Lymphoma Flashcards
Describe the age distribution of cases of Hodgkin and non-Hodgkin lymphomas.
Hodgkin: bimodal with peaks at early twenties and again in the 80’s
Non-hodgkin’s steadily increases with age after middle-age
Name and describe the characteristic cell of Hodgkin lymphoma
Reed–Sternberg cells are large and are either multinucleated or have a bilobed nucleus (thus resembling an “owl’s eye” appearance) with prominent eosinophilic inclusion-like nucleoli
note that this cell is not the predominate cell
“crippled germinal center B -cell” it does not have normal B cell surface antigens and rearranged but not non-functional immunoglobulin genes
What are key clinical features of Hodgkin’s lymphoma.
seen in young adults B symptoms: fevers, night sweats, wt. loss pruritis cough/SOB due mediastinal mass (typical) pain painless adenopathy
What are the goals of staging Hodgkin’s disease?
plan treatment
evaluate tx. results
formulate prognosis
research communication
I: one node lymphoma site
II: node lymphoma only one side of the diaphragm
III: node lymphoma on both sides to the diaphragm
IV. infiltration of organs
Contrast the spread of Hodgkins and non-Hodgkin’s disease and way in which we approach patients with either disease.
HL: tends to spread to contiguous nodal groups although common to have a localized presentation (esp. mediastinal); approach determined where the disease is located; treatment to cure
NHL: is often widespread by time of diagnosis; approach dictated by histologic subtype; treatment depends on subgroup (acute or indolent)
Contrast the differentiation of B-cells in the bone marrow v. in the follicular center
Differentiation from stem cell to the mature B cell in the bone marrow is antigen-independent.
Differentiation from follicle-center B cell to plasma cell occurs in the germinal centers of the secondary lymphoid organs and is antigen-dependent
There are three processes of rearrangement in B-cells that can play a role in malignant transformation due to the high genomic pressure, they include _____, _____, and ______
receptor editing
class switching
somatic hypermutation**
these can all be normal parts of affinity maturation
How is inflammation and infection related to development of lymphoma?
greater antigen stimulus increases receptor editing and somatic hypermutation (more chance for error/mutation), this can also occur in increased cell proliferation of inflammation
Translocation that causes a change in transcriptional deregulation is most likely to cause ________ while translation causing a fusion protein is more like in ________.
transcriptional deregulation: lymphoma (except ALCL and MALT)
fusion protein: leukemia
Name the corresponding cancer with the following oncogenes: bcl-1, bcd-2, my and bcd-6.
bcl-1: Cylcin D1, t(11:14) leads to mantle cell lymphoma
bcl-2: anti apoptotic t(14:18) leads to follicular lymphoma
myc: proliferation signal t(8;14) Burkitt’s
bcl-6: transcription factor t(3;14) DLBCL
Contrast the initial presentation of indolent and aggressive NHL.
indolent: painless adenopathy
aggressive: present with symptoms: night sweats, fever, pain
Why is reliable hematopathology crucial in NHL?
approach, treatment and prognosis is dictated mainly by histology
When would you want to treat a patient with indolent NHL?
when :
patient begins to experience constitutional symptoms
compromise of vital organ by infiltrate
bulky adenopathy
rapid progression or other evidence of transformation
When would you want to treat aggressive NHL?
admin. most effective therapy at diagnosis, if not cured, patients are likely to die within months to a year of diagnosis
What is the risk stratification system used for aggressive NHL?
international prognostic index (IPI)