Haem Malignancy- Lymphoma Flashcards
What are Lymphomas?
Lymphomas are a group of cancers that affect the lymphocytes inside the lymphatic system.
These cancerous cells proliferate within the lymph nodes and cause the lymph nodes to become abnormally large (lymphadenopathy).
Hodgkin’s lymphoma and non-Hodgkin’s lymphoma. HL is a specific disease and non-Hodgkins lymphoma encompasses all the other lymphomas.
zero to finals
What are Lymphomas?
Lymphomas are a group of cancers that affect the lymphocytes inside the lymphatic system.
These cancerous cells proliferate within the lymph nodes and cause the lymph nodes to become abnormally large (lymphadenopathy).
Hodgkin’s lymphoma and non-Hodgkin’s lymphoma. HL is a specific disease and non-Hodgkins lymphoma encompasses all the other lymphomas.
zero to finals
What is Hodgkin’s lymphoma?
a malignant proliferation of B lymphocytes characterised by the presence of the Reed-Sternberg cell.
Epideimiology of Hodgkins lymphoma?
Bimodal age distribution with peaks around aged 20 and 75 years.
Prognosis for Hodgkins lymphoma?
Overall- 75% of patients with HL survive for ten years or more.
Prognosis is better in the younger population than in the older population.
* < 40 yearshave a five-year survival rate of 95%
* > 70 years have a five-year survival rate of < 50%
What is the lymphatic system?
- network of tissues and organs that play a key role in the immune system.
- made up of: lymph nodes, lymphatic vessels, lymphatic organs and lymphatic fluid.
- mutations in lymphocytes inside lymphoid tissues results in a lymphoma.
- In HL - B lymphocytes, (derived from the germinal centres of lymphoid tissues), mutate and lead to large, multi-nucleated giant cells - ‘Reed-Sternberg’ cells and large, mono-nucleated cells called malignant ‘Hodgkin cells’.
How is Hodgkins lymphoma classified?
Nodular Sclerosing - most common
table probs overkill
Is Nodular lymphocyte-predominant Hodgkin lymphoma dangerous?
- Nodular lymphocyte-predominant Hodgkin lymphoma is indolent, low grade - not much in common with classical HL
- HOWEVER: there is a RISK it can TRANSFORM into a high grade (rapidly growing) non-Hodgkin lymphoma
What are some risk factors for Hodgkins lymphoma?
- EBV / glandular fever
- HIV
- Immunosupression (also autoimmune drugs e.g. RA that require immunosupressive drugs)
- Ciagarette smoking
- Previous non-hodgkins lymphoma
- First degree relative with Non-Hodgkins or chronic lymphocytic leukaemia
What would expect to hear in history of a patient with Hodgkin’s lymphoma?
MOST COMMON:
* painless, rubbery, enlarged lymph node/nodes, in the cervical or supraclavicular region.
B symptoms:
* fever >38°C
* drenching night sweats
* unintentional weight loss ( >10% in 6 months)
* Chest discomfort +/- cough or dyspnoea e.g. a mediastinal mass
* Abdominal pain: e.g. liver / spleen
* Alcohol-induced pain at nodal sites
* Pruritis
* Malaise
* Fatigue
What clinical examination would you do if suspect Hodgkin’s lymphoma? give findings too
DO a lymphoreticular system examination.
On examination:
- Lymphadenopathy
- Hepatomegaly
- Splenomegaly
- Superior vena cava (SVC) syndrome: a mediastinal mass may cause SVC obstruction
- Paraneoplastic syndromes such as cerebellar degeneration, neuropathy or Guillain-Barré syndrome
Differencials for Hodgkins lymphoma?
- Infectious mononucleosis
- Non-Hodgkin lymphoma
- Acquired immunodeficiency syndrome (AIDS)
- Tuberculosis
- Sarcoidosis
- Leukaemia
- Myeloma
- Toxoplasmosis
Lab investigations for Hodgkins lymphoma?
FBC: platelets
* leukaemia, infectious mononucleosis and other causes of lymphadenopathy,
* WCC / CRP /
* Ca and bone profile - rule out myeloma
U&Es:
* baseline before treatment
LFTs:
* reduced albumin levels are associated with a poorer prognosis
LDH:
* increased levels are associated with a poorer prognosis
ESR:
* increased levels are associated with a poorer prognosis
Tests to exclude differential diagnoses:
* Monospot® test for infectious mononucleosis
* sputum culture for TB
* viral screen including HIV, hepatitis B virus (HBV) and hepatitis C virus (HCV) tests
Imaging for hodgkin’s lymphoma?
Chest X-ray:
* intrathoracic lymphadenopathy and mediastinal expansion
Contrast CT neck, chest, abdomen and pelvis:
* when pt first presents
(PET-CT):
* staging in cHL
* repeated during treatment to allow guided therapy according to response (aiming to minimise toxicity by reducing chemotherapy intensity when possible)
Specialist investigations for hodgkins lymphoma? and findings?
- lymph node excision biopsy for diagnosis and classification - (US or CT guided)
- Light microscopy -Reed-Sternberg cells - giant multinucleated cell. Hodgkin cells are giant malignant mononucleated cells surround RS cells
Immunocytochemistry
* CD15 adn Cd30 antigens are expressed on Reed-Sternberg cells
Bone marrow biopsy
* less frequently needed as PET CT can detect BM involvement
Staging system for Hodgkins lymphoma?
Ann Arbor staging system
Prior to starting treatment for Hodgkins lymphoma what do patients need?
due to the potential SE of chemo and radiotherapy
* cardiac function testing
* pulmonary function testing
* reproductive counselling
Increased risk of opportunistic infections vaccinate:
- Polyvalent pneumococcal vaccine
- Influenza vaccine
- Meningococcal group C conjugate vaccine
- Haemophilus influenzae type b vaccine
What factors indicate a poor prognosis in hodgkins lymphoma?
- age > 45 years
- stage IV disease
- haemoglobin < 10.5 g/dl
- lymphocyte count < 600/µl or < 8%
- male
- albumin < 40 g/l
- white blood count > 15,000/µl
treatment of hodgkins lymphoma?
- chemotherapy is the mainstay of treatment. Two combinations may be used
- ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine): considered the standard regime
- BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone): alternative regime with better remission rates but higher toxicity
- - radiotherapy
- combined modality therapy (CMT)
- chemotherapy followed by radiotherapy
- hematopoietic cell transplantation
- may be used for relapsed or refractory classic Hodgkin lymphoma
Blood transfusion cautions in patient with hodgkins lymphoma?
If a transfusion of blood products is required, patients with or treated for Hodgkin lymphoma (at any stage of the disease) must only receive irradiated blood products. This is a lifelong requirement.
Irradiated blood products are used to reduce the risk of transfusion-associated graft-versus-host disease.
Disease related complications of hodgkins lymphoma?
Hodgkin lymphoma causes immunosuppression.
The clonal expansion of B lymphocytes are abnormal and do not function properly.
Patients are at a particularly higher risk of infection if there is bone marrow involvement.
Treatment related complications of hodgkins lymphoma?
- Neutropenia:
- neutropenic sepsis
- Secondary solid tumours: e.g. lung, skin, breast and GI
- Secondary leukaemias: AML
- Subfertility: counsel before treatment
- CVS disease: due to adriamycin/doxorubicin
- Lung fibrosis: due to bleomycin and presents months to years after treatment
- Endocrine dysfunction
- Neuropathy
- Nausea and vomiting
- Hair loss
What is non-hodgkins lymphoma?
Non-Hodgkin’s lymphoma is an umbrella term for a group of malignancies affecting the lymphoid system.
It is defined as all lymphomas without Reed-Sternberg cells.
How many types of non-hodgkins lymphoma?
- 30-60 types
classified according to:
* cell of origin (B-cell or T-cell)
or
* pathological grade (high-grade or low-grade).
reg said 60, quesmed says 30
