Haematology Flashcards

1
Q

What proportion of lymphomas are Hodgkin lymphomas?

A

15%

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2
Q

What proportion of lymphomas are non-Hodgkin lymphomas?

A

85%

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3
Q

What percentage of non-hodgkin lymphomas B cell?

A

> 90%

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4
Q

What is the peak incidence of Hodgkin’s lymphoma?

A

20-24

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5
Q

How do patients with lymphoma generally present?

A

Painless lymphadenopathy
Splenomegaly
Anaemia
B symptoms (pyrexia of unknown origin, night sweats, weight loss)

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6
Q

What are the investigations for a patient with suspected lymphoma?

A

History - symptoms, duration, B symptoms

Examination - lymph nodes, splenomegaly

Bloods - FBCs, U&Es, LFT, Ca
ESR

Imaging - CT scan, PET-CT

Bone marrow biopsy - aspirate and trephine

Additional tests: pulmonary function, ECHO

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7
Q

What staging system is used in Lymphoma?

A

Ann-Arbour Classification System

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8
Q

Define the Ann-Arbour Classification System for Lymphoma

A

Stage 1 = single lymph node group

Stage 2 = >1 lymph node group same side of diaphragm

Stage 3 = Lymph node groups both sides of diaphragm (includes spleen)

Stage 4 = Extranodal involvement

A/B to signify absence/presence of B symptoms

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9
Q

At what stage are most HL diagnosed?

A

Early stage

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10
Q

At what stage are most NHL diagnosed?

A

Advanced stage

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11
Q

Describe Follicular Lymphoma

A
Low grade lymphoma
Median age of diagnosis 65 yrs
Often present at Stage IV 
B symptoms are less common 
Indolent clinical cause 
Usually incurable
Slow growth but reduced apoptosis
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12
Q

What cells do follicular lymphoma resemble?

A

Normal germinal centre cells

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13
Q

What translocation is associated with follicular lymphomas?

A

BCL2 gene (t14:18)

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14
Q

Describe the RRx for follicular lymphomas

A

Aim: relieving symptoms

If early stage - localised radiotherapy (may be curable)

Advanced stage:
Asymptomatic + no end organ compromise - watch and wait

Symptomatic/end organ compromise –> immunochemotherapy

Ritixumab (anti-CD20) + chemotherapy

Maintenance Ritixumab every 2/12 for 2 years

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15
Q

What is the survival of follicular lymphoma like?

A

Survival >15 yrs

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16
Q

Describe Diffuse Large B-cell Lymphoma

A

High grade lymphoma
Associated with various translocations and genetic abnormalities
High proliferation fraction, variable rate of cell death

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17
Q

What do the neoplastic cells in Diffuse Large B Cell Lymphoma resemble?

A

Activated B cells

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18
Q

Describe the presentation of Diffuse Large B cell Lymphoma

A

Lymphadenopathy - rapidly enlarging LN mass

Extra-nodal presentation is common (Waldeyer’s ring, GIT, Skin, Bones, CNS)

B symptoms

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19
Q

Describe the treatment in Diffuse Large B Cell Lymphoma

A

Aggressive chemotherapy with intention to cure

1A: R-CHOP x 3 + radiotherapy

Other stages: R-CHOP x 6

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20
Q

Describe R-CHOP chemotherapy

A
Rituximab
Cyclophosphamide
Adriamycin 
Vincristine
Prednisolone
21
Q

Describe Burkitt Lymphoma

A

High grade lymphoma

Very high rate of proliferation and high rate of apoptosis (tumour lysis syndrome –> release of K –> cardiac arrest)

22
Q

What gene is involved in Burkitt Lymphomas?

23
Q

Describe the presentation of Burkitt lymphoma

A

Short history
Marked B symptoms
Rapidly growing tumour with massive tumour bulk

Presents with extra nodal disease 
Jaw and facial bones
Illeocaecal region of GIT
Ovaries
Kidneys
Breasts
24
Q

Describe the treatment for Burkitt Lymphoma

A

Requires intensive chemotherapy managed in ITU/HDU

25
Describe Classic Hodgkin Lymphoma
High grade lymphoma with prominent component of reactive cells Approximately 40% of cases are associated with EBV infections
26
What do the neoplastic cells in Hodgkin lymphoma resemble?
Atypical activated B cells as seen in some viral infections
27
What is the characteristic pathology cell type seen in Hodgkin lymphoma?
Reed-Steinburg cells
28
What is strongly expressed in Hodgkin Lymphoma?
CD30
29
Describe the presentation of Hodgkin Lymphoma
Peak in 20s and elderly patients Painless lymphadenopathy (may be sore with alcohol consumption) Cough, SOB - mediastinal disease Itch - may precede diagnosis by many months
30
How does Hodgkin lymphoma spread
Initially to immediately adjacent lymph nodes Later, haematogenous spread to liver, lungs, bone marrow
31
How is early stage Hodgkin lymphoma treated?
Chemo + Radio
32
How is late stage Hodgkin Lymphoma treated?
Chemo
33
What Chemo regimen in used to treat Hodgkin lymphoma?
ABVD Adriamycin Bleomycin Vanblastine Dacarbazine
34
When should a person being treated for Hodgkin lymphoma have a PET-CT scan?
After 2nd cycle of chemo Negative = scar tissue not active disease Positive = change regimen
35
Describe plasma cell myeloma
Neoplasm of mature plasma cells with varied clinical cause
36
What do neoplastic cells in plasma cell myeloma resemble?
Normal plasma cells
37
Describe the presentation of plasma cell myeloma
Non-specific symptoms ``` Backache/rib pain Fatigue Symptoms associated with hypercalcaemia Recurrent infections Renal impairment ```
38
Describe paraprotein
In myeloma, abnormal plasma cells produce an abnormal monoclonal protein called paraprotein or M protein 5 different types (IgA etc)
39
What is IgM myeloma associated with?
Waldenstroms macroglobulinaemia
40
What is light chain myeloma?
Only part of the immunoglobulin is produced
41
What is non-secretory myeloma
No Ig is produced
42
What is the classical myeloma triad?
1. Increased plasma cells in bone marrow 2. Clonal immunoglobulin or paraprotein 3. Lytic bone lesions
43
What blood tests should be arranged in suspected myeloma?
FBCs ESR (>100) U&E Ca Serum protein electrophoresis - rouleaux SFLC quantity (serum free light chain)
44
What proteins may be present in the urine of a patient with myeloma?
Bence-Jones proteins (immunoglobulin light chains)
45
What is the diagnostic requirement for myeloma?
Neoplastic plasma cells in bone marrow >10% of total cells AND >1 of: HyperCalcaemia Renal insufficiency Anaemia Bone lesions
46
What biomarkers are associated with myeloma?
Clonal plasm cell % >40% Serum light free chain ratio >100 >1 focal lesion on MRI
47
Describe the treatment of myeloma
Asymptomatic = watch and wait Symptomatic (CRAB criteria) Chemotherapy + steroid + thalidomide Radiotherapy - severe bone pain Bisphosphonates Blood transfusions/EPO
48
Why are bisphosphonates useful in patients with myeloma?
Reduces pain Reduces pathological fractures Reduces hypercalcaemia Reduces need for radiotherapy