Lymphoma Flashcards
Classification of lymphoma
Hodgkin’s have mirror-image nuclei (Reed Sternberg cells)
Non-hodgkin’s
HL types
Typical HL (nodular sclerosing/mixed cell/ lymphocyte rich/ lymphocyte depleted (poor prognosis)) Nodular lymphocyte predominant HL
NHL types
B cell (high grade (diffuse large b-cell most common) or low grade (follicular most common)) T cell (rarer)
HL risk factors
Affected sibling
EBV
SLE
Post-transplant
HL presentation
Non-tender, rubbery, spreading unilateral lymphadenopathy (generally cervical/ supraclavicular)
Pain when drinking alcohol
B symptoms (wt loss >10% 6mths, fever, night sweats)
Mediastinal masses in 80%, especially nodular sclerosis
Lymphoma tests
Lymph node excision biopsy is diagnostic
Raised ESR/ LDH/ lower Hb indicates worse prognosis
CXR may show mediastinal mass
PET/CT used for staging, bone marrow biopsy for NHL staging
Lymphoma staging
Ann Arbor
I - Confined to single lymph node region
II - 2+ lymph node regions on same side of diaphragm
III - Nodes on both sides of diaphragm involved
IV - Spread beyond nodes e.g. to liver/bone marrow
A/B - are B symptoms present
E - extra-nodal extension
X - >10cm bulk
HL treatment
I-A + II-A radiotherapy and short chemo courses
ABVD chemo - Adriamycin (doxyrubicin), Bleomycin, Vinblastine, Dacarbazine; stop bleomycin after 2nd cycle if CT/PET negative
Stem cell transplants in relapsed disease
HL prognosis
Very good, especially if earlier and lower stage
Lymphocyte predominant best prognosis
Worse if B symptoms present
<40% if IV-B lymphocyte depleted
NHL presentation
Superficial lymphadenopathy in 75%
Extranodal disease can be in gastric MALT/ non-MALT; skin; oropharynx
Pancytopenia symptoms if bone marrow involvement
NHL management
Low-grade generally incurable, watch and wait initially, radiotherapy if localised then R-bendamustine if symptomatic in later stages
R-CHOP for high-grade: Rituximab, Cyclophosphomide, hydroxydaunorubicin (Doxirubicin), Oncovin (vincristine), Prednisolone
NLPHL treatment
Radiotherapy
Bendamustine
R-CHOP if advanced
Rituximab in untreated follicular lymphoma
Kills CD20 +ve cells
Also useful as sensitises cells to CHOP, so cost-effective
What is CML
Uncontrolled clonal proliferation of myeloid cells
>80% have Philadelphia chromosome translocation t(9;22) forming BCR-ABL (tyrosine kinase activity)
CML presentation
Most commonly 40-60yrs, slightly more males
Splenomegaly/hepatomegaly
Anaemia/bruising
Chronic + insidious weight loss, fatigue, fever, sweats
Maybe gout, bleeding
CML tests
Very high WCC with spectrum of myeloid cells
Hb normal/dec
Inc urate + B12
Hypercellular bone marrow
CML phases
Chronic phase (mths-yrs) with few/no symptoms Accelerated phase - increasing symptoms, spleen size and deranged counts Blast transformation with features of acute leukaemia
CML treatment
Imatinib or dasitinib/nilotinib/bosutinib for Ph +ve
Hydroxycarbamide
Lymphoblastic transformation pts may have AML treatment
Allogeneic stem cell transplantation is the only cure, but significant morbidity/mortality
What is CLL
Commonest leukaemia
Hallmark is progressive accumulation of malignant clonal functionless B cells
Staging and survival CLL
Rai stage:
0 - lymphocytosis alone - >13yrs
I - lymphocytosis + lymphadenopathy - 8yrs
II - lymphocytosis + spleno/hepatomegaly - 5yrs
III - lymphocytosis + Hb<110 - 2yrs
IV - lymphocytosis + platelets <100 - 1yr
CLL presentation
Often asymptomatic, incidental FBC finding
Anaemic/infection prone
Weight loss, night sweats, anorexia if severe
Enlarged non-tender rubbery lympadenopathy
Spleno/hepatomegaly
CLL tests
Lympocytes raised
Small mature lymphocytes + smear cells on blood film
Bone marrow involvement
CLL complications
Autoimmune haemolysis
Infection
Marrow infiltration -> dec Hb/neuts/platelets
Richter’s transformation -> NHL
CLL treatment
If symptomatic, fludarabine + rituximab ± cyclophosphamide 1st line
Ibrutinib, chlorambucil, bendamustine + ofatumumab also may help
Steroids for AIHA
Radiotherapy for lymphadenopathy/splenomegaly
Supportive care
CLL progression
1/3 never progress
1/3 progress slowly
1/3 progress actively
Death often due to infection/transformation to aggressive NHL (Richter’s syndrome)
Burkitt lymphoma features
Associated with EBV
Rapid proliferation so may present with compression
Starry sky appearance on lymph node biopsy
