Lymphoma Flashcards
1
Q
What does normal B cell differentiation look like?
A
- Lymphoblasts –> naive B cells –> transform in germinal centers when exposed to mitogen (antigen selection and somatic hypermutation) –> memory type B cells, plasma cells, marginal zone cells
- Some memory type B cells and marginal zone B cells skip germinal center so non-mutated
- Resting B cells can transform when exposed to mitogen or antigen –> become larger w/ nucleoli and more abundant cytoplasm
2
Q
Normal Lymph Node Architecture
A
- Follicles have central, pale pink germinal center w/ mantle zones along outside (darker) and may have marginal zone outside of that
- Should see capsule (not too thick) and sinusoids
3
Q
Categorization of Lymphomas
A
- Hodgkin - Reed-Sternberg cells
- Nodular Lymphocyte Predominant (NLPHL)
- Classical
- Nodular sclerosis
- Lymphocyte rich (many small lymphocytes)
- Mixed cellularity (mixed inflammatory cells)
- Lymphocyte depleted (many RS cells or fibrosis instead)
- Non-Hodgkin -
- Divided by cell type and maturity level
- Can also be divided based on behavior
- Indolent NHL (slow growing but poor response)
- Follicular
- Small lymphocytic
- Marginal zone
- Mycosis fungoides
- Aggressive NHL (grow faster, respond to chemo)
- Diffuse large B cell
- Mantle cell
- Anaplastic large cell
- Highly aggressive NHL (very fast growing, may spread to CNS, respond very well to chemo)
- Burkitts
- Precursor B or T cell
- Adult T cell
- Indolent NHL (slow growing but poor response)
4
Q
Risk Factors for Lymphoma (5)
A
- Immune def (AIDS, CVID, SCID, autoimmune states)
- Infectious agents (EBV, HH8, HTLV-1)
- Herbicide exposure
- Chemicals (agent orange)
- Prior radiation
5
Q
Lymphoma Presentation
A
- Poor appetite / wt loss
- Lymphadenopathy / splenomegaly
- “B symptoms” (constitutional) - fever, chills, sweats
- Fatigue
- Night sweats
- Pruritus esp after alcohol use in HL
- Skin rash
6
Q
Lymphoma Labs
A
elevated blood counts, hepatic/ renal dysfunction, inc LDH and uric acid (esp if aggressive - cell turnover), abnormal cells on peripheral blood flow cytometry
7
Q
Tumor Lysis Syndrome
A
- sudden breakdown cells –> inc uric acid, hyperkalemia, hyperphos, uric acid crystals in urine, renal failure
- Give allopurinol, IVF, alkalinization
8
Q
Lymphoma Staging + Modifiers
A
- I - one group nodes
- II- multiple groups nodes but same side of diaphragm
- III- both sides of diaphragm
- IV - spread diffusely w/ extra-lymph sites (BM, lungs, liver)
- **Modifier - letters
- A = absent constitutional symptoms
- B = fever, chills, sweats
- E = extranodal (from node to adjacent tissues)
- X = bulky disease (large deposit > 10 cm or 1/3 mediastinum)
- S = spread to spleen
9
Q
IPI Score
A
- 1 pt for ea
- Age > 60
- Stage III or IV
- Elevated LDH
- Poor performance status
- > 1 extranodal sites
10
Q
Hodgkin’s Lymphoma
A
- Only 1% all lymphomas
- Bimodal - peaks in 20s/30s and in >50
- Associated w/ EBV
- Hallmark = Reed Sternberg cells (large, bi-nucleate w/ prominent nucleoli that looks like owl’s eyes) surrounded by reactive, non-neoplastic inflammatory cells (lymphocytes, plasma cells, eosinophils, histiocytes, neutrophils)
- Types
- Lymphocyte predominant - indolent, CD20+
- Classical
- Nodular sclerosing
- Mixed cellularity
- Lymphocyte depleted
- Lymphocyte rich
- 95% cured
- Tx
- Radiation
- Chemo - ABVD, MOPP, BEACOPP
- Combo
11
Q
Follicular Lymphoma
A
- 40% of lymphomas
- Indolent; usually in elderly
- From B cells in follicles of nodes
- Genetics - translocation of Bcl2 gene (anti-apoptosis) now next to heavy chain promoter t(14;18) so now very high levels Bcl2
- 80% 5 yr survival
- Can transform to high grade lymphoma
- Tx - rituximab w/ chemo (COMBO)
12
Q
Small Cell Lymphoma/ Chronic Lymphocytic Lymphoma (SCL/CLL)
A
- Extremely common in elderly
- Accumulation of monoclonal lymphocytes in blood
- Usually asymptomatic and notice lymphocytosis on CBC
- Rai Staging
- Low - lymphocytosis
- Intermediate - Lymphadenopathy, hepatomegaly, splenomegaly
- High - anemia, thrombocytopenia - Complications
- Early hypogammaglobinemia - infections
- Autoimmune hemolytic anemia
- Richter’s Transformation - indolent –> high grade w/ sudden lymphadenopathy, new B symptoms and BM involvement - Tx - rituximab, chemo +/- radiation
13
Q
Marginal Zone Lymphoma
A
- Indolent
- Classified by area of involvement
- Nodal marginal zone, extranodal marginal zone, splenic marginal zone (associated w/ Hep C)
- Associated w/ chronic infection or inflammation
- Gastric MALT - H pylori
- Ocular-adnexal MALT - Chlamydia psittaci
- SI MALT - C jejuni
- Cutaneous MALT - Borrelia burgdorferi
- Tx - abx/ treat underlying infection, occasionally rituximab w/ chemo
14
Q
Mycosis fungoides v. Sezary Syndrome
A
- MF - form of cutaneous T cell lymphomas w/ predominantly skin involvement
- Hallmark = Pautrier’s microabscesses - Sezary - lymphadenopathy, splenomegaly and peripheral blood involvement (beyond skin)
- Skin biopsy show sezary cells (med to large lymphocytes w/ cerebriform nuclei and scant cytoplasm)
15
Q
Diffuse Large B Cell Lymphoma
A
- Most common NHL
- Aggressive w/ risk CNS spread
- Present w/ rapidly enlarging tumor at nodal or extra-
nodal site - Genetics - 30% have abnormal 3q27 involving Bcl-6 gene
- Excellent outcome and response to chemo