Haem II Flashcards
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
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 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
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
Which of the following areas during B cell development is where B cells start the ability to make immunoglobulins
Bone marrow
Blood
Lymphoid tissue
Marrow
Which of the following areas during B cell development is where B cells start the ability to make immunoglobulins
Bone marrow
Blood
Lymphoid tissue
Marrow
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
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
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
Describe how you differentiate betwen NHL and HL with regards to:
Cells; Age; Extra-nodal disease; Systematic symptoms; contigoius spread [5]
Cells:
* HL: Reed-Sternberg cells (mature B cells) PRESENT
* NHL: Reed-Sternberg cells NOT present; instead get B or T cells at all stages of maturation
Age:
* HL: Bimodal
* NHL: More common with increasing age
Extra-nodal disease:
* HL: Extranodal disease uncommon
* NHL: Extranodal disease common
Contiguous spread:
* HL: Contiguous spread
* NHL: Non-contiguous spread
Systematic symptoms:
* HL: Systemic symptoms common
* NHL: Systemic symptoms not common
Describe the clinical presentation of NHL
- NHL is typically disseminated at presentation, with two-thirds of patients presenting with painless lymphadenopathy: cervical, axillary, inguinal, and femoral lymph nodes are the most commonly involved
- Extranodal involvement is also common, and may affect any organ; the most common sites are the GI tract (stomach, in particular), skin, and bone marrow
- Fever
- Night sweats
- Weight loss (unexplained, > 10% in the last 6 months)
- Pruritus
- Splenomegaly
- Hepatomegaly
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 areas of the body are most commonly affected by NHL (aside from lymphadenopathy) [3]
GI tract (stomach, in particular)
skin
bone marrow
* If bone marrow involvement: cytopenia - anaemia, infections or purpura.
Describe some implications of enlarged LNs due to lymphadenopathy [5]
Enlarging nodes can cause symptoms due to mass effect:
* Compression of the superior vena cava: shortness of breath and facial oedema
* Compression of the external biliary tree: jaundice
* Compression of the ureters: hydronephrosis
* Bowel obstruction: vomiting and constipation
* Impaired lymph drainage: chylous pleural or peritoneal fluid, or lymphoedema of the lower limbs
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 large mediastinal mass, SVC syndrome and cranial nerve palsies
Lymphoblastic lymphoma
Burkitt’s lymphoma
Adult T-cell leukaemia-lymphoma
Anaplastic large cell lymphoma
Lymphoblastic 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
Which symptoms help differentiate between HL and NHL? [3]
- Alcohol-induced pain at sites of nodal disease is specific to HL, but only occurs in < 10% of patients.
- Pruritis is common in HL
- Lymph node involvement occurs in a contiguous manner in HL, but is non-contiguous in NHL
State 5 reasons for NHL oncological emergencies [5]
- Superior vena cava obstruction (SVCO)
- Cord compression
- Hypercalcaemia
- Tumour lysis syndrome
- Neutropenic sepsis
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
When would a lumbar puncture be indicated as a biospy in a NHL patient? [1]
What is meant by Fluorescence in situ hybridisation (FISH) and when is it used? [1] What further tests would you perform if positive? [1]
Lumbar puncture:
- In patients with suspected CNS disease, cytology and flow cytometry may be required.
Fluorescence in situ hybridisation (FISH):
- NICE advise using FISH to identify MYC rearrangement (Burkitt lymphoma) in those with high-grade B-cell lymphoma.
- If found, further testing to identify BCL2 and BCL6 rearrangements and the immunoglobulin partner should be arranged.
What is the commonly used chemotherapy regimen used in NHL? [5]
Describe the MoA of each [5]
R-CHOP:
Rituximab:
* A monoclonal antibody with activity against CD20 - an antigen found on the surface of B-cells.
Cyclophosphamide:
- An alkylating agent, inhibits DNA synthesis through cross linking of DNA.
Doxorubicin:
- An anthracycline that inhibits topoisomerase II leading to inhibition of DNA and RNA synthesis.
Vincristine:
- Inhibits microtubule formation by binding to tubulin.
Prednisolone
- a glucocorticoid steroid.
State common side effects of each of R-CHOP [5]
R-CHOP:
Rituximab:
* infusion reactions
* hepatitis B reactivation
* mucocutaneous reactions
Cyclophosphamide:
- transitional cell carcinoma of the bladder
- bone marrow suppression
- infertility.
Doxorubicin:
- cardiomyopathy
- myelosuppresion
- skin reactions.
Vincristine:
- peripheral neuropathy
- bladder atony.
Prednisolone
- steroid effects
A patient is undergoing treatment for NHL. They are subsequently diagnosed as having transitional bladder cancer. You suspect this is because of their treatment used for their NHL.
Which of the following is most likley to have caused this?
Rituximab
Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone
Cyclophosphamide
Which of the following is most likley to have cause cardiomyopathy?
Rituximab
Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone
Doxorubicin
Which of the following is most likley to have caused bone marrow suppression?
Rituximab
Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone
Which of the following is most likley to have caused bone marrow suppression?
Rituximab
Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone
Which of the following is most likley to have caused peripheral neuropathy?
Rituximab
Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone
Which of the following is most likley to have caused peripheral neuropathy?
Vincristine
Which of the following is most likley to have caused bladder atony?
Rituximab
Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone
Vincristine
Which of the following is most likley to have caused Hep B reactivation
Rituximab
Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone
Rituximab
Which of the following is most likley to cause infertility?
Rituximab
Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone
Which of the following is most likley to cause infertility?
Rituximab
Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone
Which of the following is most likley to cause an infusion reaction?
Rituximab
Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone
Rituximab
Describe the common presentation of the following types of NHL [3]
- Diffuse large B cell lymphoma (DLBCL) [3]
- Follicular lymphoma [1]
- Burkitt’s lymphoma [1]
Diffuse large B cell lymphoma (DLBCL):
- rapidly enlarging mass, commonly in the neck, abdomen or mediastinum
- Extranodal is common in GI tract
- Disease in the mediastinum may lead to SVCO
Follicular lymphoma:
- insidious manner with gradually worsening, painless lymphadenopathy
Burkitt’s lymphoma:
- rapidly enlarging tumour in the jaw of a child
- associated with EBV
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
Describe the treatments for the following types of NHL [3] (in both local and advanced stages diseases)
- Diffuse large B cell lymphoma (DLBCL) [2]
- Follicular lymphoma [2]
- Burkitt’s lymphoma [1]
Diffuse large B cell lymphoma (DLBCL):
* Limited stage disease: R-CHOP; Combined modality therapy (CMT) may be used where chemoimmunotherapy is combined with radiotherapy.
* Advanced stage disease: Treatment may involve R-CHOP or other regimens such as (da)-EPOCH-R.
Follicular lymphoma:
- Limited stages: local radiotherapy
- Advanced disease: immunotherapy with rituximab if asymptomatic. If
symptomatic: RCHOP
Burkitt’s lymphoma
- Intensive chemotherapy
Any patient suffering from NHL who exhibits neutropenia should be given what treatment? [1]
Any patient with severe neutropenia should be given antibiotic prophylaxis with chemotherapy
Describe what is meany by tumour lysis syndrome [1]
What electrolyte disturbances may this cause? [5]
Tumour lysis syndrome commonly occurs after treatment of high-grade lymphomas; when tumor cells release their contents into the bloodstream, either spontaneously or in response to therapy
- hyperuricaemia
- hyperkalaemia
- hyperphosphataemia
- hypocalcaemia
- acute kidney injury
Which of the following is most likley to cause hypogammaglobulinaemia?
Rituximab
Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone
Which of the following is most likley to cause hypogammaglobulinaemia?
Rituximab
A patient undergoes a biopsy of a mass in their ceceum. It exhbitis a ‘starry sky’ pattern. What is the most likely infection? [1]
EBV: causes Burkitt’s lymphoma
Which lymph nodes does Burkitt’s lymphoma typically present in? [1]
Abdomen and mesenteric lymph nodes
Burkitt’s lymphoma typically causes what complication? [1]
Tumour lysis syndrome
Hodgkin’s lymphoma stage III:
Either side of diaphragm
