Hodgkins Lymphoma, ASPHO Flashcards
1
Q
what are the types of classic HL?
A
LLMN
- Nodular sclerosing
- mixed cellularity
- lymphocyte predominant
- lymphocye depleted
2
Q
What is the type of HL that is not classic?
A
Nodular Lymphocyte predominant
3
Q
Give 5 types of NHL
A
- Burkitt
- Diffuse large B
- Primary mediastinal B cell
- Lymphoblastic
- Anaplastic large cell
4
Q
Give a B-cell derived, immature lymphoma
A
B-lymphoblastic
5
Q
Give a B-cell derived mature lymphoma (4)
A
burkitt
diffuse large b
hodgkin
primary mediastinal b
6
Q
Give a t-cell derive, mature lymphoma
A
anaplastic large cell
7
Q
most common peds malignancies in 0-14 age range? #1-5
A
1- leukemia 2-cns 3-lymphoma 4-neuroblastoma 5-rhabdo/STS
8
Q
most common peds malig in age 15-19? #1-5
A
1-lymphoma 2-germ cell 3-leuekmia 4- CNS 5- Rhabdo
9
Q
Below the age of ___, NHL more common than HL
A
10
10
Q
HL: classic cell?
A
Reed Sternberg= owl eye
11
Q
describe reed sternberg
A
owl’s eye, binucleate, thick nuclear membrane, pale hcromatin, large eosinophilic nucleoli
12
Q
Lymphocyte predom: describe cell
A
mononuclear variant of hodkgin reed sternberg cell, may have several nucleoi, convoluted irreg nucleus= popcorn
13
Q
in HL, and LP, neoplastic cells compreise
A
<1%
14
Q
artifcant of formulin fixation: islands…called what?
A
lacunar cells
15
Q
Nodular sclerosing HL:; have a __ capsule
A
thick
16
Q
NS HL tends to involve what area of body? (2)
A
neck, mediastinum
17
Q
what = #2 most common type of classic HL?
A
mixed cellularity HL
18
Q
mixed cellularity HL tends to be seen in what age?
A
<10
19
Q
EBV often involved in what type of HL?
A
mixed cellularity..seen lymphocytes, histiocytes, eos
20
Q
what type of HL is often advanced at dx?
A
mixed cellularity HL…and almost always in lymphocyte depeleted
21
Q
lymphcoyte rich HL: see what?
A
SHEETS of lymphs!
22
Q
lymphocyte depleted HL: see what?
A
large bizarree malig cells, few lymphocytes, disorganized fibrossi, rare in kids
23
Q
immunophenotyping: in classic HL, what do you find?
A
CD15+, CD30+, CD45 NEG
24
Q
immunophenotype in noedular lymphocyte predom HL, what do you see?
A
CD15 neg, CD30 neg, CD45 POS
25
what is most common histo of HL?
nodular sclerosing
26
NS HL becomes more common with what?
wiht age
27
young adults: thinking which type of HL?
NS
28
<10 years and not advanced: think which type of HL?
lymphocyte rich
29
<10 years AND ADVANCED: think which type of HL?
mixed cellularity HL
30
adults with HIV: think which type of HL?
lymphocyte depleted
31
young, male, localized: think which type of HL?
nodular lymphocyte predominant
32
in terms of HL, list type most seen with EBV--> least
Lymphocyte depleted>mixed cellularity>lypohcyte-rich (42%)>NS>nodular lympohcyte preodmaintn (rare)
33
Histo subtypes of classic HL are based off what 3 thigngs>
tissue architectures, fibrosis, inflammatory infiltrate...not used in risk stratification
34
classic HL: most common--> least common type?
NS>mixed cellularity>lymphocyte rich
35
risk of having HL if your parent has it? sibling? monozygotic twin?
Parent: 3x
Sibling: 6x (more likely if sister)
Mono twin: 99x
36
pateints who are immunodef: increased risk of HL-- 4 egs?
sarcoid, ALPS (51x), Ataxia telangiectasia, HIV
37
4 immune-related ways that HL can present?
ITP, AIHA, AI neutropenia, nephrotic syndrome
38
in 0-14 age range, 3 RFs or HL?
low SES, larger family, early/intense expsoure to infections?
39
in 15-25 age range, 4 RVFs for HL?
higher SES, small family/less siblings/playmates, single family house, late expsoure to infections
40
what is always active in classic HL cells?
NFkappaB, either via LMP1 if EBV+ or IkappaBmutation
41
what are the mechanisms by which HL cells resist immune system?
MHC 1 expression is low/absent in most HL--> less T-cell recognition; PD-1 is present on HRS cells--> T-cell anergy, growth inhibition
42
Give the malignant cell of classic HL vs. nodular lympohcyte predominant HL:
classic: Hodgkin reed-sternberg cell; NLP: LP cells (popcorn)
43
Give the origin of classic HL vs NLP
classic: pre-apoptotic GC B cell; NLP: GC/post-GC B cell
44
give the phenotype of classic vs NLP HL
classic: CD15+, CD30+, CD45 neg, b-cell markers downregulated...NLP: cd15 neg, cd30 neg, cd45 pos, cd20 pos, pos ofr CD19, 20, 79a, PAX5
45
clicial pres of HL?
- painless cervical or supraclav LAD
- mediastinal mass (~2/3)
- constitutional symptoms
- evdience of inflamm and RES activaiton (increased CRP, ESR, ferritin, anemia of chronic inflamm)
- immune dysreg (uncommon)= AIN, AIHA, ITP, nephrotic syndrome
46
which constitutional symptoms =B symptoms are prognostic in HL?
wt loss of 10% in 6 months, drenching night sweats, unexplained fevers >38 for 3 consecutive days
47
which symptoms are NOT prognostic in HL?
fatigue, anorexia, mild wt loss, pain immeidately following alochol, pruritus
48
NLP: what percent of HL? what % are pre-pubertal? what % male?
5-8%, 10-20%, 75%
49
describe presentation of NLP HL
localized disease, no b symptoms, not bulky, not systemic, EBV negative
50
good thing about NLP HL?
might only need surgery for stage 1A
51
bad thing about NLP HL?
10-14% transform into DLBCL
52
buzzwords for NLPL HL? 4
YMCA! young, male, contained, asymptomatic
53
steps to eval HL?
- H&P, including b sytmpoms, LAD< SVC syndrome, superior mediastinal syndrome
- CBC, biochem, inflamm markers
- CXR
- excisional LN bx
- PET-CT or PET-MRI has largely replaced BM bx
- CT of chest to assess for lung involvemnt (MRI inadequate)
54
Give 10 ddx for mediastinal mass
- normal thymus
- infection (ebv, atypical myocbacterium, histoplasmosis, toxoplasmosis)
- lymphoprolierative disrodres
- progressive transformaton fo the germinal centres (PTGC)
- HL
- Lymphobolastic lymphoma
- DLBCL
- Priamry mediastinal b cell lymphoma
- anaplastic large cell lymphoma
- germ cell tumour
- soft tissue sarcoma
- lymph node mets from other sites
55
FDG-PET: glucose analog: 3 reasons for false +?
inflamm, thymic rebound, brown fat
56
When do you use FDG PET for HL?
- staging at dx
| - response to therapy (complete metabolic response-=remission)
57
what's more sens/spec...FDG PET or CT for lymphoma?
PET
58
is FDG PET appropriate for off therapy surveillance for recurrence?
No
59
what's the scale used to eval HL response to tx?
Deauville scale
60
describe teh deauville scale
```
1- no uptake above background
2-uptake= mediastinum
3- uptake >mediastinum but = liver
4- uptake moderately> liver
5- uptake markedly>liver or new lesions
...1,2= complete metab response...3= usually CMR but context depedent...4,5= active disease....3 at interim analysis often considered cMR
```
61
what's the staging used for HL?
Ann Arbor Staging
62
Describe the stages of Ann Arbor Staging
Stage I: single node (I) or single extra-lymphatic organ or site (IE)
Stage II: 2 or more node regions on the same side fo the diapghram (II) or one node region and one localized lymphatic site on same side of the diaphgram (IIE)
Stage III: node regions are involved on both sides of the diaphragm (III), may include an extranodal site (IIIE) the spleen (IIIs) or both (IIISE)
Stage IV: diffuse/disseminated involement of 1 or more extranodal site or tissue (liver, lung, marrow)
63
in ann arbor staging, give the stage classifications (HL):
```
A= asymptomatic
B= b symptoms
E= invoment of extra-lympathic organ
S= splenic involvmenet
X= bulky mediastinal disease
```
64
describe bulky disease in ann arbor staging (HL):
mediastinal mass: tumour >1/3 thoracic diameter on PA CXR; nodal aggregate >6 cm in the longest transverse diameter; macroscopic splenic nodules: focal defects on CT, PET, or MRI
65
give 5 unfavourable prognostic factors in peds HL
```
Advanced disease at dx
B symptoms
bulk disease
Extranodal extension
Slow/incomplete response (PET>CT)
```
66
What qualifies as Low risk HL for COG?
I/IIA, no bulk
67
What qualifies as Intermed risk in HL for COG?
I/IIA + bulk
I/IIAE
IIIA
IB, IIB no bulk
68
What qualifies as High risk in HL for COG?
IVA
IIB+Bulk
IIIB, IVB
69
does HL require LP, IT chemo, craniospinal rad?
NO
70
survival in HL?
>90% even with adv disease
71
what determines tx in HL?
stage/prog factors at dx
| objective resposne to therapy (PET>CT)
72
ongoing trials in HL are designed to waht?
decrease chemo, decrease rad, use targeted therapy (brentuximab anti-cd 30, checkpoint inhibitors)
73
radiation dose in HL?
20-25.5 Gy in 1.5-1.8 Gy fractions over 6-8 weeks
74
trial for IR HL? chemo? radiation?
AHOD0031...ABVE-PC...rad not needed in RER after cycle 2, CR after 4 cycles
75
tx relapsed HL how?
nove therapy--> high dose chemo and autologous transplant if respond...if not, do something diff
76
problems with transplant for relapsed HL cases?
- Late relapse (>12 months) of low stage disease don't need autologous SCT.
- primary refractory disease <3 months don't do well EVEN with transplant
77
targets for HL?
- CD30= classic HL
- EBV
- CD20 (NLPHL)
- PD-1, PD-L1, PD-L2
- NFkappaB, JAK/STAT
- block histone deacetylation
78
late effects in HL
SKELETAL: decreased bone growth, AVN from radiation and steroids
CARDIAC: cardiomyoapthy, effusion, pericardiits, CHF, CAD, AMI from radiation, alkyalating agents, anthracyclines
PULMONARY: pneumonitis, fibrosis from radiation, bleo, anthracylcines
ENDO: hypot4, female infert from radiation adn alkylating agents, male infert from alkylating agents
MALIG: solid tumours from raidation, alkylating agents; AML/MDS from alkylating agnets and epipodophyllotoxins
79
what's the msot common secondary malig in HL?
bresat cancer in women...esp if radiation at age 10 or older for rad....decreases with age though...no excess risk in women aged 30+....the greatest RELATIVE RISK is leukemia however
