Lymphoma (Hodgkin's and non-Hodgkin's) Flashcards
Define lymphoma.
Neoplasms of the lymphoid cells, originating in lymph nodes, lymphoid organs, skin or rarely “anywhere”.
Define Hodgkin’s lymphoma.
An uncommon haematological malignancy arising from mature B cells. It is characterised by the presence of Hodgkin’s cells and Reed-Sternberg cells.
Define non-Hodgkin’s lymphoma.
Malignancy of lymphoid cells originating in LNs without Reed Sternberg cells and consisting of 85% B cell, 14% T cell and NK cell forms, ranging from indolent to aggressive disease and referred to as low, intermediate and high grades.
What is the epidemiology of Hodgkin’s lymphoma?
Bimodal age distribution peaks at 20-30yrs and >50yrs
More common in males
Annual European incidence is 2-5 /100,000
What is the epidemiology of NHL?
Incidence increases with age
More common in males
More common in West
What is the aetiology of Hodgkin’s lymphoma? What are the risk factors?
50% of cases associated with EBV but pathogenesis unknown
Unknown aetiologies
What is the aetiology of NHL? What are the risk factors?
Associated with EBV, HIV, SLE, Sjogren’s, HBV, HCV
Oncogenic viruses can introduce foreign genes and cause NHL.
Radiotherapy, immunosuppressive agents, chemotherapy.
- EBV –> Burkitt’s lymphoma and AIDs associated lymphomas
- HTLV-1 –> ATLL
- HHV-8 –> body-cavity-based lymphomas
What is the typical presentation of Hodgkin’s lymphoma?
- Painless enlarging mass (most often in the neck, occasionally in axilla or groin)
- May become painful after alcohol ingestion
- Skin excoriations
- Signs of intrathoracic disease (e.g. pleural effusion, SVC obstruction)
- B symptoms - fever >38 (if cyclical referred to as Pel–Ebstein fever), night sweats, weight loss (>10% in last 6 months)
- Examination: non-tender lymphadenopathy, splenomegaly +/- hepatomegaly
What is the typical presentation of NHL?
- Painless enlarging mass - neck/axilla/groin -
- Systemic symptoms - fever, night sweats, weight loss>10% body weight, symptoms of hypercalcaemia.
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Symptoms related to organ involvement - extranodal disease more common than in cHL
- Gastric (dyspepsia, dysphagia, weight loss, abdominal pain), bone marrow (pancytopenia, bone pain), lungs, skin, central nervous system (nerve palsies)
- Establish performance status of patient
Signs:
- Examination: painless, rubbery lump. Oropharyngeal (Waldeyer’s ring lymph nodes) involvement.
- Skin rashes - mycosis fungoides (well defined indurated scaly plaque-like lesions with raised ulcerated nodules caused by cutaneous T-cell lymphoma) and Sezary’s syndrome
- Abdominal mass
- Hepatosplenomegaly
- Signs of bone marrow involvement - anaemia, infections, purpura.
What does presence of B symptoms imply?
Poor prognosis -
- weight loss > 10% in last 6 months
- fever > 38ºC
- night sweats
How do you diagnose Hodgkin’s lymphoma?
Lymph node biopsy under microscopy = Reed Sternberg cells aka Owl’s eyes
Bloods:
- FBC - anaemia of chronic disease, leukocytosis, raised neutrophils, raised eosinophils. Lymphopaenia with advanced disease.
- ESR/CRP - raised
- LFT -raised LDH, raised transaminases
Imaging:
- CXR
- CT thorax, abdo, pelvis
- Gallium scan
- PET scan
Bone marrow aspirate and trephine biopsy - involvement seen only in very advanced disease
What staging system is used for Hodgkin’s lymphoma?
Ann Arbour staging
- I Single lymph node region.
- II Two or more lymph node regions on one side of the diaphragm.
- III Lymph node regions involved on both sides of diaphragm.
- IV Extranodal involvement (liver or bone marrow).
- A Absence.
- B Presence of B symptoms.
- E Localized extranodal extension.
- S Involvement of spleen.
What are the histological subtypes of Hodgkin’s lymphoma?
- Nodular sclerosing (70%);
- Mixed cellularity (20%);
- Lymphocyte predominant (5%);
- Lymphocyte depleted (5%).
What cells are pathognomonic of Hodgkin’s lymphoma?
The Reed–Sternberg cell is pathognomonic. It is a large cell with abundant pale cytoplasm and
two or more oval lobulated nuclei containing prominent ‘owl-eye’ eosinophilic nucleoli
(can appear as lacunar or ‘popcorn’ cells).
What is the name for the cyclical fever sometimes seen in Hodgkin’s lymphoma?
Pel-Ebstein fever
What investigations would you do for NHL?
Bloods:
- FBC - anaemia, neutropenia, thrombocytopenia (if BM involved)
- U&E, uric acid
- CRP and ESR - raised
- LDH -raised
- LFTS - raised transaminases with liver involvement
- Ca2+ - may be high
- HIV, HBV, HCV serology
Blood film - Hodgkin cells (aka Reed-Sternberg cells)
Lymph node biopsy (DIAGNOSTIC) and…
- Histology - evaluation of architecture and cells
- Immunophenotyping - CD markers and expression of abnormal proteins; clonality of B cells
- Cytogenetics - FISH or PCR for chromosome translocations
Imaging - CXR, CT thorax, abdomen, pelvis, CT plus PET (in extranodal involvement)
Staging - as for Hodgkin’s (Ann Arbour for removed lymph node)
What is the most common subtype of lymphoma?
Diffuse large B-cell lymphoma is the most common type of lymphoma.
What is the difference between lymphoma and leukaemia?
Leukemia and lymphoma are both forms of blood cancer. The main difference is that leukemia affects the blood and bone marrow, while lymphomas tend to affect the lymph nodes.
Which subtype of NHL has a starry sky appearance under microscopy? How does it present?
- Burkitt’s lymphoma
- Usually in an African child
- Large LN in the jaw which is fast growing