lymphoid malignancy Flashcards
lymphoma
lymphoid origin can present with enlarged lymph nodes (lymphadenopathy) or extranodal involvement or bone marrow involvement systemic B symptoms
diagnosis
biopsy tells us what type
clinical exam and imaging tells staging
-info of prognosis
therapy
never surgical resection
hodgkin lymphoma
specific disease
non hodgkin
broad term/everything else
lymphoproliferative disorders
ALL CLL Hodgkin Non-hodgkin - high grade (difffuse large B cell lymphoma) - low grade (follicular, marginal zone)
ALL
disorder of lymphoid progenitor cells
Leukaemia = No differentiation. Instead, rapid, uncontrolled growth and accumulation
Usually in bone marrow but they can go anywhere
-75-90% in children under 6
B cell lineage mostly affected
present with 2-3 week of bone marrow failure or bone/joint pain
typical ALL case
17yr old male 1 month impaired vision(both eyes) half stone weight loss breathless on minimal exertion hb and platelets low white cell very high
Characteristics
large cells
express CD19- all B cells
CD34,TDT-markers of very early immature cells
treatment
standard -induction chemotherapy to obtain remission -consolidation therapy -CNS directed treatment -maintenance treatment for 18 months transplantation
ALL treatment
Newer therapies
1) Bi specific T cell engagers?
2) Chimeric antigen receptor T cells
- Healthy T cells harvested
- transfected to express a specific T cell receptor expressed on leukemia cells (CD19)
T cell immunotherapy side effects
not in exam
Cytokine release syndrome -fever, hypotension, dyspnoea
neurotoxicity- confusion
seizure, headache, coma
Poor risk factors ALL
increasing age white count cytogenetics t(9,22); t(4,11) poor response
summary ALL
bone marrow failure +/- raised white cell count
Bone pain, infection, sweats
Treated with multiagent intensive chemo +/- allogeneic stem cell transplant
CLL
the abnormal cells are mature – they usually resemble normal, well-behaved lymphocytes
Grow slowly (or not at all)
Classic example of a low-grade condition
Carry many of the normal markers that B lymphocytes have
Requires a lymphocyte count of > 5 (normal is < 4)
CLL continued
Commonest leukemia occasionally familial 2 makes: 1 female often asymptomatic -bone marrow failure -lymphadenopathy -splenomegaly fever and sweats Less common findings: hepatomegaly infections weight loss
associated findings
immune paresis (loss of Ig production) Haemolytic anaemia
CLL staging
Stage A <3 lymph nodes median survival same as age matched controls stage B 3 or more lymph nodes Stage C Stage B- anaemia or thrombocytopenia
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
treatment
incurable low grade watch and wait cytotoxic chemotherapy monoclonal antibodies eg Rituximab novel agents
poor prognostic marker
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) Unmutated IgVH gene status
what is an adverse prognostic factor
loss/mutation of P53
ann arbour staging system
I-IV
B cells
majority of leukemias
high grade lymphoma
Aggressive, fast-growing
Require combination chemotherapy
Can be cured
low grade
Indolent, often asymptomatic
responds to chemotherapy but incurable
non hodgkin lymphoma
Diffuse large B-cell lymphoma
Commonest subtype of lymphoma (of any kind)
High-grade lymphoma
Follicular lymphoma
2nd commonest subtype of lymphoma
Low-grade lymphoma
Like CLL, leave alone if not causing problems – “watch and wait”
Both are treated with combination chemotherapy – typically anti-CD20 monoclonal antibody + chemo
hodgkin lymphoma
30% of all lymphomas
-association with epstein barr virus; familial and geographical clustering
Treatment (dont need to know) Combination chemotherapy (ABVD) \+/- radiotherapy Monoclonal antibodies (anti-CD30) Immunotherapy (checkpoint inhibitors) 1st peak at 15-35y (not associated to virus) 2nd peak later in life
PET scanning central to assessment of response to treatment
associated to immunity to fight viruses
features
hodgkin lymphoma
contiguos lymph nodes
