Lymphoproliferative Disorders Flashcards
What is a lymphoma
Cancers of lymphoid origin ,
Can present with:
- enlarged lymph nodes,
- extranodal involvement
- bone marrow involvement
- systemic B symptoms
What are the systemic B symptoms of lymphoma
Weight loss (>10% in 6 months), Fever, Night sweats, Pruritis, Fatigue
How to diagnose a lymphoma/ leukaemia
Biopsy (diagnose)
Clinical examination
Imaging (eg CT) (staging)
Types of lymphoma
Hodgkin lymphoma
Non-Hodgkin Lymphoma (split into high-grade and low-grade)
Examples of lymphoproliferative disorders
Acute Lymphoblastic Leukaemia,
Chronic Lymphoblastic Leukaemia,
Hodgkin lymphoma,
Non-Hodgkin lymphoma
(High grade- diffuse large Bcell lymphoma
And Low grade- follicular, marginal zone)
What cells are affected in acute Lymphoblastic leukaemia
Lymphoid progenitor cells
Occurs in bone marrow but could spread anywhere
Most common age group for acute lymphoblastic leukaemua
75% occur in children <6 years
Presentation of acute lymphoblastic leukaemia
2-3 week history of bone marrow failure (low haemoglobin, low platelets, high WCC)
Bone/joint pain
Infiltration of bone marrow:
Hepatosplenomegaly,
Lymphadenopathy,
CNS involvement (CN palsies, menigism)
Typical ALL presentation
17year old
1 month impaired vision (retinal haemorrhage),
1/2 stone weight loss,
Breathless on minimal exertion
ALL cells characteristics
Large cells
Express CD19
CD34, TDT are markers of early immature cells
ALL treatment
Induction chemotherapy,
Maintenance treatment for 18 months,
CNS directed treatment (LP methotrexate)
Stem cell transplantation
ALL newer therapies
Bi-specific T cell engagers eg Blintumumab
CAR (Chimeric Antigen receptor Tcells)
ALL poor risk factors
Increasing age,
Increased WCC,
Cytogenetics/ molecular genetics,
Slow/ poor response to treatment
ALL summary
Presentation and treatment
Bone marrow failure,
Bone pain,
Infection,
Sweat
T:
multi-agent intensive chemotherapy
Allogenic stem cell transplant
Why is CLL different from ALL even though it is a leukaemia
The abnormal cells are mature
So they:
Grow slowly,
Resemble normal well behaved lymphocytes
Requires a lymphocyte count of >5
CLL presentation
Often asymptomatic, Bone marrow failure, Lymphadenopathy, Splenomegaly, Fever and sweats
Hepatomegaly,
Infections,
Weight loss
CLL associated findings
Immune paresis,
Haemolytic anaemia
CLL Binet staging?
A < 3 lymph node areas
No affect
B 3 or more lymph node areas
8 year median survival
C Stage B + anaemia or thrombocytopenia
6 year median survival
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 cytopenia
CLL treatment
Watch and wait, Cytotoxic chemotherapy (fludarabine, bendamustine) Monoclonal antibodies (rituximab, obinatuzumab) Novel agents ( Bruton tyrosine kinase inhibitor, P13K inhibitor, BCL-2 inhibitor)
CLL poor prognostic markers
Advanced disease (Binet stage B or C), Atypical lymphocyte morphology, Rapid lymphocyte doubling time (<12 months), CD 38+ expression, Loss/ mutation of p53, Unmutated IgVH gene status
Lymphoma presentation
Lymphadenopathy,
Hepatosplenomegaly,
B symptoms,
Bone marrow involvement
Assessment of lymphoma
Lymph node biopsy,
CT scan,
Bone marrow aspirate and trephine
Ann Arbor staging of Lymphoma
Stage 1- one lymph node affected
Stage 2- 2 lymph node groups on the same time
Stage 3- both sides affected
Stage 4- extranodal involvement
A- absence of B symptoms
B- B symptoms present
How is Non-Hodgkin lymphoma classified
Lineage (B cell or T cell)
(Majority are B cell)
“Grade”
(High grade or low grade)
Difference between low grade lymphoma and high grade
Low grade:
- Indolent, often asymptomatic
- responds to chemotherapy but incurable
High grade:
- aggressive fast growing,
- requires combination chemotherapy
- can be cured
Describe the specific disease entities of Non-Hodgkin lymphoma
Diffuse large B-cell lymphoma:
- commonest subtype of lymphoma
- high grade lymphoma
Follicular lymphoma:
- low grade lymphoma
- watch and wait treatment
Treatement of Non-Hodgkin lymphoma
Combination chemotherapy
Anti-CD20 monoclonal antibody + chemotherapy
Hodgkin lymphoma risk factors
Peak 15-35 years 2nd peak later in life, EBV, Familial and Geographical clustering
Hodgkin Lymphoma
Combination chemotherapy,
+/- radiotherapy,
Monoclonal antibodies (anti-CD30),
Immunotherapy
ALL investigations
Blood film, Bone marrow (>20% lymphoblasts in bone marrow), WCC, CXR, FBC (Hb), LP
