Lymphoma Flashcards

1
Q

Define lymphoma

A

Cancer of lymph nodes

- effects the main cells in B cell maturation

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2
Q

what cells are effected in lymphoma

A
  • 90% B cell

- 10% T and NK cell

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3
Q

What is the clinical presentation of lymphoma

A
  • Indolent/low grade

- Aggressive/high grade

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4
Q

What are the two types of lymphoma

A

Hodgkin and non-Hodgkin lymphoma

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5
Q

what lymphoma is more common

A

Non-hodgkin lyphoma

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6
Q

What are the common types of non Hodgkin lymphoma

A
  • Diffuse large B cell lymphoma – most common (High grade)
  • Burkitt lymphoma (High grade)
  • Follicular lymphoma (Low grade)
  • Marginal zone lymphoma (Low grade)
  • Lymphocytic lymphoma (Low grade)
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7
Q

What are the classical types of Hodgkin and other types of Hodgkin lymphoma

A

Classical types

  • Nodular sclerosing - most common
  • Mixed cellularity
  • Lymphocyte rich
  • Lymphocyte depletes - only one with a poor prognosis

Other types
- Nodular lymphocyte predominant

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8
Q

What can cause lymphomas

A

Can be caused/Associated with/ by viral infections

  • EBV- Hodgkin and diffcule large B cells
  • HIV
  • HTLV
  • Hep C
    • HHV8

Bacterial infections

  • H.pylori – treatment can involve H.pylori removal
  • Chlamydia psittaci

Inflammatory conditions

  • Coeliac disease
  • Sjogren’s syndrome

Industrial/medical exposure

  • Ionising radiation
  • Benezene
  • Immunosuppression
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9
Q

What are the 5 different types of classification of lymphoma

A
  • Mature B cell expansion
  • Hodgkin lymphoma
  • Histiocytic and dendritic cell neoplasma
  • Mature T and NK neoplasma
  • Posttransplant lymphoproliferative disorders
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10
Q

What investigations are used in lymphoma

A
  • Blood tests
  • Biopsy
  • Imaging
  • Disease specific tests
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11
Q

What are the symptoms of lymphoma

A
  • Fever – greater than 38 degrees
  • Night sweats
  • Greater than 10% weight loss
  • Can present with no symptoms
  • Fatigue
  • Pain/swelling from lymphadenopathy
  • Pruritus – itching, sometimes you can get skin rashes
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12
Q

What is the characteristic cell that is present in Hodgkin’s lymphoma

A
  • Cells with mirror imaging age nuclei are found – these are called Reed-Sternberg cells
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13
Q

What are the signs of lymphoma

A
  • Hepatomegaly
  • Lymphadenopathy – neck, axillae, abdomen, groin
  • Skin
  • Other targeted organ specific
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14
Q

What blood tests are used for lymphoma

A
  • FBC and film
  • Immunophenotyping – looking at the CD markers that tell you whether a blood type is one or another
  • LDH, uric acid
  • Serum electrophoresis
  • B2 microglobulin
  • Serology; Hepatitis B and C, HIV
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15
Q

Where do you do a biopsy in lymphoma

A
  • Lymph node

- Bone marrow

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16
Q

describe how to get a lymph node biopsy

A
  • Core/excision preferred

- Not a fine needle as don’t get a sense of core architecture, do in core and even more in excision

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17
Q

describe when you would get a bone marrow transplant

A
  • Not needed when PET scan imaging suggests bone marrow uptake
  • Only in PET negative when it might change treatment or management
  • Does change management if you have bone marrow involvement
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18
Q

What imaging do you use for lymphoma

A
  • CT neck, chest, abdominal, pelvis
  • CT PET preferred
  • MRI if CNS involvement
  • Abdominal USS optional
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19
Q

Define the Ann Arbor classification

A

Stage 1 – one node above diaphragm

Stage II – more than one node above diaphragm

Stage III – more than one node above the diaphragm and one node below

Stage IV – multiple nodes above and below and the diaphragm and bone marrow involvement

A= no associated symptoms

B = unexplained fever, night sweats and weight loss greater than 10% in the last 6 months

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20
Q

Name the B symptoms in lymphoma

A
  • weight loss >10% in the last 6 months
  • unexplained fever >38 degrees
  • night sweats
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21
Q

what are the cytogenic for follicular lymphoma

A

follicular lymphoma t(14:18)

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22
Q

what are the markers for follicular lymphoma

A

CD10 particularly, CD79a, bcl2, bcl6, cd10, MYC, KI67, IRF4, Cyclin D1, CD5, CD23

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23
Q

name a marker on the B cell

A
  • B cell CD20 positive
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24
Q

What are the marker for Hodgkin lymphoma

A

CD15, CD30

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25
how does the lymphocyte look in follicular lymphoma
- Cleaved nucleus | - clumped chromatin
26
what does a biopsy look like in follicular lymphoma
- Darker smaller centrocytes – predominate – follicular lymphoma - Larger paler centroblasts – follicular lymphoma
27
What does the bone marrow biopsy look like in follicular lymphoma
- Paratrabecular infiltration of small lymphocytes characteristic
28
what should you evaluate before you decide to treat follicular lymphoma
- Prognosis stage, stage, FLIPI 1/2 grade - Symptoms - Patient priority, longer survival, long remission, better quality of life
29
What should the treatment for follicular lymphoma be in - asymptomatic - mild symptoms - High tumour burden
Asymptomatic - Watch and weight Mild symptoms - Non chemotherapy - Treatment - Rituximab - Radioimmunotherapy High tumour burden - Chemoimmunotherapy - R-CHOP - R- CVP - Consider rituximab maintenance - R- Bendamustine
30
What are the risk factors for follicular lymphoma
- Age over 60 years - Elevated LDH (high tumour burdern) or B2- microglobulin - Advanced according to Ann Arbor classification - Bone marrow involvement - Haemoglobin less than 12g/dl - Diffuse or disseminated extra lymphatic organ involvement - 6-15 blasts/high power field
31
What is the pathophysiology for follicular lymphoma
- Secondary genetic alterations are needed for transporation to follicular lymphoma - T (14:18) and transportation into the germinal centre transform the disorder into follicular lymphoma
32
what is the median age of diagnosis of follicular lymphoma
- Male : female = equal | - Median age of diagnosis: 65years
33
what type of lymphoma is follicular lymphoma
- Type of lymphoma: indolent, low grade, hard to cure
34
what stage of presentation does follicular lymphoma usually present
- Presence of B symptoms: uncommon | - Stage at presentation: most stage III or IV
35
what is the prognosis of follicular lymphoma
- Prognosis: 5 year overall survival low risk– 90%, high risk – 50%
36
describe diffuse large B cell lymphoma
- Highly aggressive but highly curable
37
What does the blood film look like in diffuse large B cell lymphoma
- Normal segmented neutrophil | - In advanced cases there might be lymphoma cells in the blood
38
What does the biopsy look like in diffuse large B cell lymphoma
- Effacement with centroblasts lack of other cells/ inflammatory infiltrate - Diffuse large B cell as there is just centroblasts with no centrocytes and no other cells in that biopsy
39
What is the treatment of diffuse large B cell lymphoma
- R-CHOP (Rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone) x 6 - Given every 14-21 days, for 6 cycles - 1st relapse – platinum based chemotherapy and consolidation with autologous transplantation – take cells, give high dose chemotherapy, put cells back - 2nd relapse – allogenic transplantation/clinical trial - Special considerations – CNS prophylaxis in high risk disease
40
What does R-CHOP stand for
``` Rituximab, cyclophosphamide, doxorubicin, vincristine prednisolone ```
41
What are the two main types of diffuse large B cell lymphoma and what has better prognosis
- Germinal centre type – better prognosis (BCL2 pathway) | - Activated B cell type – worse prognosis (NF- Kappa B pathway)
42
what is the median age of diffuse large B cell lymphoma
- Median age is 64
43
What is the stage at presentation of diffuse large B cell lymphoma
- Presence of B symptoms – common | - Stage at presentation I-II
44
What is the prognosis of diffuse large B cell lymphoma
- Prognosis – low risk >90%, high risk – 50% for 5 years
45
List the statistics for diffuse large B cell lymphoma
- Incidence 7/1000,000 – 30-40% of non hodkgin lymphoma - More males - Type of lymphoma – aggressive/high grade and potentially curable
46
What does the blood film look like in Classical Hodgkin lymphoma (nodular sclerosing type)
- Normal
47
What does the biopsy look like in Classical Hodgkin lymphoma (nodular sclerosing type)
- Bilobed – owls eyes appearance = reed Sternberg cell (not exclusive but it is this type of cell in the presence of inflammatory infiltration and eosinophils that makes the diagnosis of classical Hodgkin lymphoma) - Lacunar cells - Inflammatory infiltration/fibrosis - Eosinophil
48
What is the treatment pathway for Classical Hodgkin lymphoma (nodular sclerosing type)
- Get ABVD treatment no mater what stage – get 2 cycles - ALL stage 2 x ABVD chemotherapy - ABVD – doxorubicin, bleomycin, vinblastine, dacarbazine - Then you get a PET scan - PET scan positive – escalate chemotherapy, involved node radiotherapy - PET scan negative – continue chemo, involved node radiotherapy - Special considerations – breast screening in females less than 40 at time of chest axillary radiation
49
What does ABVD stand for
doxorubicin bleomycin vinblastine dacarbazine
50
what is the pathophysiology for Classical Hodgkin lymphoma (nodular sclerosing type)
- Germinal centre cell, normally a transforming event and you get a reed Sternberg cell, loss of apoptosis, and cytokine infiltration leading to an inflammatory micro environment, this feeds back on the reed Sternberg cell
51
What is the median age of Classical Hodgkin lymphoma (nodular sclerosing type)
- Mediationa ge of diagnosis – bimodal so two peaks one in the mid twenties and one in the mid 70s
52
what is the stage of presentation of Classical Hodgkin lymphoma (nodular sclerosing type)
- Presence of B symptoms 40% | - Stage at presentation – most stage I/II
53
What is prognosis of Classical Hodgkin lymphoma (nodular sclerosing type)
- Aggressive but curable in 80% of patients | - Prognosis – 80-90% cured
54
What are the statistics of Classical Hodgkin lymphoma (nodular sclerosing type)
- 2-4/100,000 - 10% of all lymphoma - Slight male predominance
55
how do you assess the response to treatment in Classical Hodgkin lymphoma (nodular sclerosing type)
- Assessment at 6-8 weeks at the completion of treatment – clinical assessment, PET-CT scan Follow up - 3 monthly for the first 1-2 years - Then 6 monthly - Yearly from 5 years
56
list some new treatments being used for lymphoma
Anti CD20 mab - Rituximab - Ofatunmab Kinase inhibitors - Ibrutinib HDAC inhibitros - Romidepsin Alkylaotrs - Bendamustine BCL2 inhibitors - Venetoclax Checkpoint inhibitors - Nivolumab - Pembrolizumab
57
List differences between Hodgkin and Non-hodking
- Non-Hodgkin lymphoma is more common than Hodgkin lymphoma. - The majority of non-Hodgkin patients are over the age of 55 when first diagnosed, whereas the median age for diagnosis of Hodgkin lymphoma is 39. - Non-Hodgkin lymphoma may arise in lymph nodes anywhere in the body, whereas Hodgkin lymphoma typically begins in the upper body, such as the neck, chest or armpits. - Hodgkin lymphoma is often diagnosed at an early stage and is therefore considered one of the most treatable cancers. - Non-Hodgkin lymphoma is typically not diagnosed until it has reached a more advanced stage.
58
What is the most common type of Hodgkin's lymphoma
- Nodular sclerosing
59
What is the type of Hodgkin lymphoma with the poorest prognosis
- Lymphocyte depleted
60
What are the symptoms and signs for a Hodgkin lymphoma
Symptoms - Enlarged non-tender, rubbery superficial lymph nodes – cervical also axillary or inguinal - E.g. fever, weight loss, night sweats, pruritus’ and lethargy - Mediastinal lymph node involvement can cause mass effect e.g. bronchial or SVC obstruction or direct extension e.g. causing pleural effusions Signs - Lymphadenopathy - also cachexia, anaemia, splenomegaly or hepatomegaly
61
What tests do you carry out for a Hodgkin lymphoma
- Lymph node excision biopsy if possible - Image guided needle biopsy, laparotomy or mediastinoscopy - FBC, blood film, ESR, LFT, LDH, urate and calcium - Raised ESR or lowered HB indicate a worse prognosis - Imaging – CXR, CT/PET of thorax, abdominal and pelvis
62
What chemotherapy do you use for Hodgkins lymphoma
- ABVD = Adriamycin (doxorubicin), Bleomycin, Vinblastine, Dacarbazine = cures 80% of patients
63
What are the complications of treatment for Hodgkin lymphoma
- Radiotherapy may increase risk of secondary malignancies – solid tumours in lung and breast - Ischaemic heart disease, hypothyroidism and lung fibrosis due to the radiation field - Chemotherapy side effects include = myelosuppression, nausea, alopecia, infection - AL, non-Hodgkin’s lymphoma and infertility may be due to chemo and radiotherapy
64
What are the signs and symptoms for a non-hodgkin lymphoma
- Superficial lymphadenopathy - Extra nodal disease – Gut is most common - Gastric MALT is caused by H.pylori and may regress with its eradication - Fevers, night sweats, weight loss - Pancytopenia from marrow involvement – anaemia, infection, bleeding (decreased platelets)
65
What tests do you do for non-Hodgkin lymphoma
- Blood = FBC, U&E, LFT, increased LDH means that there is a worse prognosis - Marrow and node biopsy for classification - Staging – Ann Arbor system – CT+/- PET of chest, abdomen and pelvis
66
What are the types of grades of non-hodgkin lymphoma that you can get and which ones have better prognosis
- Low grade = if symptomless none may be needed = better prognosis - High grade = R-CHOP - Rituximab, Cyclophosphamide, Hydroxydaunorubicin, vincristine (Oncovin®) and Prednisolone.
67
What are the complications of non-hodgkin lymphoma
- Bone marrow infiltration causing neutropenia, anaemia, thrombocytopenia - Superior vena cava obstruction - Metastasis - Spinal cord compression - Complications related to treatment e.g. side effects of chemotherapy
68
What is the difference in treatment for Hodgkin V non Hodgkin Lymphoma
Hodgkin - ABVD = doxorubicin, bleomycin, vinblastine, dacarbazine Non- Hodgkin - RCHOP = rituximab, cyclophophosphamide, doxorubicin, vincristine, prednisolone