Lymphoma 2 Flashcards
What is the presentation of lymphoma?
Painless progressive lymphadenopathy Infiltrate/impair an organ system Recurrent infections Constitutional symptoms Coincidental
How may painless lymphadenopathy be detected?
Palpable node
Extrinsic compression of any ‘tube’ e.g. ureter, bile duct, large blood vessel, bowel, trachea, oesophagus
What are the diagnosis and staging steps of lymphoma?
Histological diagnosis Anatomical stage (scans) Blood tests (baseline, give effect of lymphoma on the patient)
What is the key malignant cell in Hodgkin Lymphoma?
Reed Sternberg cells
What are the more common patient characteristics of Hodgkin lymphoma?
Bimodal age incidence - age 20-29, smaller peak affecting >60
More common in males
What are the subcategories of classical Hodgkin Lymphoma?
Nodular sclerosing
Mixed cellularity
Lymphocyte rich/depleted (rare)
What is stage I for HL?
One group of nodes
What is stage II for HL?
> 1 group of nodes same side of diaphragm
What is stage III for HL?
Nodes above and below the diaphragm
What is stage IV for HL?
Extranodal spread
What does suffix A/B mean in HL staging?
A is none, B is any of
Fever
Unexplained weight-loss
Night sweats
What are the characteristics of sclerosing subtype of HL?
Young women (>men) 20-29yrs
Neck nodes and mediastinal mass
May have B symptoms
Needs a tissue diagnosis
What is the treatment of HL?
Chemotherapy
ABVD 2-6 cycles
+/- radiotherapy
What is the role of radiotherapy in HL?
Results in low/negligible risk of relapse within field
What is Non-Hodgkin Lymphoma?
Neoplastic proliferation of lymphoid cells
What are the steps of management of Non Hodgkin Lymphoma?
Stage disease
Prognostic markers
Plant therapy
How is NHL staged?
CT scan
PET scan (indicated in aggressive lymphomas)
BM biopsy
Lumbar puncture (if risk of CNS involvement
What are the two most common types of non Hodgkin Lymphoma?
Follicular lymphoma
Diffuse large B cell lymphoma
What is the clinical behaviour of Burkitt Lymphoma and T or B cell lymphoblastic leukaemia/lymphoma
Very aggressive
What is the clinical behaviour of Diffuse large B cell and mantle cell lymphoma?
Aggressive
What is the clinical behaviour of follicular, small lymphocytic/CLL and mucosa associated (MALT)?
Indolent
What is the prognosis of Diffuse Large B cell NHL determined by?
IPI (International Prognostic Index) Age Stage (Ann Arbor) LDH Extra-nodal disease sites ECOG performance status
What is the genetic association of follicular NHL?
t(14;18) which results in over expression of bcl2 an anti-apoptosis protein
What is the median survival of follicular NHL?
12-15yrs
What are the management options for follicular lymphoma?
Watch and wait
Treatment
What would be indications for treatment while watching and waiting follicular NHL?
Nodal extrinsic compression: bowel, Bile duct, ureter, vena cava
Massive painful nodes
Recurrent infections
What is the treatment for follicular NHL?
Combination immuno-chemotherapy
What is MZL?
Marginal Zone Lymphoma involving extra-nodal lymphoid tissue (e.g. Gastric mucosa-associated lymphoid tissue MALT/H. Pylori, Parotic MZL/Sjogren syndrome)
What is enteropathy associated T cell lymphoma (EATL)?
T cell NHL seen in patients with Coeliac disease
Has an aggressive clinical course
What is the presentation and clinical course of EATL?
Abdominal pain, obstruction perforation, GI bleeding
Malabsorption
Systemic symptoms
Responds poorly to chemo, generally fatal
Aim to prevent (strict adherence to Gluten Free diet)
What is chronic lymphocytic leukaemia?
Proliferation of mature B-lymphocytes
What are the laboratory findings in CLL?
Lymphocytosis between 5 and 300 x 10^9/l Smear cells Normocytic normochromic anaemia Thrombocytopenia Bone marrow lymphocytic replacement of normal marrow elements
What are the different progressions of CLL?
Never progress
Progress but respond to treatment
Progress, require multiple lines of treatment –> death
What is a genetic indicator of poor prognosis in CLL?
Deletion of 17p
What are the clinical issues in CLL?
Increased risk of infection
Bone marrow failure
Lymphadenopathy +/- splenomegaly, lymphocytosis
Transform to high grade lymphoma (Richter Transformation)
Auto-immune complications
What are the indications to treat CLL?
Progressive lymphocytosis Progressive marrow failure Massive or progressive lymphadenopathy / splenomegaly Systemic symptoms Autoimmune cytopenias
What are the treatment options for CLL?
Combination Immuno-chemotherapy Targeted therapy (BTK inhibitor, BCL2 inhibitor) Cellular therapy for relapsed high risk cases
Give an example of BCR Kinase inhibitors
Ibrutinib (BTK)
Idelalisib (PI3K)
Give an example of BCL2 inhibitors
Venetoclax
Give an example of experimental Cell based therapies
Chimaeric Antigen Receptor T cells (CAR-T)
