Haem: Lymphoma 2, CLL and Lymphoproliferative disorder Pt.1 Flashcards
Describe the typical presentation of lymphoma.
Painless progressive lymphadenopathy
- Can cause compression symptoms e.g. dysphagia if compressing oesophagus
Inflitration symptoms - skin rash, visual changes, neurological deficits
Recurrent infection (BM supression)
Constitutional symptoms (B symptoms)
How is lymphoma diagnosed?
Biopsy with histological WHO classification of lymphoma subtype
Which investigations may be used to stage lymphoma?
- Imaging - PET, CT, MRI
- Bone marrow biopsy
- Lumbar puncture (if CNS involvement)
What are some important tests to perform in lymphoma
and why are they important?
- LDH - marker of cell turnover
- HIV serology - HIV can predispose to NHL (HTLV1 serology may also be important)
- Hepatitis B serology - NHL treatment may deplete B cells resulting in fulminant liver failure due to reactivation of hepatitis B in chronic carriers
What are the 2 main types of Hodgkin’s lymphoma
Classical (95%)
Nodular lymphocyte predominant (5%) - causes recurrent disease in elderly
What is the main type of classical HL and which type has a poor prognosis?
Nodular sclerosing is the main type of classical HL (predominantly affects young women aged 20-30)
Lymphocyte depleted HL is the type that has a poor prognosis
What is the chemotherapy regime used in cHL
ABVD
- Adriamycin
- Bleomycin
- Vinblastine
- Dacarbazine (DTIC)
Given at 4 weekly intervals for 2-6 cycles (dependant on stage and response)
How is HL relapse treated
High dose chemotherapy with PB autologous stem cell transplant
Broadly speaking, what are the treatment approaches to non-Hodgkin lymphoma?
- Monitor only (in indolent lymphoma)
- Urgent chemotherapy
- Non-chemotherapy treatment (e.g. antibiotics to eradicate H. pylori in gastric marginal zone lymphoma)
What are the two most common types of non-Hodgkin lymphoma?
Diffuse large B cell lymphoma (DLBCL) - 30%
Follicular lymphoma - 22%
List some types of non-Hodgkin lymphoma that are:
- Very agressive
- Aggresive
- Indolent
-
Very agressive
- Burkitt’s lymphoma
- T or B cell lymphoblastic lymphoma/leukaemia
-
Aggressive
- Diffuse large B cell lymphoma
- Mantle cell lymphoma
-
Indolent
- Follicular lymphoma
- Small lymphocytic lymphoma (CLL)
- MALToma (marginal zone lymphoma)
What is the correlation between how aggressive a lymphoma is and how curable it is?
The more aggressive it is, the more curable
Indolent lymphoma is more likely to recur
Which factors are taken into account by the international prognostic index (IPI) for lymphoma?
- Age
- Ann Arbor stage
- LDH
- Presence of extra-nodal disease
- ECOG performance status
Which chemotherapy treatment is usually used for diffuse large B cell lymphoma?
- R-CHOP
- Rituximab
- Cyclophosphamide
- Doxorubucin
- Vincristine
- Prednisolone
NOTE: usually 6-8 cycles
NOTE: achieves a 50% cure rate
What treatment option may be considered for patients with diffuse large B cell lymphoma who relapse?
Autologous stem cell transplantation
Which genetic abnormality is associated with follicular lymphoma?
t(14;18) - over-expression of Bcl-2 (anti-apoptosis gene)
NOTE: follicular lymphoma is incurable but is indolent - median survival 12-15 years
What is the usual first-line treatment approach to follicular lymphoma?
Watch and wait
Only treat it clinically indicated (e.g. compression symptoms, massive nodes, recurrent infection)
Which chemotherapy regimen may be used in the treatment of follicular lymphoma?
R-CVP (rituximab, cyclophosphamide, vincristine, prednisolone)
Which lymphoid tissue tends to be affected by marginal zone lymphoma?
Extranodal lymphoid tissue (e.g. MALT)
What is the cause of marginal zone lymphoma?
- H. pylori infection - gastric MALToma
- Sjogren’s syndrome - parotid lymphoma
- Hashimoto’s thyroiditis - thyroid lymphoma
Where is marginal zone lymphoma most commonly seen and how does it tend to present?
- Usually in the stomach
- Presenting with dyspepsia or epigastric pain
- Usually Stage 1{E} (E=extranodal)
- B symptoms are uncommon
Outline the process of MALToma pathogenesis.
- Lymphocytes will respond chronic antigen stimulation from H. pylori infection and proliferate
- At some point, they will over-proliferate and develop cancer-like features but they will still be dependent on antigenic stimulation by H. pylori
- At this point, treating H. pylori will treat the lymphoma
What are the symptoms of early stage gastric MALToma
Epigastric pain, ulceration, bleed
B-symptoms uncommon
How might gastric MALToma stage I-II disease be treated?
- Tripy therapy to eradicate H. pylori (2 antibiotics + 1 PPI)
- Repeat breath test at 2 months
- Repeat endoscopy every 6 months for 1-2 years then annually
NOTE: failure may require chemotherapy
What are the main features of enteropathy-associated T cell lymphoma?
- Mature T cells
- Involves small intestines
- Aggressive
- Caused by chronic antigenic stimulation by gliadin/gluten
