Lymphoma Flashcards
1
Q
What are some of the different causes of lymphadenopathy ?
A
- Lymphoma
- Infection (viral, bacterial).
- Metastatic Cancer eg breast, ovarian.
- Connnective tissue disease (sarcoidosis);SLE.
2
Q
What is the problem with the symptoms of lymphoma ?
A
Some of the symptoms such as night sweats and weight loss can be caused by a number of different things so it can be difficult to decide if it is lymphoma causing them or something else:
- Night sweats: lymphoma, infection, menopause, too thick a duvet/central heating.
- Wt loss: Lymphoma, other malignancy, infections etc.
3
Q
What type of lymphadenopathy distribution does bacterial infections and viral infections cause?
A
- Bacterial infections tend to cause a more regional lymphadenopathy
- Viral infections tend to cause a more generalised lymphadenopathy
4
Q
What is the differences in lymphadenopathy felt for a viral, bacterial, lymphoma or metastatic cancer cause ?
A
5
Q
What are the two main types of lymphoma?
A
Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL)
6
Q
What are the main classifications of lymphoma in practice that you need to know about ?
A
- Hodgkin’s Lymphoma.
- High grade B cell NHL.
- Low grade B cell NHL.
- Burkitt’s Lymphoma. – very aggressive
- Mantle cell Lymphoma. – look low grade but actually they are horrendously aggressive and not cureable
- Marginal Zone NHL.- very slow changing and often only observation
7
Q
Describe how hodgkins lymphoma develops
A
- In Hodgkin lymphoma, B-lymphocytes start to multiply in an abnormal way and begin to collect in certain parts of the lymphatic system, such as the lymph nodes (glands).
- The affected lymphocytes lose their infection-fighting properties, making you more vulnerable to infection.
8
Q
What is the most common way hodgkins lymphoma presents ?
A
Often presents with enlarged, painless, non-tender, ‘rubbery’ superficial lymph nodes typically in the cervical, also axillary or inguinal lymph nodes
9
Q
What are the general features of hodgkins lymphoma ?
A
- B symptoms - weight loss, fever, night sweats
- Cough or dyspnoea
- Persistent itching of the skin all over the body
- Normocytic anaemia - fatigue, tirdeness etc
- Increased risk of infections
- In some cases, people with Hodgkin lymphoma experience pain in their lymph glands when they drink alcohol - very suggestive sign
- Excessive bleeding e.g. nosebleeds, heavy periods & purpura
- Other symptoms will depend on where in the body the enlarged lymph glands are. For example, if the abdomen (tummy) is affected, you may have abdominal pain or indigestion
10
Q
What are some of the additional signs which may be present in someone with hodgkins lymphoma ?
A
- Cachexia – weakness and wasting of the body
- Anaemia
- Splenohepatomegaly
11
Q
What may happen if medistinel nodes become involved in someone with lymphoma ?
A
- Bronchial or SVC obstruction may cause ariway obstruction
- Or pleural effusions
12
Q
Who is most commonly affected by hodgkins lymphoma
A
Mostly affects young adults in their early 20s and older adults over the age of 70.
13
Q
What are the risk factors for developing hodgkins lymphoma?
A
- you have a medical condition that weakens your immune system e.g. HIV (more for non-hodgkins tho)
- you take immunosuppressant medication e.g. taking immunosuppressant after organ transplantation
- Exposure to Epstein-Barr virus (EBV) – which causes glandular fever
- A first degree relative has been affected by lymphoma
- Previously has non-hodgkin lymphoma
14
Q
What are the main types of hodgkins lymhoma ?
A
Classical Hodgkin lymphoma is classified into the following 4 types:
- Nodular sclerosing Hodgkin lymphoma (NSHL)
- Mixed-cellularity Hodgkin lymphoma (MCHL)
- Lymphocyte-depleted Hodgkin lymphoma (LDHL)
- Lymphocyte-rich classical Hodgkin lymphoma (LRHL)
A fifth sub type, Nodular lymphocyte-predominant Hodgkin lymphoma (Reed- Sternberg cells are rarely seen in this one)
15
Q
How is hodgkins lymphoma diagnosed ?
A
- Excisional biopsy of a complete lymph node which then undergoes histological analyses e.g. fine needle and core biopsies are often insufficient
16
Q
What are the different tests done to assess the lymph node and bone marrow pathology ?
A
- Histology-microscopic appearances.
- Immunohistochemistry for solid node.
- Immunophenotyping for blood/marrow.
- Genetic analysis.
- Molecular analysis.
17
Q
What is the use of immunohistochemistry ?
A
- Confirming it is a lymphoma, and helping to subclassify.
- Looks at pattern of proteins on the surface of lymphoma cells.
18
Q
What is the use of cytogenetic studies +/- FISH?
A
- Many lymphomas have chromosomal abnormalities - for example, translocations - that can be helpful for diagnosis and prognosis.
- FISH is useful in confirming diagnoses of follicular lymphoma (14;18 translocation), mantle cell lymphoma (11;14 translocation), and Burkitt’s lymphoma (8;14 translocation).
- FISH is also useful in identifying C-MYC translocations which predict more aggressive behaviour of diffuse large B-cell lymphoma and transformed follicular lymphoma.
- Consider using FISH (fluorescence in situ hybridisation) to identify a MYC rearrangement in all people newly presenting with histologically high‑grade B‑cell lymphoma.
19
Q
What is the use of molecular analysis ?
A
- Patterns of gene expression.
- Multiple analyses looking at patterns of genes that are switched both on an off.
- Helping to further classify lymphoma and identify subtypes suitable for specific treatment.
20
Q
If lymph node biopsy confirms hodgkins lymphoma what further tests are needed to stage the cancer?
A
- Blood tests – FBC, film, LFT, LDH, Urate Ca2+, ESR
- CXR
- PET-CT scan (co-administered) of chest, abdo & pelvis +/- bone marrow biopsy
21
Q
What characteristic type of cell seen on analyses of the biopsy is seen in hodgkins lymphoma ?
A
Reed sternberg cells – pic saved as reed sternberg cells
22
Q
What are the main stages of hodgkins lymphoma ?
A
- Stage 1 – the cancer is limited to one group of lymph nodes, such as your neck or groin nodes either above or below your diaphragm (the sheet of muscle underneath the lungs)
- Stage 2 – two or more lymph node groups are affected, either above or below the diaphragm
- Stage 3 – the cancer has spread to lymph node groups above and below the diaphragm (present on both sides)
- Stage 4 – the cancer has spread through the lymphatic system and is now present in organs e.g. liver or bone marrow
Note - “A” is put after your stage if you have no additional symptoms other than swollen lymph nodes. “B” is put after your stage if you have additional symptoms of weight loss, fever or night sweats.
23
Q
What is the treatment of the classical types of hodgkins lymphoma ?
A
Chemo + radiotherapy:
- Start of with ABVD + radiotherapy regime for higher stage give longer course of chemo
- ABVD = Adriamycin, Bleomycin, Vinblastine and Dacarbazine
- ABVD for stage III-IV typically get 6-8 cycles of chemo
- stage I-II typically get 2-4 cycles of chemo
- Escalate to BEACOPP regime + radio if not able to get to remission with ABVD regime
- BEACOPP = Bleomycin, Etoposide, doxorubicin (also called adiramycin), Cyclophosphamide, Oncovin (also called vincritstine), Procarbazine, Prednisolone
- BEACOPP associated with more toxicity and a higher risk of secondary acute leukaemias
24
Q
What is the treatment of Stage I-II nodular lymphocyte predominant HL?
A
Radiotherapy alone
25
What is the treatment of Stage III-IV nodular lymphocyte predominant HL:
Chemo and radiotherapy – ABVD regime
26
What is the treatment of refractory or replapsed HL?
* The goal is still to cure here
* High-dose chemotherapy (with radiotherapy for eligible patients), followed by autologous stem cell transplantation (ASCT) is the preferred approach
27
Describe the development of non-hodgkins lymphoma (NHL)
* In non-Hodgkin lymphoma, the affected lymphocytes start to multiply in an abnormal way and begin to collect in certain parts of the lymphatic system, such as the lymph nodes (glands).
* The affected lymphocytes lose their infection-fighting properties, making you more vulnerable to infection.
* Non-Hodgkin's lymphoma may affect either B or T-cells and can be further classified as high grade or low grade.
28
Which is more common - hodgkins lymphoma or NHL?
NHL
29
What are the 2 main classes of non-hodgkins lymphoma ?
B and T cell types NHL
30
What is the 2 main categories of B cell NHL?
1. Low grade - incurable
2. High grade - curable
31
What are the different types of low grade NHL?
**Follicular lymphoma** is the **most common** type of low grade NHL. Other types include:
* mantle cell lymphoma – behaves very aggresively so treated more like a high grade lymphoma
* marginal zone lymphoma
* small lymphocytic lymphoma
* lymphoplasmacytic lymphoma
* skin lymphoma
32
What is the most common symptom of NHL?
* A painless swelling in a lymph node, usually in the neck, armpit or groin.
33
Who is usually affected by NHL?
* Can occur at any age but most cases diagnosed in people over 65
34
What are the risk factors to developing non-hodgkins lyphoma ?
* Elderly
* Caucasians
* History of viral infection (specifically Epstein-Barr virus)
* Family history
* Certain chemical agents (pesticides, solvents)
* History of chemotherapy or radiotherapy
* Immunodeficiency (transplant, HIV, diabetes mellitus)
* Autoimmune disease (SLE, Sjogren's, coeliac disease)
35
What are the symptoms of NHL ?
* Painless lymphadenopathy (non-tender, rubbery, asymmetrical)
* Constitutional/B symptoms (fever, weight loss, night sweats, lethargy)
* Extranodal Disease - gastric (dyspepsia, dysphagia, weight loss, abdominal pain), bone marrow (pancytopenia, bone pain), lungs, skin, central nervous system (nerve palsies)
36
While differentiating Hodgkin's lymphoma from non-Hodgkin's lymphoma is done by biopsy, what certain elements of the clinical presentation can help point towards one rather than the other?
* Lymphadenopathy in Hodgkin's lymphoma can experience alcohol-induced pain in the node
* 'B' symptoms typically occur earlier in Hodgkin's lymphoma and later in non-Hodgkin's lymphoma
* Extra-nodal disease is much more common in non-Hodgkin's lymphoma than in Hodgkin's lymphoma
37
What are the signs of NHL?
* Signs of weight loss
* Lymphadenopathy (typically in the cervical, axillary or inguinal region)
* Palpable abdominal mass - hepatomegaly, splenomegaly, lymph nodes
* Testicular mass
* Fever
38
How is NHL diagnosed ?
Lymph node excisional biopsy like in hodgkins but also would do a bone marrow biopsy
39
How is NHL staged ?
CT chest, abdomen and pelvis
40
What is the staging for NHL?
* I: Single lymph node group
* II: Multiple lymph node groups on same side of diaphragm
* III: Multiple lymph node groups on both sides of diaphragm
* IV: Extra-nodal involvement e.g. Spleen, bone marrow or CNS
The stage is combined with the letter A or B to indicate the presence of 'B' symptoms. With the letter A indicating no B symptoms present and B indicating any of the beta symptoms present.
41
What are the main types of high grade NHL?
Types of high grade lymphoma include:
* **diffuse large B cell lymphoma (DLBCL)** – most common one
* **Burkitt lymphoma**
* peripheral T cell lymphoma
* lymphoblastic lymphoma
* blastic NK cell lymphoma
* enteropathy associated T cell lymphoma (EATL)
* hepatosplenic gamma delta T cell lymphoma
* treatment related T cell lymphomas
42
Describe the key features additional features (ontop of those for NHL) that would be suggestive in a question of burkitts lymphoma
* African patient
* Causes characteristic jaw lymphadenopathy
* Microscopy - 'starry sky' appearance: lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells
43
What are gastric MALT lymphomas primarily associated with ?
H. pylori infection in 95% of cases
44
What is the treatment of burkitt's lymphoma ?
**Chemotherapy**
* If you have **low-risk Burkitt lymphoma**, a common regimen is 3 cycles of R-CODOX-M (rituximab, cyclophosphamide, vincristine, doxorubicin, methotrexate).
* If you have **high-risk Burkitt lymphoma**, a common regimen is 2 cycles of R-CODOX-M alternating with 2 cycles of R-IVAC (rituximab, ifosfamide, etoposide, cytarabine).
* Granulocyte colony stimulating factors (G-CSF) given to help prevent netropenia e.g. filgrastim or lenograstim
45
Burkitts lymphoma tends to have a rapid response to chemotherapy, what is the risk of this rapid response however and what is give to reduce this risk ?
Tumour lysis syndrome - Rasburicase often give to reduce risk of it
46
What are the potential complications of tumour lysis syndrome ?
Complications of tumour lysis syndrome include:
* hyperkalaemia
* hyperphosphataemia
* hypocalcaemia
* hyperuricaemia
* acute renal failure
47
What is the treatment of diffuse large B cell lymphoma/high grade lymphoma or mantle cell lymphomas?
* RCHOP – rituximab, cyclophosphamide, hydroxydanuorubicin, vincristine (oncovin), prednisolone
* The risk factors for CNS relapse include being HIV-positive; stages III and IV and/or testicular, paranasal sinus, epidural, adrenal, kidney, or bone marrow involvement; and high LDH. – if present also give methotrexate
* growth factor (G-CSF) should be given to older patients at risk of neutropenia
48
What is the treatment of marginal zone /MALT cell lymphoma?
* 1st line = H.pylori eradication - amoxicillin, clarithromycin, metronidazole
* 2nd line if no response = chemo with bendamustine and ratuximab
49
What is the treatment of low grade B cell lymphomas (primarily follicular cell lymphomas)?
Stage I-II give radiotherapy
Stage III-IV give chemo from the options below:
1. RCHOP + growth factor for older people
2. Bendaamustine and ratuximab
3. R CVP – rituximab, cyclophosphmaide, vincristine (oncovin) and prednisolone
Rituximab maintenance therapy is recommended as an option for the treatment of people with follicular non‑Hodgkin's lymphoma that has responded to first‑line induction therapy with rituximab in combination with chemotherapy
50
What is the treatment of replased low grade lymphoma?
* RCHOP +/- stem cell transplant
51
What is the treatment of T cell lymphoma ?
CHOP chemo +/- stem cell transplantation
52
What are the potential complications of lymphomas?
* Bone marrow infiltration causing anaemia, neutropenia or thrombocytopenia
* Superior vena cava obstruction
* Metastasis
* Spinal cord compression
* Complications related to treatment e.g. Side effects of chemotherapy
53
What is the prognosis of hodgkins & NHL?
* Low-grade non-Hodgkin's lymphoma has a better prognosis
* High-grade non-Hodgkin's lymphoma has a worse prognosis but a higher cure rate
