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
