3.2 Lymphoma Flashcards
What is the common presentation of lymphoma?
Asymptomatic Lump: enlarged nodes, extranodal mass Organomegaly B symptoms: fever, weight loss, night sweats Constitutional symptoms Unusual sites: lump, CNS, abdominal Metabolic complications: hyperclacaemia
What are the methods used for diagnosis in lymphomas?
Fine needle aspiartion
Excision biopsy
Routine histology
Ancillary investgations: flow cytometry, immunohistochemistry, cytogenetics, molecular studies
Which leukaemia has bone marrow involvement?
Chronic lymphocytic
What is the ann Arbor staging of lymphoma?
I: single lymph nod region or single extralymphatic site
II: two or more nodes regions on same side of diaphragm, may include localized extralymphatic involvement on same side
III: lymph node regions on both sides of diaphragm, may include spleen or localized extranodal disease
IV: diffuse extralymphatic disease (liver, bone marrow, lung, skin)
What differentiates CLL from mantle cell lymphoma?
CD23
What is the immunophenotype of CLL?
CD19, 23, 5 +
Which B cells are CLL thought to arise from?
Memory B cells and pre germinal centre naive B cell
What predicts a poor outcome in CLL?
Lack of somatic mutations (Ig)
What will you see histologically in CLL?
Disrupted tumour cells (SMUDGE CELLS) - little cytoplasm, brittle cells
What is the binet staging of CLL?
A: = 3 lymphoid areas
B = > 3
C: anaemia or thrombocytopenia
What is the prognosis of CLL based on?
Binet staging, mutated vs unmutated IgVH, cytogenetics
What B cell does follicular lymphoma originate from?
Follicular centre B cell
What will you see on flow cytometry for follicular lymphoma?
CD10/19, smlg+ (monoclonal)
What genetic mutation occurs in follicular lymphoma?
t(14;18) for gene BCL2
What scoring system is used for folicular lymphoma?
FLIPI score = 5 nodal sites elevated LDH Age >60 Stage 3 or 4 Hb
What characterises follicular lymphoma?
malignant follicles (loss of apoptotic process due to the overexpression of BCL2)
What is the usual presentation of follicular lymphoma?
asymptomatic for a long time (generalized lymphadenopathy) - usually very progressed at presentation
What is the treatment of follicular lymphoma?
Only when symptomatic - low dose chemo
What is used for the prognosis of DLBCL?
Age (60) Stage (I/II vs. III/IV) Extranodal (1 vs. >1 site) LDH ECOG performance (0-1 vs. 2-4)
What are the two cells of origin for DLBCL?
Germinal centre and post germinal centre B cell
What gene is thought to be involved in DLBCL?
Bcl6 which is required for the formation of normal germinal centres, growth arrest and apoptosis
What are the histologic features of DLBCL?
tumour cell shave large nucleus, open chromatin, and prominent nucleoli
What is the genetic involvement of Burkitt lymphoma?
Translocation of the MYC gene on chromosome 8 leading to overexpression of MYC protein
Usually t(8;14)
What infection is associated with Burkitt lymphoma?
EBV
Why is Burkitt lymphoma so aggressive?
The cell shave a 100% proliferation rate
What distinguishes Burkitt lymphoma on flow cytometry?
Lack of BCL2 (anti apoptotic protein)
What is the histologic charaterising feature of Hodgkins lymphoma?
Reed Sternberg cells (owl eyes) - multiple nuclei or a single nucleus with multiple nuclear lobes each with a small incision like nucleolus about the size of a small lymphocyte
Who normally gets Hodgkins lymphoma?
Bimodal - Young and old
What is thought to occur in Hodgkins lymphoma?
Activation of NF-kB which rescues crippled germinal centre B cells from apoptosis resulting in the acquisition of other unknown mutations that collaborated to produce the Reed Sternberg cells which support the growth of tumour cells
What infection if Hodgkins lymphoma associated with?
EBV and immunosuppressed patients
What is the usual progression of Hodgkins lymphoma?
Nodal disease - splenic - hepatic - marrow and other tissues
What are the treatment options for lymphomas?
Chemoherapy
Monoclonal antibodies (Rituximab - against CD20)
Radiotherapy
Wait and watch for indolent
Anti-emetics
G-CSF antibiotics
Transfusion support
What are the markers of B cell differentiation?
CD20, CD22, CD19
