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33 Cards in this Set

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What are the four categories of lymphomas?
CD5+ lymphomas (small lymphocytic lymphoma,)
follicle-derived (follicular small cleaved lymphoma and diffuse large cell lymphoma,)
Hodgkin's Disease, and
aggressive lymphoma (Burkitt's and lymphoblastic lymphoma.)
What is the working formulation?
Low grade lymphomas have slow courses that progress over many years but are often incurable.
High grade lymphomas have acute presentations, pose a high risk of death, but are often curable.
Intermediate grade lymphomas are in between.
How are the different grades of lymphomas treated?
Low grade - palliative care,
Intermediate grade - R-CHOP +/- XRT,
High grade - like ALL
Hodgkin's - ABVD +/- XRT
Abnormalities in which chromosomes are commonly associated with lymphomas?
Chromosome 14 (Ig heavy chain)
Chromosome 2 (kappa light chain)
Chromosome 22 (lambda light chain)
The changes leading to transformation begin at the ______________.
Stem cell.
What conditions are associated with lymphomas and why?
H. pylori, HIV, EBV, SLE, and Hepatitis C because the more lymphocytes that mature, the higher the risk of lymphoma.
What causes the proliferation in Burkitt's lymphoma?
MYC, a transcription factor, gets overexpressed, driving proliferation and elevating LDH.
How is lymphoblastic lymphoma different? What is its leukemia counterpart?
Lymphoblastic lymphoma is a disease of T cells. It is the lymph node version of ALL (and is treated like ALL.)
What age group does lymphoblastic lymphoma affect?
Lymphoblastic lymphoma affects children and young adults.
What markers are expressed in lymphoblastic lymphoma?
CD4, CD8, and TdT.
Immunoglobulin is to B-cell lymphomas as what is to lymphoblastic lymphoma?
TCR (T-Cell Receptor)
What organs/systems need special treatment in lymphoblastic lymphoma and ALL?
CNS and testicles
What grade lymphoma is Diffuse Large Cell Lymphoma?
Diffuse large cell lymphoma is intermediate grade.
What are the two types of Diffuse Large Cell Lymphoma?
Germinal center B-cell like (expresses genes characteristic of gernimal B cells), and
Activated B-cell like (expresses genes characteristic of plasma cells, post-germinal cell phenotype)
What gene is associated with Diffuse Large Cell Lymphoma?
BCL6 (3q27). BCL6 is a trancription repressor; when it is overactive and turns genes off, maturatioin is arrested and cells persist in an undifferentiated state.
At what stage does the genesis of follicular small cleaved lymphoma occur?
Class switching, somatic hypermutation, affinity maturation (post-germinal)
What translocations are follicular lymphomas associated with?
Most follicular lymphomas are associated with translocations of BCL2 (18q21) and the heavy chain (14q23.) When turned on, BCL2 inhibits apoptosis.
What lymphoma is the equivalent of CLL?
SLL, Small Lymphocytic Lymphoma
What is the clinical course of SLL?
SLL is slow-growing, incurable, and often presents with stage IV disease.
What is the malignant cell in Hodgkin's Disease?
Reed-Sternberg cell (likely derived from a B cell, many typical B cell genes are not expressed)
What are the four types of Hodgkin's Disease, and which one has a clinical course that is different?
Nodular Sclerosis
Lymphocyte Depleted
Mixed Cellularity
Lymphocyte Predominant (affects older individuals, has a more indolent course.)
What treatment is used for HD?
ABVD (MC, LD, and NS)
rituximab (LP)
How is Hodgkin's different from Non-Hodgkin's Lymphoma?
A majority of the cells are reactive, spreads to adjacent lymph node groups, often presents in mediastinum, mass after treatment, bimodal age distribution, often curable.
When should you suspect lymphoma?
Asymptomatic (large nodes, anemia)
Mass effects (mediastinal mass = substernal pain, cough, dyspnea, abdominal mass = pain, early satiety, hyperbilirubinemia)
Weight loss, night sweats, fever
What are some characteristics of a lymph node that would make you want to biopsy it?
Size (over 2 cm),
Growth rate (over weeks to months)
Pain (painless is c/w lymphoma)
What are the four stages of lymphoma?
Stage I: single nodal region
Stage II: two or more on the same side of the diaphragm
Stage III: two or more on opposite sides of the diaphragm
Stave IV: liver or bone marrow involvement
What do E and B stand for?
E = extranodal site
B = B symptoms (weight loss, night sweats, fever)
What is LASSS?
IPI calculation:
LDH: > normal LDH
Age: > 60
Stage: III or IV
Site: > 1 extranodal site
Status: > or equal to 2
What are the three types of lymphoma treatment?
Palliative (SLL, follicular small cleaved cell)
Curative (DLCL, Burkitt's, lymphoblastic, Hodgkin's)
Instensive (bone marrow transplant, generally for relapsed patients)
When are radiation/chemo used in Hodgkin's and DLCL?
Stage I, II = RADIATION + chemo
Stage III, IV = CHEMO + radiation
What is the standard therapy for Hodgkins?
ABVD:
adriamycin
bleomycin
vinblastine
dacarbazine
What is the standard therapy for DLCL?
R-CHOP:
rituximab
cyclophosphamide
adriamycin
vincristine
prednisone
What is rituximab?
Rituximab is a monoclonal a-CD20 antibody