Lymphoma Flashcards
What is lymphoma?
Malignant proliferation of lymphocytes (B and T cells) which accumulate in lymph nodes or other organs
What are the different types of lymphoma?
- Hodgkin’s Lymphoma→ malignant lymphoma of B-cell origin. Uncommon malignancy. Characterised by presence of reed-sternberg cells.
- M>F, bimodal age distribution 1st peak 25-30 years and 2nd peak 50-70 years - Non-Hodgkin’s Lymphoma → every other type of lymphoma that is not HL (ie. no reed-sternberg cells)
- May affect B or T cells. Much more common than HL.
- Incidence increases with age
What are the causes of Hodgkin’s lymphoma?
- UNKNOWN
- Likely to be an environmental trigger in a genetically susceptible individual
- EBV genome has been detected in 50% of Hodgkin’s lymphomas
- strong association with Epstein-Barr Virus, immunodeficiency and autoimmune disease (eg. RA, sarcoidosis)
What are the different sub-types of Hodgkin’s lymphoma?
- Nodular sclerosing:
- most common
- good prognosis
- more common in women
- asssociated with lacunar cells - Mixed cellularity
- around 20%
- good prognosis
- associated with a large number of reed- sternberg cells - Lymphocyte predominant
- around 5%
- best prognosis - Lymphocyte depleted
- rare
- worst prognosis
What are the causes of Non-Hodgkin’s Lymphoma?
Infectious associations include:
1. Helicobacter pylori – gastric MALT (mucosa-associated lymphoma tissue) lymphoma
2. Epstein–Barr virus – Burkitt lymphoma (high-grade NHL) and AIDS-related CNS lymphoma
3. hepatitis C virus – diffuse large B-cell lymphoma and splenic marginal zone lymphoma
4. human T-cell lymphotropic virus type 1 (HTLV1) – T-cell lymphoma
Other aetiological associations include:
1. immunodeficiency states (eg. HIV/AIDS and post-organ transplant)
2. autoimmune disorders (eg. Sjögren’s disease and coeliac disease)
3. inherited disorders affecting DNA repair (eg. ataxia telangiectasia and Fanconi anaemia)
What are the risk factors for Hodgkin’s lymphoma?
- Affected sibling
- SLE
- Post-transplantation
- Westernisation
- Obese
- Epstein–Barr virus
- HIV
- Immunosuppression
- Cigarette smoking
What are the presenting symptoms of Hodgkin’s lymphoma?
- Painless enlarging mass – enlarged, painless, non-tender, rubbery, superficial
o Most commonly in the neck
o Can also be in the axilla or groin - The mass may become painful after alcohol ingestion
- Mediastinal lymph node involvement can cause mass effect e.g. bronchial/SVC obstruction
- B symptoms of Lymphoma (systemic)
- Fever > 38 degrees
- If this is cyclical it is referred to as Pel-Ebstein fever
- Night sweats
- Weight loss > 10% body weight in the past 6 months - Other symptoms
o Pruritis
o Cough
o Dyspnoea
What signs of Hodgkin’s lymphoma can be found on physical examination?
- Non-tender firm rubbery lymphadenopathy (may be cervical, axillary or inguinal)
- Splenomegaly (or sometimes, hepatosplenomegaly)
- Cachexia
- Anaemia – pallor
- Skin excoriations
- Signs of intrathoracic disease (e.g. pleural effusion, superior vena cava obstruction)
What investigations are used to diagnose/ monitor Hodgkin’s lymphoma?
- Bloods
a. FBC: high WCC
* Anaemia of chronic disease
* Leucocytosis
* High neutrophils
* High eosinophils
* Lymphopaenia in advanced disease
b. High ESR and CRP
c. High LDH (released during cell turnover)- MOST IMPORTANT, high LDH is a poor prognostic marker - Lymph Node Biopsy – will show REED-STERNBERG cells
- Bone Marrow Aspirate and Trephine Biopsy – can spread to bone marrow in late stage
- Imaging - CXR, CT, PET
What criteria is used to stage Hodgkin’s lymphoma?
“Ann Arbor Staging”
o I = single lymph node region
o II = 2+ lymph node regions on one side of the diaphragm
o III = lymph node regions on both sides of the diaphragm
o IV = extranodal/ organ involvement e.g. liver/bone marrow
o Each stage is either A/B +/- subscript A/E
o A = absence of B symptoms except pruritis
o B = presence of B symptoms – worse prognosis
o E = localised extranodal extension
o S = involvement of spleen
How is Hodgkin’s lymphoma managed?
Treatment is usually with chemoradiotherapy
a. Classic stage 1 disease can be managed with radiotherapy, with combination chemotherapy given for the more advanced stages
- The most commonly used chemotherapy is ABVD = Adriamycin (doxorubicin), bleomycin, vinblastine and dacarbazine
b. Relapsed patients can be salvaged with second-line chemotherapy or an autologous transplant (transplantation in which stem cells are removed from a person, stored, and later given back to that same person), especially if the relapse occurs >1 year after completion of treatment
- A common second-line treatment is ESHAP (etoposide, cytosine arabinoside, methylprednisolone (high dose steroids) and cisplatin)
Describe the prognosis of Hodgkin’s lymphoma
long-term prognosis for patients with early favourable HL is excellent with long-term disease control of 85% to 90% after brief chemotherapy followed by low-dose involved-field radiotherapy. Signs of poor prognosis include B-symptoms, increasing age (>45), male, stage 4 disease and lymphocyte depleted subtype.
What are the risk factors for Non-Hodgkin’s lymphoma?
o Radiotherapy
o Immunosuppressive agents
o Chemotherapy
o HIV, HBV, HCV
o Connective tissue disease (e.g. SLE)
Summarise the epidemiology of non-Hodgkin’s lymphoma
- Incidence increases with age
- More common in MALES
- More common in the WESTERN WORLD
What are the presenting symptoms of non-Hodgkin’s lymphoma?
- Painless enlarging mass (in neck, axilla or groin) – superficial lymphadenopathy- In contrast to Hodgkin lymphoma, the lymphadenopathy is more likely to be symmetrical, at multiple sites, and spread discontinuously across nodal sites
- Systemic Symptoms (occurs less frequently than in Hodgkin’s):
o Fever
o Night sweats
o Weight loss > 10% body weight
o Symptoms of hypercalcaemia - Symptoms related to organ involvement
o Extranodal disease is MORE COMMON in NHL than in Hodgkin’s lymphoma
o Skin rashes
o Headache
o Sore throat
o Abdominal discomfort
o Testicular swelling
