Lymphoid malignancy Flashcards
what are the different presentations of lymphoma ?
lymphadenopathy
extranodal involvement
bone marrow involvement
systemic (B) symptoms - weight loss, night sweats, pruritus, fatigue
how is lymphoma diagnosed ?
biopsy (marrow, node) malignant cell characteristics
how is staging assessed ?
clinical exam
imaging - CT
what is the difference between Hodgkins and non-hodgkins ?
Hodgkin’s lymphoma is a specific disease
NHL is everything else - 70 subtypes
what are the different types of lymphoproliferative disorders ?
NHL -high grade (diffuse large B cell lymphoma), low grade (follicular, marginal zone)
HL
Chronic lymphocytic leukaemia
acute lymphocytic leukaemia
what is the order of how common the lymphoproliferative disorders are ?
high grade NHL low grade NHL HL CLL ALL
what is acute lymphoblastic leukaemia ?
cancerous disorder of lymphoid progenitor cells
no differentiation, rapid, uncontrolled growth and accumulation
usually in bone marrow but can be anywhere
what proportion of cases occur in <6 y/o in ALL
75%
what are the presenting symptoms ?
2-3 wk hx of bone marrow failure or bone/joint pain
infection
sweats
what is a typical ALL history ?
17 male
1 month impaired vision
1/2 stone weight loss
SOB minimal exertion
what investigations for ALL ?
FBC - decreased Hb and platelets, elevated WCC
bone marrow aspirate - 90% B-lymphoblasts
what are characteristics of ALL cells ?
large cells
express CD19 - all B cells do
CD34, TDT - markers of immature cells
what is the treatment for ALL ?
chemotherapy - consolidation, CNS, maintenance for 18 months
stem cell transplant if high risk
newer- Bi specific T cell engagers - blinatumumab
CAR - T cell infusion
what are side effects of T cell immunotherapy ?
cytokine release syndrome - fever, hypotension, SOB
neurotoxicity - confusion, seizure, headache, focal neurology, coma
what are risk factors for poor ALL prognosis ?
increased age
increased WCC
genetics t(9;22), t(4;11)
what is the prognosis of ALL in adults and children ?
adults - complete remission 90%, leukaemia free survival 5y 30-35%
children - 5y survival 90%, poor risk 5y 45%
what is different about the CLL cells compared to ALL cells ?
grow slowly
low-grade concentration
carry many markers B lymphocytes have
requires a lymphocyte count of >5
what is significant about the incidence of CLL ?
commonest leukaemia in the world
M2:1F
occasionally familial
rare in Far East
what is commonly seen in CLL presentation ?
often asymptomatic
bone marrow failure - anaemia, thrombocytopenia
lymphadenopathy
splenomegaly
fever and sweats
less commonly- hepatomegaly, infection, weight loss
what are associated findings in CLL ?
immune paresis - loss of Ig production
haemolytic anaemia - 20% +ve direct antiglobulin test
what is the staging for CLL and what are the stages ?
Binet staging
A - <3 lymph nodes, median survival normal
B - =>3 lymph nodes, survival 8 years
C - Stage B + anaemia/thrombocytopenia, survival 6 yrs
what are indications for treatment in CLL ?
progressive marrow failure massive lymphadenopathy progressive splenomegaly lymphocyte doubling time <6 months/ 50% over 2 years systemic symptoms autoimmune cytopenias
what is the treatment for CLL ?
often nothing
chemotherapy - bendamustine
monoclonal antibodies - rituximab
novel - TK inhibitors
what are poor prognostic factors for CLL ?
advanced disease B,C atypical lymphocyte morphology rapid lymphocyte doubling time <12 month CD 38+ expression loss/mutation p53; del 11q23 unmutated IgVH gene status
how does lymphoma present ?
lymphadenopathy/ hepatosplenomegaly
extranodal disease
B symptoms
bone marrow involvement
how is lymphoma staging assessed ?
node biopsy, CT, marrow aspirate and trephine
how is lymphoma staged ?
1 - single node
2 - multi nodes
3 - nodes, spleen
4 - nodes, spleen, liver, guts
A - absence of B symptoms
B - fever, night sweats, weight loss
how are NHL lymphomas classified ?
lineage - B or T cells (90% B)
grade - high or low
what is low - grade NHL ?
indolent, often asymptomatic
responds to chemo
incurable
what is high grade NHL ?
aggressive, fast growing
require combo chemo
can be cured
what are the specific diseases entities for NHL ?
diffuse large B-cell lymphoma - commonest, high grade
follicular - 2nd commonest, low grade, leave if no problems
how is NHL treated ?
combination chemotherapy - anti-CD20 monoclonal antibody + chemo
how many lymphomas are HL ?
30%
what are the two peaks for HL ?
15-35
later in life
what is HL associated with ?
EBV
familial
geographic clustering
