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Haematology Flashcards

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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
What are some appropriate investigations for suspected ALL?
``` CXR - ?Mediastinal Mass Bloods - Raised WCC, Lymphocytes Bone Marrow Biopsy Cytogenetic Testing Lymph Node Biopsy CT Scan LP - ?CNS Involvement ```
26
How may ALL be treated?
``` Chemotherapy - Mainstay treatment Steroids Radiotherapy Growth Factors Stem cell transplant ```
27
What are the stages of ALL treatment?
Phase 1 - Remission Induction Phase 2 - Consolidation/Intensification Therapy Phase 3 - Maintenance Therapy
28
What is Acute Myeloid Leukaemia (AML)?
Leukaemia that arises out of malignant transformation of a myeloid precursor
29
In which age group is AML commonly found?
Older, incidence increases with age
30
How is AML classified?
Using the French-American-British (FAB) Classification which is based on the appearance of Leukaemic cells on Bone Marrow Aspirate
31
What are some symptoms of AML?
Asymptomatic Bone Marrow Infiltration - Anaemia, Neutropenia, Thrombocytopenia Infection haemorrhage
32
Which chromosomal translocation is associated with development of AML?
t(15;17)
33
What are some appropriate investigations for suspected AML?
``` Bloods - Raised WCC, Anaemia Bone marrow aspirate and trephine Cytochemistry Immunophenotyping Cytogenetics Molecular Biology ```
34
What are some general supportive treatment measures for AML?
Correct Anaemia | Prompt IV Abx for infections
35
Which chemotherapeutic agents are commonly used in AML?
Cytarabine | Idarubicin
36
When does Chronic Lymphocytic Leukaemia (CLL) commonly occur?
Later in life
37
What occurs in CLL?
Clonal expansion of Lymphocytes, mainly B-Cell
38
Which cell type is usually affected in CLL?
B Cell
39
How can CLL present?
Asymptomatic Recurrent infections - functional leucopenia and immune failure Anaemia - Haemolysis and marrow infiltration Painless Lymphadenopathy LUQ discomfort due to Splenomegaly
40
What are some possible clinical signs of CLL?
Anaemia Fever secondary to infection Lymphadenopathy Hepatosplenomegaly
41
What abnormalities may be found in FBC with CLL?
Normal/Low Hb Raised WCC with Lymphocytosis Normal/Low Platelets
42
What is Lymphocytosis?
An increase in the number of Lymphocytes present in blood
43
How may a blood film appear in CLL?
Normal appearing Lymphocytes
44
What may a bone marrow aspirate demonstrate in CLL?
Heavy lymphocyte infiltration
45
Which CD receptors may be found on lymphocytes in CLL?
CD19+ CD5+ CD23+
46
How is CLL staged?
Rai-Binet staging
47
What percentage of confirmed CLL cases require no treatment?
30%
48
What are some absolute indications for treatment of CLL?
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
Which agents can be used to treat CLL?
Chlorambucil Rituximab Cyclophosphamide
50
Which transformation can CLL undergo?
Lymphomatous (Richters) Transformation
51
What happens in Richters transformation of CLL?
It converts to large B-Cell lymphoma
52
Following Richters transformation of CLL, what is the associated prognosis?
Poor, with survival often being short
53
What is Lymphoma?
Malignancy of the lymphatic system which can present at any site containing lympoid tissue
54
What are the two primary subtypes of Lymphoma?
Hodgkin's | Non-Hodgkin's
55
What is Hodgkin's Lymphoma?
B cell lymphoma characterised by the presence of Reed-Sternberg cells
56
What are Reed-Sternberg cells?
Derrivations of abnormal B cells
57
What are some syb-types of Hodgkin's Lymphoma?
Classical Hodgkin's Lymphoma | Nodular Lymphocyte Predominant Hodgkin's Lymphoma
58
What are some symptoms of Hodgkin's Lymphoma?
``` Painless Cervical Lymphadenopathy Pain following consumption of alcohol Hepatomegaly Splenomegaly B Symptoms ```
59
What are B symptoms?
Fever Weight loss Pruritus Drenching night sweats
60
What may investigations of Hodgkin's Lymphoma demonstrate?
``` Anaemia - Normochromic, normocytic, autoimmune haemolytic anaemia Leucocytosis Raised ESR Raised LDH Abnormal LFTs ```
61
How is Hodgkin's Lymphoma staged?
Cotswold modification of Ann-Arbor staging
62
Can Hodgkin's Lymphoma be cured?
Yes, cure is possible and likely if in early stages
63
What treatment can be given to early stage Hodgkin's Lymphoma?
Radiotherapy
64
What treatment can be given to advanced Hodgkin's Lymphoma?
Adrimycin Bleomycin Vinblastine Dacarbazine
65
What is Non-Hodgkin Lymphoma?
Malignant clonal expansion of Lymphocytes
66
Which lymphocytes are usually affected in Non-Hodgkin Lymphoma?
B Lymphocytes - 80% | T Lymphocytes - 20%
67
What are some risk factors for developing Non-Hodgkin Lymphoma?
``` Abnormal Response to Viral Infection Bacterial Infection Radiation Drugs - Phenytoin Autoimmune - Sjogrens Immunosuppression ```
68
Which virus is associated with the development of Burkitt's Lymphoma?
Epstein-Barr Virus
69
Which infection is associated with the development of Gastric Lymphoma?
Chronic H.Pylori
70
What are some symptoms of Non-Hodgkin Lymphoma?
Widely disseminated lymphadenopathy Splenomegaly Hepatomegaly Extra-nodal disease is common
71
What may be demonstrated biochemically in NHL?
Anaemia/Pancytopenia due to marrow failure Peripheral blood lymphocytosis Paraproteinaemia/Hypogammaglobulinaemia Raised LDH
72
Which radiological investigations are used for investigation of suspected NHL?
XR CT PETCT
73
What is high grade NHL?
More aggressive, cure is possible
74
What is low-grade NHL?
More indolent, treatable but not curable
75
What are some examples of high grade NHL?
Diffuse Large B Cell Lymphoma | Burkitt Lymphoma
76
What are some examples of low grade NHL?
``` Small lymphocytic Lymphoma Lymphoplasmacytic Lymphoma Follicular Lymphoma Marginal Cell Lymphoma Mantle Cell Lymphoma ```
77
What are some potential treatment options for NHL?
``` Excision Watch and Wait Chemotherapy Radiotherapy Monoclonal Antibody therapy Small Molecules/Targeted Therapy ```
78
What is Chronic Myeloid Leukaemia (CML)?
Myeloproliferative Neoplasm, nearly exclusively in adults with a peak incidence of 40-60y
79
Which mutation is found in 95% of CML cases?
Philadelphia Chromosome - t(9;22)
80
What can CML transform into?
Acute leukaemia or myelofibrosis due to a blast crisis transformation
81
What are some presenting symptoms of CML?
``` Asymptomatic Symptomatic Anaemia Abdominal Discomfort due to Splenomegaly B Symptoms Headaches Bruising Bleeding ```
82
What are some clinical signs suggestive of CML?
``` Pallor Massive splenomegaly Lymphadenopathy if blast crisis Extramedullary soft tissue leukaemic deposits - Chloroma Retinal Haemorrhage ```
83
What is a Chloroma?
Extramedullary soft tissue leukaemic deposits
84
What may bloods show in CML?
Low or normal HB Raised WCC Normal/Abnormal platelet count
85
What may a blood film demonstrate in CML?
Neutrophilia with Myeloid Precursors
86
What may a Bone Marrow aspirate demonstrate in CML?
Increased cellularity, increased myeloid precursors | Cytogenetic reveal 9;22 translocation
87
Which agent is used to manage CML?
Imantinib - Tyrosine Kinase inhibitor
88
How does Imantinib help to manage CML?
Suppresses activity of the fusion protein produced by the Philadelphia Translocation
89
What are some side-effects of Imantinib?
Nausea Headaches Rash Cytopenia
90
How should an acute transformation of CML be managed?
Chemotherapy, then stem cell transplant for remission induction
91
If Imantinib is ineffective in managing CML, how should this be managed?
Allogeneic Stem Cell Transplant

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