms of Anaemia
Recurrent infections
Symptoms of Renal Failure
Symptoms of Hypercalcaemia
Symptoms of Hyperviscosity secondary to Thrombocytopenia

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10
Q

Which blood investigations are appropriate for suspected Myeloma?

A

FBC - Hb, WCC and Platelets either normal or low
ESR - Elevated
U+E - Kidney Injury
Serum Ca - Raised

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11
Q

What may a blood film demonstrate in Myeloma?

A

Aggregates of Red Blood Cells - Rouleaux

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12
Q

What will serum protein electrophoresis demonstrate in Myeloma?

A

Monoclonal Paraprotein Band with Immune Phoresis

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13
Q

Which Radiological investigation is appropriate in suspected Myeloma?

A

Skeletal Survey

CT/MRI

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14
Q

How is the diagnosis of Myeloma confirmed?

A

One of either:
Significant Paraproteinaemia
Increased Bone Marrow Plasma Cells on biopsy

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15
Q

What are some general supportive measures to manage Myeloma?

A

Correct Anaemia with Transfusion +/- EPO
Prompt Abx for infections
Bone Pain - Radiotherapy +/- Chemotherapy
Orthopaedic intervention for at-risk bones

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16
Q

Which specific therapies can be used in Myeloma?

A

Thalidomide

Cyclophosphamide

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17
Q

What is Acute Leukaemia?

A

Uncontrolled proliferation of partially developed WBCs in the blood over a short period of time

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18
Q

What causes Acute Leukaemia?

A

Mutation of precursor blood cells within the bone marrow

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19
Q

Which types of Acute Leukaemia are there?

A

Acute Lymphoblastic Leukaemia

Acute Myeloid Leukaemia

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20
Q

What is Acute Lymphoblastic Leukaemia (ALL)?

A

Mutations within bone marrow give excessive proliferation of T-Lymphoblasts and B-Lymphoblasts (precursors of T-Lymphocytes and B-Lymphocytes)

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21
Q

What is the primary cause of ALL?

A

Chromosomal Translocations

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22
Q

Which Chromosomal Translocations are associated with development of ALL?

A

t(12:21)

t(9-22) - Philadelphia Translocation

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23
Q

What are some symptoms of ALL?

A

Bone Marrow Failure - Anaemia, Neutropenia, Thrombocytopenia
Bone Pain - Secondary to marrow infiltration
Splenomegaly
Hepatomegaly
Fever

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24
Q

What are some subtypes of ALL?

A

Pre-B Cell ALL
Pre-T Cell ALL - Thymus/Mediastinal mass, mainly teenagers
Mature B-Cell ALL

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25
Q

What are some appropriate investigations for suspected ALL?

A
CXR - ?Mediastinal Mass
Bloods - Raised WCC, Lymphocytes
Bone Marrow Biopsy
Cytogenetic Testing
Lymph Node Biopsy
CT Scan
LP - ?CNS Involvement
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26
Q

How may ALL be treated?

A
Chemotherapy - Mainstay treatment
Steroids
Radiotherapy
Growth Factors
Stem cell transplant
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27
Q

What are the stages of ALL treatment?

A

Phase 1 - Remission Induction
Phase 2 - Consolidation/Intensification Therapy
Phase 3 - Maintenance Therapy

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28
Q

What is Acute Myeloid Leukaemia (AML)?

A

Leukaemia that arises out of malignant transformation of a myeloid precursor

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29
Q

In which age group is AML commonly found?

A

Older, incidence increases with age

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30
Q

How is AML classified?

A

Using the French-American-British (FAB) Classification which is based on the appearance of Leukaemic cells on Bone Marrow Aspirate

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31
Q

What are some symptoms of AML?

A

Asymptomatic
Bone Marrow Infiltration - Anaemia, Neutropenia, Thrombocytopenia
Infection
haemorrhage

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32
Q

Which chromosomal translocation is associated with development of AML?

A

t(15;17)

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33
Q

What are some appropriate investigations for suspected AML?

A
Bloods - Raised WCC, Anaemia
Bone marrow aspirate and trephine
Cytochemistry
Immunophenotyping
Cytogenetics
Molecular Biology
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34
Q

What are some general supportive treatment measures for AML?

A

Correct Anaemia

Prompt IV Abx for infections

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35
Q

Which chemotherapeutic agents are commonly used in AML?

A

Cytarabine

Idarubicin

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36
Q

When does Chronic Lymphocytic Leukaemia (CLL) commonly occur?

A

Later in life

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37
Q

What occurs in CLL?

A

Clonal expansion of Lymphocytes, mainly B-Cell

38
Q

Which cell type is usually affected in CLL?

A

B Cell

39
Q

How can CLL present?

A

Asymptomatic
Recurrent infections - functional leucopenia and immune failure
Anaemia - Haemolysis and marrow infiltration
Painless Lymphadenopathy
LUQ discomfort due to Splenomegaly

40
Q

What are some possible clinical signs of CLL?

A

Anaemia
Fever secondary to infection
Lymphadenopathy
Hepatosplenomegaly

41
Q

What abnormalities may be found in FBC with CLL?

A

Normal/Low Hb
Raised WCC with Lymphocytosis
Normal/Low Platelets

42
Q

What is Lymphocytosis?

A

An increase in the number of Lymphocytes present in blood

43
Q

How may a blood film appear in CLL?

A

Normal appearing Lymphocytes

44
Q

What may a bone marrow aspirate demonstrate in CLL?

A

Heavy lymphocyte infiltration

45
Q

Which CD receptors may be found on lymphocytes in CLL?

A

CD19+
CD5+
CD23+

46
Q

How is CLL staged?

A

Rai-Binet staging

47
Q

What percentage of confirmed CLL cases require no treatment?

A

30%

48
Q

What are some absolute indications for treatment of CLL?

A

Marrow failure demonstrated by increasing anaemia and/or thrombocytopenia
Recurrent infection
Significant Splenomegaly/lymphadenopathy
Progression of disease with lymphocyte count doubling in 6m
Presence of haemolysis
Systemic Sx - Fever, Night sweats, weight loss

49
Q

Which agents can be used to treat CLL?

A

Chlorambucil
Rituximab
Cyclophosphamide

50
Q

Which transformation can CLL undergo?

A

Lymphomatous (Richters) Transformation

51
Q

What happens in Richters transformation of CLL?

A

It converts to large B-Cell lymphoma

52
Q

Following Richters transformation of CLL, what is the associated prognosis?

A

Poor, with survival often being short

53
Q

What is Lymphoma?

A

Malignancy of the lymphatic system which can present at any site containing lympoid tissue

54
Q

What are the two primary subtypes of Lymphoma?

A

Hodgkin’s

Non-Hodgkin’s

55
Q

What is Hodgkin’s Lymphoma?

A

B cell lymphoma characterised by the presence of Reed-Sternberg cells

56
Q

What are Reed-Sternberg cells?

A

Derrivations of abnormal B cells

57
Q

What are some syb-types of Hodgkin’s Lymphoma?

A

Classical Hodgkin’s Lymphoma

Nodular Lymphocyte Predominant Hodgkin’s Lymphoma

58
Q

What are some symptoms of Hodgkin’s Lymphoma?

A
Painless Cervical Lymphadenopathy
Pain following consumption of alcohol
Hepatomegaly 
Splenomegaly
B Symptoms
59
Q

What are B symptoms?

A

Fever
Weight loss
Pruritus
Drenching night sweats

60
Q

What may investigations of Hodgkin’s Lymphoma demonstrate?

A
Anaemia - Normochromic, normocytic, autoimmune haemolytic anaemia
Leucocytosis
Raised ESR
Raised LDH
Abnormal LFTs
61
Q

How is Hodgkin’s Lymphoma staged?

A

Cotswold modification of Ann-Arbor staging

62
Q

Can Hodgkin’s Lymphoma be cured?

A

Yes, cure is possible and likely if in early stages

63
Q

What treatment can be given to early stage Hodgkin’s Lymphoma?

A

Radiotherapy

64
Q

What treatment can be given to advanced Hodgkin’s Lymphoma?

A

Adrimycin
Bleomycin
Vinblastine
Dacarbazine

65
Q

What is Non-Hodgkin Lymphoma?

A

Malignant clonal expansion of Lymphocytes

66
Q

Which lymphocytes are usually affected in Non-Hodgkin Lymphoma?

A

B Lymphocytes - 80%

T Lymphocytes - 20%

67
Q

What are some risk factors for developing Non-Hodgkin Lymphoma?

A
Abnormal Response to Viral Infection
Bacterial Infection
Radiation
Drugs - Phenytoin
Autoimmune - Sjogrens
Immunosuppression
68
Q

Which virus is associated with the development of Burkitt’s Lymphoma?

A

Epstein-Barr Virus

69
Q

Which infection is associated with the development of Gastric Lymphoma?

A

Chronic H.Pylori

70
Q

What are some symptoms of Non-Hodgkin Lymphoma?

A

Widely disseminated lymphadenopathy
Splenomegaly
Hepatomegaly
Extra-nodal disease is common

71
Q

What may be demonstrated biochemically in NHL?

A

Anaemia/Pancytopenia due to marrow failure
Peripheral blood lymphocytosis
Paraproteinaemia/Hypogammaglobulinaemia
Raised LDH

72
Q

Which radiological investigations are used for investigation of suspected NHL?

A

XR
CT
PETCT

73
Q

What is high grade NHL?

A

More aggressive, cure is possible

74
Q

What is low-grade NHL?

A

More indolent, treatable but not curable

75
Q

What are some examples of high grade NHL?

A

Diffuse Large B Cell Lymphoma

Burkitt Lymphoma

76
Q

What are some examples of low grade NHL?

A
Small lymphocytic Lymphoma
Lymphoplasmacytic Lymphoma
Follicular Lymphoma
Marginal Cell Lymphoma
Mantle Cell Lymphoma
77
Q

What are some potential treatment options for NHL?

A
Excision
Watch and Wait
Chemotherapy
Radiotherapy
Monoclonal Antibody therapy
Small Molecules/Targeted Therapy
78
Q

What is Chronic Myeloid Leukaemia (CML)?

A

Myeloproliferative Neoplasm, nearly exclusively in adults with a peak incidence of 40-60y

79
Q

Which mutation is found in 95% of CML cases?

A

Philadelphia Chromosome - t(9;22)

80
Q

What can CML transform into?

A

Acute leukaemia or myelofibrosis due to a blast crisis transformation

81
Q

What are some presenting symptoms of CML?

A
Asymptomatic
Symptomatic Anaemia
Abdominal Discomfort due to Splenomegaly
B Symptoms
Headaches
Bruising
Bleeding
82
Q

What are some clinical signs suggestive of CML?

A
Pallor
Massive splenomegaly
Lymphadenopathy if blast crisis
Extramedullary soft tissue leukaemic deposits - Chloroma
Retinal Haemorrhage
83
Q

What is a Chloroma?

A

Extramedullary soft tissue leukaemic deposits

84
Q

What may bloods show in CML?

A

Low or normal HB
Raised WCC
Normal/Abnormal platelet count

85
Q

What may a blood film demonstrate in CML?

A

Neutrophilia with Myeloid Precursors

86
Q

What may a Bone Marrow aspirate demonstrate in CML?

A

Increased cellularity, increased myeloid precursors

Cytogenetic reveal 9;22 translocation

87
Q

Which agent is used to manage CML?

A

Imantinib - Tyrosine Kinase inhibitor

88
Q

How does Imantinib help to manage CML?

A

Suppresses activity of the fusion protein produced by the Philadelphia Translocation

89
Q

What are some side-effects of Imantinib?

A

Nausea
Headaches
Rash
Cytopenia

90
Q

How should an acute transformation of CML be managed?

A

Chemotherapy, then stem cell transplant for remission induction

91
Q

If Imantinib is ineffective in managing CML, how should this be managed?

A

Allogeneic Stem Cell Transplant