Lymphoid Malignancy Flashcards
What are lymphomas?
Cancers of lymphoid origin
How do lymphomas present?
Enlarged lymph nodes
(Extranodal involvement)
(Bone marrow involvement)
Systemic symptoms
Systemic symptoms associated with lymphoma
Weight loss Fever Night sweats Pruritis Fatigue
How is a lymphoma/leukaemia diagnosed?
Biopsy
How is a lymphoma/leukaemia staged?
Clinical examination and imaging - where it is
Which cancer is associated with lymphoid progenitor cells?
ALL
Which cancer is associated with B cells and T cells?
Lymphomas/CLL
Which cancer is associated with plasma cells?
Multiple myeloma
What are the types of lymphoma?
Hodgkin lymphoma
Non hodgkin lymphoma
What are the types of non Hodgkin lymphomas?
High grade
Low grade
Lymphoproliferative disorders
Hodgkin lymphoma
Non-Hodgkin lymphoma
CLL
ALL
What is ALL?
Cancerous disorder of lymphoid progenitor cells
What normally happens with lymphoid progenitor cells?
Immature, rapidly differentiateinto lymphocytes
What normally happens with lymphoid progenitor cells in leukaemia?
No differentiation - instead, rapid, uncontrolled growth and accumulation (usually in bone marrow but they can go anywhere)
How does ALL present?
2-3 week history of bone marrow failure or bone/joint pain
Infection
Sweats
+/- raised white cell count
Laboratory results in ALL
Decreased Hb
Decreased platelets
Increased white cell count
What is the main cell type involved in ALL?
B cell lineage
ALL cell characteristics
Large cells
Express CD 19 (contained by all B cells)
CD34, TDTD (markers of early, immature cells)
ALL treatment
Chemo for remission
Consolidation therapy
CNS directed treatment
Maintenance treatment for 18 months
Stem cell transplantation if high risk
Newer treatment ALL
Bi-specifc T-cell engagers
CAR (chimeric antigen receptor T-cells)
T-cell immunotherapy side effects
Cytokine release syndrome
Neurotoxicity
Cytokine release syndrome
Fever
Hypotension
Dyspnoea
Neurotoxicity
Confusion with normal conscious level Seizure Headache Focal neurology Coma
ALL poor prognostic factors
Increasing age
Increased white cell count
Slow/poor response to treatment
What is the difference between cells in ALL and CLL?
Abnormal cells in CLL are very mature - usually resemble normal lymphocytes
What lymphocyte count is required for a diagnosis of CLL?
> 5 (normal is <4)
CLL presentation
Often asymptomatic
Bone marrow failure
Lymphadenopathy
Splenomegaly
Fever and sweats
CLL associated findings
Immune paresis
Haemolytic anaemia
Immune paresis
Loss of normal immunoglobulin production
Binet CLL staging
A - <3 lymph node areas
B - 3 or more lymph node areas
C - stage B + anaemia or thrombocytopenia
CLL indications for treatment
Progressive bone marrow failure Massive lymphadenopathy Progressive splenomegaly Lymphocyte doubling time <6 months or >50% increase over 2 months Systemic symptoms Autoimmune cytopenias
CLL treatment
Often watch and wait
Cytotoxic chemotherapy
Monoclonal antibodies
Novel agents
Cytotoxic chemotherapy agents
Fludarabine
Bendamustine
Monoclonal antibody agents
Rituximab
Obinatuzumab
Novel agents CLL
Bruton tyrosine kinase inhibitor
CLL poor prognostic factors
Advanced disease (Binet stage B or C) Atypical lymphocyte morphology Rapid lymphocyte doubling time (<12 mth) CD 38+ expression Loss/mutation p53; del 11q23 (ATM gene)
Presentation of lymphoma
Lymphadenopathy/ hepatosplenomegaly
Extranodal disease
“B symptoms”
Bone marrow involvement
How is lymphoma staged?
Stage 1-4 => increasing number of sites
A - absence of B symptoms
B - Fever, night sweats, weight loss
How is non Hodgkin lymphoma classified?
Lineage - B cell or T cell (90% B cell)
Grade - high/low
Low grade lymphoma
Indolent, often asymptomatic
Responds to chemotherapy but incurable
High grade lymphoma
Aggressive, fast-growing
Require combination chemotherapy
Can be cured
What are the two main types of non Hodgkin lymphoma?
Diffuse large B-cell lymphoma
Follicular lymphoma
Diffuse large B-cell lymphoma
High grade
Commonest subtype of lymphoma (of any kind)
Follicular lymphoma
Low grade lymphoma
What is Hodgkin lymphoma associated with?
EBV
Familial and geographical clustering
Hodgkin lymphoma treatment
Combination chemotherapy +/- radiotherapy
Monoclonal antibodies (anti-CD30) Immunotherapy (checkpoint inhibitors) Stem cell transplant
Hodgkin lymphoma presentation
Systemic symptoms => Night sweats Unintentional weight loss Fever SOB/persistant cough
Swelling in neck, armpit or groin
High grade NHL
Diffuse large B-cell lymphoma
Low grade NHL
Follicular, marginal zone
Poor cancer prognosis factor
loss/mutation of p53
What is p53?
Tumour suppression gene
Lymphoma staging
A - absence of B symptoms
B - fever, night sweats, weight loss
Stage 1 - 1 node
Stage 2 - more than one node but on the same side of the diaphragm
Stage 3 - no extranodal involvement but both sides of the diaphragm
Stage 4 - extranodal involvement