lymphoma I and II Flashcards
what is staging?
- looking for where the tumor is
- this is done in hodgkin and non-hodgkin lymphoma
some tests done in staging…
Labs: cbc, lytes, creatinin, BUN, LFTs
LDH: can be elevated in non-hodgkins
CT/PET: abd, pelvis, chest
BMB: bilateral
stage definitions I-IV
I - single lymph node
II - two or more lymph nodes on same side of diaphragm
III - two or more lymph nodes on different sides of diaphragm
IV - either multifocal involvement with one or more extra lymphatic site +/- associated lymph nodes OR involvement of extra lymphatic organ with distant nodal involvement
staging subclassifications
A - asymptomatic
B - either night sweats, fever, or greater than 10% weight loss in 6 months
what is meant by extra-lymphatic
tissue other than lymph nodes, thymus, spleen, Waldeyer’s ring, appendix, and Peyer’s patches
list some indolent lymphomas
- CLL
- follicular lymphoma
- marginal zone lymphoma
- MALT
list some aggressive lymphomas
- ALL
- Burkitt’s lymphoma
list some intermediately aggressive lymphomas
- diffuse large B cell lymphoma
- peripheral T cell lymphoma
- mantle cell lymphoma
DLBCL who gets it?
adults and children, but mostly adults, median 64
DLBCL curability and percent presence in bone marrow?
- curability 30-40%
- BM 15-20%
DLBCL benign equivalent
large replicating cells of germinal center and paracortex
DLBCL pathophysiology
diffuse infiltration of lymph node, increased mitotic rate, necrosis may be present
DLBCL markers
- CD19+, CD20+
- possible: bcl-6 and/or bcl-2 expression
- rearrangement of IgH, IgL chains
DLBCL subtypes of germinal center B cell origin, and which is better?
GCB - germinal center B cells (better survival)
ABC - activated B cell
characters of newly discovered highly aggressive DLBCL
over expression of “myc” oncogene and bcd-2
DLBCL five point scale and rating
1) over 60
2) stage III or IV
3) elevated LDH
4) poor performance status
5) extra nodal involvement
0-1 - low
2 - low intermediate
3 - high intermediate
4-5 - high risk
DLBCL treatment
- R-CHOP
- usually chemo as well
- autologous transplant only in refractory cases
what is R-CHOP?
- rituximab
- cyclophosphamide
- adriamycin (anthracycline backbone)
- vincristine
- prednisone
rituximab is anti-CD….
CD20
what percent of NHL is follicular lymphoma?
22%
who gets FL?
adults over 40, median 59
FL grades and aggressiveness
grade I - 5 or less large cells in follicle
grade II - 6-15 large cells
grade III - more than 15
grades I/II are considered indolent while grade III is treated as an intermediate large cell lymphoma
FL presents at what stage?
III or IV
FL life expectancy
7-10 years but highly variable because there is an aggressive type
FL benign equivalent
small cleaved cell of germinal center
FL cell characteristics
- clumped chromatin
- infrequent nucleolus
- like small lymphocyte but irregular nuclear profile with nuclear folds
FL follicle characteristics
- maintains normal structure, but monotonous proliferation of one cell type
FL chromosomal translocation
- t(14;18)
- upregulates bcl-2 which is anti-apoptosis
FL immunophenotype
positive: CD19, CD20, CD10, bcl-2
negative: CD5, cyclin D1
clinical course of FL
indolent - slow growing with relapses
FLIPI scale
five point scale
1) over 60yo
2) elevated LDH
3) stage III or IV
4) greater than 4 LN sites
5) hemoglobin less than 12
3-5 - high
2 - intermediate
0-1 - low
FL - reasons to move from watchful waiting to treatment
1) Bulky, painful adenopathy
2) Organ involvement or impairment
3) B symptoms (severe)
4) Cytopenias due to bone marrow involvement
5) Autoimmune cytopenias (Idiopathic Thrombocytopenic Purpura; Autoimmune Hemolytic Anemia)
6) High FLIPI score
FL treatment
stages I and II - radiation alone is usually sufficient
stages III and IV - gentle chemo including rituximab (R-CVP)
MZL aggression?
indolent
three subtypes of MZL
1) extranodal marginal zone B cell lymphoma (MALT)
2) nodal marginal zone
3) splenic marginal zone lymphoma
MALT MZL can be in the….
GI tract, tear duct, salivary gland, etc
splenic MZL can be in the
spleen or bone marrow
MZL treatment
same as FL
stages I and II - radiation alone is usually sufficient
stages III and IV - gentle chemo including rituximab
MZL different from other indolent lymphomas because…
tends to be curable
MZL cell of origin
post germinal memory B cell that has the capacity to become a marginal zone cell.
MZL cell markers
positive: CD19, CD20
negative: CD5, CD10, cyclin D1
gastric MALT lymphoma symptoms similar to…
peptic ulcer
gastric MALT lymphoma is driven by…which can lead to which translocation?
- H. pylori
- t(11;18) which can produce a more aggressive MALT lymphoma
gastric MALT treatment
if H. pylori positive: antibiotics
if H. pylori negative: radiation therapy or chemotherapy is necessary. Rituxan has also been proven to be beneficial
hairy cell leukemia aggressiveness
indolent
why HCL called a leukemia?
it’s in the blood and bone marrow but derived from a lymphocyte
HCL cell markers
positive: CD19, CD20
negative: CD5, CD10
HCL visualization
- “hairy” cytoplasmic projections
- in bone marrow, fried egg cytoplasm
HCL treatment
- purine analogue such as cladribine
- rituximab in relapse
Burkitt’s lymphoma agressiveness
highly aggressive
who gets Burkitt’s?
children, young adults, and immunocompromised
three types of Burkitt’s
1) endemic - africa, EBV, children’s jaws
2) immunocompromised - HIV/AIDS, 30% EBV positive, abdominal masses, marrow and nodal involvement, higher in cases with higher CD4 counts
3) sporadic - same as immunocomromised but in healthy children and young adults
Burkitt’s benign equivalent
small non-cleaved cell of germinal center
Burkitt’s pattern under a microscope
- starry sky
- because of macrophages attracted by ineffective proliferation, want to phagocytose
- this is all due to high mitotic rate with high failure
Burkitt’s chromosomal translocation
- t(8;14) - causes up regulation of “myc” oncogene
- myc is major “on switch” of cell cycle and causes rapid proliferation
how long is survival with Burkitt’s without treatment?
just a couples weeks
Burkitt’s treatment
aggressive multi-drug chemo for 6-8 months
who gets mantle cell lymphoma?
adults, mean age 63
mantle cell lymphoma aggressiveness
appears indolent but acts intermediate to aggressive
mantle cell benign equivalent
lymphocyte of inner mantle zone
mantle cell pathology
t(11;14) upregulates bcl-1, increases cyclin D1 production
treatment of mantle cell
- poor response to therapies
- 5 year survival is 30-50%
- allogeneic and autologous transplants can be helpful
commonality of T cell lymphoma
only 20% of non-hodgkin lymphoma
where are most T cell lymphomas?
extranodal
if T cell lymphoma is nodal……
very bad, worse than any B cell lymphoma
common skin T cell lymphoma
mycosis fungoides
anaplastic large cell lymphoma is what type of lymphoma?
T cell
what is the translocation and protein in anapestic large cell lymphoma?
t(2;5) - ALK1
ALK1 vs non-ALK1 lymphoma
ALK1 positive has 93% survival rate while ALK1 negative has only 37% survival
3 basic characteristics of Hodgkin lymphoma
- less common than non-hodgkin
- bimodal (adolescence and then again 6th decade)
- spreads contiguously in lymphatics and doesn’t often skip a region
what diagnoses hodgkin lymphoma?
reed sternberg cell, which is an altered B cell
hodgkin cell indicators
positive: CD15, CD30
negative: CD45
virus sometimes associated with hodgkin
EBV
4 subtypes of classical hodgkin lymphoma
1) nodular sclerosing
2) lymphocyte rich
3) lymphocyte depleted
4) mixed cellularity
nodular sclerosing classical hodgkin characteristics
- most common
- neck and anterior mediastinal adenopathy in young females
- fibrotic capsule and bands of subdividing tissue
- lacunar varient reed-sternberg cells
lymphocyte rich classical hodgkin characteristics
- limited disease in neck of young adults
- “L and H” or “popcorn” RS cells
lymphocyte depleted classical hodgkin characteristics
- often retroperitoneal
- aggressive
- pleomorphic RS cells
mixed cellularity classical hodgkin characteristics
- aggressive
- older patients
- mononuclear RS cells - more of them
crude disease activity measure in hodgkins
ESR
hodgkin overall cure rate
over 80%
hodgkin treatment of stages I and II
abbreviated chemo and radiation, in stage one radiation alone may suffice
hodgkin treatment of stages III and IV
ABVD x 6, anthracycline backbone
what is considered primary refractory disease in hodgkin and what is the prognosis?
- grows through therapy or relapses in less than six months
- aggressive and often fatal
three stages of mycosis fungoides
three stage process of mycosis fungoides
1) patch
2) plaque
3) tumor
mycosis fungoides tumor stage
- mushroom shaped
- predominant in groin, face, axilla, body folds
- generalized lymphadenopathy
- ulceration or necrosis
50% of those with CTCL die from….
staph or pseudomonas
sezary syndrome triad
1) erythroderma
2) leukemia (defining characteristic)
3) lymphadenopathy
sezary cells in peripheral blood
- large cells
- cleaved nuclei
- scant cytoplasm
