Haem III (HL; NHL) Flashcards
Describe what is meant by Hodgkin’s lymphoma
Uncontrolled proliferation of B-lymphocytes, causing lymphadenopathy
The infection of which pathogen is linked to / often precedes HL? [1]
Epstein-Barr Virus
State 4 risk factors for HL [4]
EBV
Immunosuppression:
* Organ transplantation
* Immunosuppressant therapies
* Patients with HIV
Autoimmune conditions
* rheumatoid arthritis
* systemic lupus erythematosus
* sarcoidosis
Familial
Which autoimmune conditions increase the liklihood of HL? [3]
- rheumatoid arthritis
- systemic lupus erythematosus
- sarcoidosis
What are the two types of HL? [2]
Classical Hodgkin’s lymphoma (95%)
Nodular lymphocyte-predominant Hodgkin’s lymphoma (5%)
Describe the features of nodular lymphocyte-predominant Hodgkin’s lymphoma (5%) [4]
- More commonly affects males (75%)
- Not associated with EBV
- Absence of Reed-Sternberg (RS) cells, and is characterised by LP (“popcorn”) cells
- Presents with peripheral adenopathy, and is the only type of Hodgkin’s lymphoma that affects the mesenteric lymph nodes
Which of the following types of classical HL is the most common?
Lymphocyte-depleted
Nodular sclerosis
Lymphocyte-rich
Mixed cellularity
Nodular sclerosis
What are the 4 subtypes of classical Hodgkins Lymphoma? [4]
Nodular sclerosis:
- Most common subtype, accounts for around 70% of cHL.
Mixed cellularity:
- Accounts for around 20% of cHL.
Lymphocyte-rich:
- Accounts for around 5% of cHL. Has the best prognosis.
Lymphocyte-depleted:
- Rare, accounting for < 1% of cHL. Has the the worst prognosis.
Which of the following types of classical HL has the worst prognosis?
Lymphocyte-depleted
Nodular sclerosis
Lymphocyte-rich
Mixed cellularity
Which of the following types of classical HL has the worst prognosis?
Lymphocyte-depleted
Which of the following types of classical HL has the best prognosis?
Lymphocyte-depleted
Nodular sclerosis
Lymphocyte-rich
Mixed cellularity
Which of the following types of classical HL has the best prognosis?
Lymphocyte-rich
Which of the following types of classical HL is most associated with HIV patients?
Lymphocyte-depleted
Nodular sclerosis
Lymphocyte-rich
Mixed cellularity
Lymphocyte-depleted
Which of the following types of classical HL is most associated with older patients
Lymphocyte-depleted
Nodular sclerosis
Lymphocyte-rich
Mixed cellularity
Which of the following types of classical HL is most associated with older patients
Lymphocyte-rich
Which of the following types of classical HL is most associated with mediastinal lymphadenopathy and bulk nodes?
Lymphocyte-depleted
Nodular sclerosis
Lymphocyte-rich
Mixed cellularity
Nodular sclerosis
Which of the following types of classical HL is most associated with peripheral adenopathy and spleen involvement?
Lymphocyte-depleted
Nodular sclerosis
Lymphocyte-rich
Mixed cellularity
Mixed cellularity
Which of the following types of classical HL is most associated with retroperitoneal adenopathy and abdominal lymphadenopathy?
Lymphocyte-depleted
Nodular sclerosis
Lymphocyte-rich
Mixed cellularity
Which of the following types of classical HL is most associated with retroperitoneal adenopathy and abdominal lymphadenopathy?
Lymphocyte-depleted
Which of the following types of classical HL are most asscoiated with EBV? [2]
Lymphocyte-depleted
Nodular sclerosis
Lymphocyte-rich
Mixed cellularity
Mixed cellularity
Lymphocyte-depleted
How do you stage lymphomas? [1]
Lecture content
PET scans
Describe the classical clinical features of HL [5]
Lymphadenopathy:
- Painless
- Non-tender and firm / rubbery
- Asymmetrical
- Cervical or mediastinal involvement
- Pain in lymph nodes after drinking alcohol
Describe the additional clinical features of HL than lymphadenopathy [5]
Pel-Ebstein fever
- cyclical fever followed by periods of being afebrile for 1-2 weeks (rare)
Abdominal pain (if abdominal lymphadenopathy is involved)
Pruritus (30%)
Clinical hepato/splenomegaly is rare (although liver and spleen involvement determined by laparoscopy/laparotomy occurs in up to 30% of the patients)
Bone marrow involvement (5-8%)
systemic - ‘B symptoms’ (25%)
* weight loss
* pruritus
* night sweats
* fever (Pel-Ebstein)
Describe the Cotswolds-modified Ann Arbor staging system for HL [5]
Stage I - limited to a single lymph node region.
Stage II - two or more lymph node regions involved, on the same side of the diaphragm.
Stage III - lymph node regions involved on both sides of the diaphragm.
Stage IV - spread to other organs, such as liver, spleen, or bone marrow, with or without nodal involvement.
Each stage is followed by a letter:
- A - without B symptoms.
- B - with B symptoms, including fever, weight loss, night sweats.
- E - an extra-nodal site involved, that is localised to the known lymph node region.
- X - bulky disease: mediastinal mass exceeds 1/3 of the intrathoracic diameter or nodal mass >10cm in dimension.
Describe the treatment plan for Stage I/II classical Hodgkin’s lymphoma [2]
and
Stage III/ IV classical Hodgkin’s lymphoma [2]
Stage I/II classical Hodgkin’s lymphoma:
- Chemotherapy followed by radiotherapy
- Main treatment regimen: ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) + radiotherapy
Stage III/ IV classical Hodgkin’s lymphoma:
- Chemotherapy alone: ABVD or BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, prednisolone)
Describe the treatment plan for Stage I/ II nodular lymphocyte-predominant Hodgkin’s lymphoma: [1]
and
Stage III/ IV nodular lymphocyte-predominant Hodgkin’s lymphoma [2]
Stage I/ II nodular lymphocyte-predominant Hodgkin’s lymphoma:
- Involved-field radiotherapy alone is the main treatment for this group of patients with excellent clinical benefit.
Stage III/ IV nodular lymphocyte-predominant Hodgkin’s lymphoma:
- R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone) is administered in symptomatic patients or those with rapid disease progression .
- Regular monitoring is adequate in asymptomatic patients.
Describe the management plan for HL in refractory disease [2]
Chemotherapy followed by autologous stem cell transplantation (ASCT)
Immunotherapeutic agents can be considered:
- Brentuximab vedotin
- nivolumab
- pembrolizumab
doxorubicin has a risk of causing which cardiac abnormality? [1]
doxorubicin causes a higher risk of developing cardiomyopathy.
Describe the common complcations of HL [6]
Complications of Hodgkin’s lymphoma are usually chemotherapy or radiotherapy related.
Thyroid:
- Around 50% of the patients who received radiotherapy experience symptoms of hypothyroidism.
- thyroid cancer and hyperthyroidism also possible
Chemotherapy drugs,: especially alkylating agents are associated with secondary malignancies:
- acute myeloid leukaemia
- paraneoplastic syndrome
Cardiac abnormalities:
- doxorubicin are at a higher risk of developing cardiomyopathy
- acute pericarditis shortly after receiving radiotherapy
- valvular heart disease or coronary heart disease in the long-term after radiotherapy
Pulmonary toxicity:
- bleomycin-related pulmonary toxicity
- Radiation-induced pneumonitis
Infertility :(
Infections:
- Neutropenia is common in patients taking chemotherapeutic regimens such as ABVD, and almost all patients receiving BEACOPP are neutropenic.
How do you differentiate between leukaemia and lymphoma from a FBC? [1]
Leukaemias tend to have high circulating serum WCCs
Lymphomas tend not to have circulating cells; have solid tumours; enlarged LN with high WCC
Describe the pathway of where normal B lymphocyte development occurs [4]
B cells:
- Bone marrow
- Blood (as niave B-lymphocytes)
- Lymphoid tissue (e.g. spleen / LNs / liver); devell
- (back to the) Bone Marrow and become plasma cells
Through which process do T lymphocytes make new TCR for antigens [1]
T cells actively mutate their DNA via VDJ recombination to make a unique T cell receptor for new antigens as immature T cells
Describe the process of somatic hypermutation [2]
B cells transit in peripheral blood to germinal centres
May detect an antigen, but often this is not specific, so they undergo somatic hypermutation.
- Rapid and aggressive mutation of DNA to try and improve specificity and recognition of their receptor for the antigen
- Go back to bone marrow, become plasma cells and make specific antibodies
During B-lymphocyte development, cells start to make antibodies in the lymphoid tissue, before they move back to bone marrow.
What Ig do they make in the lymphoid tissues?
IgA
IgG
IgM
IgE
IgD
During B-lymphocyte development, cells start to make antibodies in the lymphoid tissue, before they move back to bone marrow.
What Ig do they make in the lymphoid tissues?
IgA
IgG
IgM
IgE
IgD
Describe the process of normal T cell development [4]
State and explain which organs are commonly affected in chronic lymphomas [3]
Lymphadenopathy
Splenomegaly
Hepatomegaly
Because they’re all germinal centres - which is and important stage in developement in lymphomas
Also get B-symptoms
State where the following generally occur: [4]
State for the following if they’re generally aggressive or slow [4]
- ALL
- CLL
- Lymphomas
- Myelomas
ALL:
- Bone marrow
- Aggressive
CLL:
- Blood
- Slow
Lymphomas:
- Lymphoid tissue
- Agrresive (high grade); slow (low grade)
Myelomas:
- BM
- Slow
How can you work out if a lymphoma is aggressive or low grade?
Low grade lymphomas quite often have pathological descriptions of the cells (e..g follicular lymphoma)
High grade lymphomas are named
For lymphomas, what type of testing do you normally perform for
- Diagnostic test
- Prognostic test
- Staging test
Diagnostic test:
- Biopsy; morphology; phenotyping tests
- Sometimes supported by cytogenetic / molecular
Prognostic test:
- Cytogenetic test
Staging test:
- Usually imaging
What is the name for this skin condition related to NHL? [1]
Mycosis fungoides: skin lesions including an eczematous reaction which proceeds to form plaques, tumours, and fungating ulcers.
Which of the following are associated with large abdominal mass and symptoms of bowel obstruction
Lymphoblastic lymphoma
Burkitt’s lymphoma
Adult T-cell leukaemia-lymphoma
Anaplastic large cell lymphoma
Burkitt’s lymphoma
Which of the following are associated with:
fulminating clinical course with skin infiltrates, lymphadenopathy, hepatosplenomegaly, and leukaemia. May also be associated with symptoms of hypercalcemia
Lymphoblastic lymphoma
Burkitt’s lymphoma
Adult T-cell leukaemia-lymphoma
Anaplastic large cell lymphoma
Adult T-cell leukaemia-lymphoma
Describe the initial investigations used to investigate NHL [5]
FBC:
- anaemia
- thrombocytopenia
- neutropenia
- lymphocytosis
U&Es:
- acute kidney injury from obstructive nephropathy
LDH:
- Often elevated in high grade lymphomas
Chest x-ray:
- mediastinal adenopathy
- pleural or pericardial effusions and parenchymal involvement
MRI brain / spinal cord if neurological symptoms
Which of the following is most likley to cause an infusion reaction?
Rituximab
Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone
Rituximab
This patient most likely has
Diffuse large B cell lymphoma (DLBCL)
Follicular lymphoma
Burkitt’s lymphoma
Burkitt’s lymphoma
This patient most likely has
Diffuse large B cell lymphoma (DLBCL)
Follicular lymphoma
Burkitt’s lymphoma
Diffuse large B cell lymphoma (DLBCL)
suffering from SVCO
