Lymphoid malignancies Flashcards
(35 cards)
what is used to diagnose a leukaemia?
Biopsy (bone marrow, lymph node)
how Is staging done?
thorugh clinical examination and imaging (CT) - this tells us WHERE it is = location and extent, prognosis, influences tx.
what are the types of lymphorpoliferative disorders?
Acute lymphoblastic leukaemia (ALL)
Chronic lymphocytic leukaemia (CLL)
Hodgkin lymphoma
Non-Hodgkin lymphoma (NHL) -High-grade (diffuse large B-cell lymphoma), Low-grade (follicular, marginal zone)
ALL (Acute lymphoblastic leukamia) - what is it?
Cancerous disorder of lymphoid progenitor cells, Usually in the bone marrow but they can go anywhere.
Normal = Immature, rapidly proliferating cells that differentiate into lymphocytes
Leukaemia = No differentiation. Instead, rapid, uncontrolled growth and accumulation.
75% of cases occur in people of what age group?
children below the age of 6. present with a 2-3 wk hx of bone marrow failure or bone/joint pain.
RF’s of ALL
Increasing age, Increased white cell count, Cytogenetics/molecular genetics t(9;22); t(4;11), Slow/poor response to treatment
what is a TYPICAL ALL hx?
17m, 1 month impaired vision in both eyes, 1/2 stone weight loss, dypnoea on minimal exertion
general features of acute leukaemia?
BONE PAIN, INFECTION, SWEATS, dyspnoea, fever, pale, splenomegaly, lymphadenopathy, purpura, bleeding gums, fatigue
TYPICAL ALL Ix and results
FBC = low Hb, raised WCC, low platelets. Bone marrow shows 90% B-Lymphoblasts, BONE MARROW FAILURE WITH RAISED WCC
what are the characteristics of ALL cells? And what do they express?
large, EXPRESS CD19!!!, cd34 and tdt
Tx of ALL…
chemo to obtain remission, consolidation therapy, CNS directed tx, maintainance of tx for 18 months, Allogenic Stem cell transplantation may be used if risk is high.
ALL tx newer therapies…
bi-specific t-cell engagers e.g.blinatumab, CAR (chimeric antigen receptor T-cells) - this is where healthy t cells are harvested, made to express a specific receptor for cd19 on leukaemia cells then reinfused into the pt.
KEY SIDE EFFECTS OF T CELL IMMUNOTHERAPY
cytokine release syndrome (fever, hypotension, dyspnoea)
Neurotoxicity (confusion, normal conscious level, seizure, headache, focal neurology, coma)
prognosis of ALL
adults and children = 90% remission rate.
how is ALL different to CLL?
the abnormal cells are mature - they usually resemble normal, well behaved lymphocytes (grow slowly, carry many of the normal markers which b lymphocytes have)
what lymphocyte count is required to be diagnostic of CLL?
> 5
epidemiology
> 1700 new cases CLL per year in the UK, Commonest leukaemia worldwide, 2 males: 1 female, Occasionally familial, Rare in far East – this likely indicates a Geographic/ ethnic role in pathogenesis.
CLL sy/sx
of asymptomatic. Frequent findings: bone marrow failure (anaemia, thrombocytopenia), lymphadenopathy, splenomegaly, fever and sweats. Less common: hepatomegaly, infetions, weight loss.
what are associated ifndings with CLL?
immune paresis (loss of normal immungoglobulin production), Haemolytic anaemia
Outline BINET STAGING for CLL…
A = <3 lymph nodes, B = >3 lymph nodes, C = B + anaemia or thrombocytopenia (6yr survival)
what are the indications for tx of CLL?
progressive bone marrow failure, huge lymphadenopathy, progressive splenomegaly, lymphocyte doubling time <6 months, systemic sy, autoimmune cytopenias.
CLL Tx
often do nothing. Cytotoxic chemotherapy e.g. fludarabine, bendamustine
Monoclonal antibodies e.g. Rituximab, obinatuzumab
Novel agents - Bruton tyrosine kinase inhibitor eg ibrutinib, PI3K inhibitor eg idelalisib, BCL-2 inhibitor eg venetoclax.
what are poor prognostic markers for CLL?
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 the Px of a LYMPHOMA?
lymphadenopathy/ hepatosplenomegaly
Extranodal disease
“B symptoms” = fever, drenching night sweats, unexplained weight loss over the last 6 months.
bone marrow involvement
