9- Haematological malignancy (Lymphoma) Flashcards
lymphoma background general
Lymphomas are a group of cancers that affect the lymphocytes inside the lymphatic system- malignnacy of the lymphatic system
- Clonal proliferation of lymphoid lineage
- These cancerous cells proliferate within the lymph nodes and cause the lymph nodes to become abnormally large (lymphadenopathy).
types of lymphoma
1) Hodgkin’s lymphoma
2) Non-Hodgkin’s lymphoma
- Just over 50%
- Broad range of diagnoses (low grade to high grade)
which lineage of cells affected
clonal proliferation of lymphoid cells
presentation of lymphoma
presentation same in both Hod and Non-Hod-> can only be differentiated by nodal biopsy
Palpable mass
- Non-tender (usually)
- Rubbery
- Could also be a sign of metastatic malignancy from another site- more common in adults
B symptoms
* Weight loss
* Night sweats
* Fevers
Others
* Lethargy
* Recurrent infections
* Itchiness
* Abdominal pain
* Anorexia
Mediastinal lymphadenopathy or superior vena cava obstruction may present with
* Cough
* Wheeze
* Difficulty breathing
* Airway compromise
presentation of lymphoma
presentation same in both Hod and Non-Hod-> can only be differentiated by nodal biopsy
Palpable mass
- Non-tender (usually)
- Rubbery
- Could also be a sign of metastatic malignancy from another site- more common in adults
B symptoms
* Weight loss
* Night sweats
* Fevers
Others
* Lethargy
* Recurrent infections
* Itchiness
* Abdominal pain
* Anorexia
Mediastinal lymphadenopathy or superior vena cava obstruction may present with
* Cough
* Wheeze
* Difficulty breathing
* Airway compromise
Hodgkins lymphoma background
- Overall 1 in 5 lymphomas are Hodgkin’s lymphoma.
- It is caused by proliferation of lymphocytes.
- There is a bimodal age distribution with peaks around aged 20 and 75 years.
RF Hodgkins lymphoma
- HIV
- Epstein-Barr Virus
- Autoimmune conditions such as rheumatoid arthritis and sarcoidosis
- Family history
metastasis of Hodgkins lymphoma
- Liver
- Lungs
- Bone marrow
Investigations for Hodgkins lymphoma
Bloods
- FBC (bone marrow function), UEs, LFTs
- Haemitinics (ferritin)
- Bone profile
- Virology (CMB, EBV, HIV),
- Immunoglobulins (IgM, IgG)
- Blood film
- Lactate dehydrogenase (LDH) is a blood test that is often raised in Hodgkin’s lymphoma but is not specific and can be raised in other cancers and many non-cancerous diseases.
Lymph node core biopsy is the key diagnostic test.
The Reed-Sternberg cell is the key finding from lymph node biopsy in patients with Hodgkin’s lymphoma. They are abnormally large B cells that have multiple nuclei that have nucleoli inside them. This can give them the appearance of the face of an owl with large eyes. The Reed-Sternberg cell is a popular feature in medical exams.
Bone marrow biopsy may be useful
Imaging
- CT, MRI and PET scans can be used for diagnosing and staging lymphoma and other tumours.
key finding of Hodgkin lymphoma from core biopsy
Reed- Sternberg Cell
- X5 larger
- Owl sign
- Popcorn sign
staging of Hodgkin Lymphoma
Ann-Arbor staging
Ann-Arbor staging
The Ann Arbor staging system is used for both Hodgkins and non-Hodgkins lymphoma. The system puts importance on whether the affected nodes are above or below the diaphragm. A simplified version is:
Stage 1: Confined to one region of lymph nodes.
Stage 2: In more than one region but on the same side of the diaphragm (either above or below).
Stage 3: Affects lymph nodes both above and below the diaphragm.
Stage 4: Widespread involvement including non-lymphatic organs such as the lungs or liver.
Each stage subdivided into A or B
A= no systemic symtoms other than pruritius
B= presence of B symptoms
e.g. Stage 1B - would be lymphoma confined to one region of lymph nodes with the presence of B symptoms (weight loss, night sweats etc)
management of Hodgkin lymphoma
The key treatments are chemotherapy and radiotherapy.
ABVD
The aim of treatment is to cure the condition. This is usually successful however there is a risk of relapse, other haematological cancers and side effects of medications.
chemotherapy used in Hodgkin lymphoma
ABVD
- Adriamycin
- Bleomycin
- Vinblastine
- Dacarbazine
Complications of Hodgkin lymphpoma treatment
key: infertility and secondary cancers e.g. Leukaemia
prognosis of Hodgkin lymphoma
better prognosis than Non-Hodg
- more curable
Non-Hodgkins lymphoma background
Another group of lymphomas. There are almost endless types of lymphoma.
- More common than Hodgkins
- Occur in older people e.g. mid 50s
Types (over 60 diff)
1) B cell lymphoma
e.g. Diffuse large B-cell lymphoma
e.g. Follicular lymphoma (most common)
2) T cell lymphoma
e.g. Peripheral T-cell lymphoma
e.g. Skin lymphomas
Risk factors for non-Hodgkin’s lymphoma include:
- HIV
- Epstein-Barr Virus
- H. pylori (MALT lymphoma)
- Hepatitis B or C infection
- Exposure to pesticides and a specific chemical called trichloroethylene used in several industrial processes
- Family history
the spectrum of NHL
Ranges from:
- Indolent low-grade lymphomas that are incurable, yet compatible with a number of years of survival
- To aggressive high-grade lymphomas that, left untreated, are rapidly fatal, but which modern treatment can cure in a significant proportion of patients
- Divided into low grade and high grade (more aggressive but more curable)
difference between presentations in HL and NHL
Hodgkin is rarely extra nodal, vs N-H which is extranodal
o Hodgkin’s
- No hepatosplenomegaly
- No leukemic phase
o Non- Hodgkin
- Hepatosplenomegaly
- Leukemic phase
extranodal sites of NHL
- Stomach
- Spleen
- Waldeyer ring
- Central nervous system
- Lung
- Bone
- Skin
- Thryoid
- Breast
- Testes
medical emergencies in presentation of NHL due to extranodal involvement
- Spine cord compression
- Hypercalcaemia
- Ascites and Pleural effusions are common end-stage features
medical emergency often seen in follicular lymphoma (Hodgkin)
o Mediastinal obstruction
o Obstructive nephropathy
obstructive nephropathy in lymphoma
due to retroperitoneal mass which causes renal ureter obsturction -> hydronephrosis -> AKI
investigations: USS
management: nephrostomy
investigations for Hodgkin lymphoma
Bloods
- FBC (bone marrow function), UEs, LFTs
- Haemitinics (ferritin)
- Bone profile
- Virology (CMB, EBV, HIV),
- Immunoglobulins (IgM, IgG)
- Blood film
- Lactate dehydrogenase (LDH) is a blood test that is often raised in Hodgkin’s lymphoma but is not specific and can be raised in other cancers and many non-cancerous diseases.
Lymph node core biopsy is the key diagnostic test.
Bone marrow biopsy may be useful
Imaging
*CT, MRI and PET scans can be used for diagnosing and staging lymphoma and other tumours.
management of Non- Hodgkin Lymphoma:
If low grade
- Watchful waiting
If higher grade thats curable
- Chemotherapy (CHOP)
- Monoclonal antibodies such as rituximab
- Radiotherapy
- Stem cell transplantation
Chemotherapy for NHL
R- CHOP
- Rituximab
- Cyclophosphamide
- Doxorubicin hydrochloride (hydroxydaunorubicin)
- Vincristine sulfate (Oncovin)
- Prednisone
side effects of monoclonal antibodies such as rituximab
reactivation of virus e.g. Hep, infusion reaction e.g. hypotension
concern in patients starting chemo for lymphoma
tumour lysis syndrome
NHL example case:
tranformed to a higher grade lymphoma -> will now require treatment
Hodgkins vs Non-Hodgkins
Similarities
- Both cancers originate in lymphatic systems
- Similar presentation
o Fatigue
o Weight loss
o Anorexia
o Fever
o Night sweats
differences between HL and NHL
- Non-Hodgkin is more common than Hodgkins
- Hodgkins has a much better prognosis
Biopsy - Hodgkin lymphoma- Reed-Sternberg cells
- Non-H – no Reed-sternberg cells
Median age - Hodgkin- 39
- Non-Hodgkin- 67
Involement
Hodgkin is rarely extra nodal, vs N-H which is extranodal
management of SVCO
- Emergency if mediastinal mass with airway compromise – treat with high dose steroids and airway support if required
- SVCO may also require stenting
