Lymphomas in Children (see also adults) Flashcards
Lymphomas in Children
HL
What is the most common subtype of HL (in all ages)?
nodular sclerosing classic HL
Lymphomas in Children
HL
classic HL is divided into two categories based on age groups.
What are these?
childhood (0-14) young adults (15-34)
Lymphomas in Children
NHL is more common in childhood (0-14) than childhood HL.
TRUE or FALSE?
True
Lymphomas in Children
NHL is more common in adolescents than HL.
TRUE or FALSE?
False.
HL is more common
Lymphomas in Children
HL-Epidemiology
Childhood HL, which is rare in <4 but is common in >10 is associated with ____gender, _____ socioeconomic status, and ____family size.
male
decreasing
increased
Lymphomas in Children
HL-Epidemiology
Young adult classic HL is associated with ____ socioeconomic status, ____ family size, and ____ birth order.
higher
smaller
later
Lymphomas in Children
HL-Epidemiology
Childhood HL and early exposure to infectious agents are associated with ___ risk?
increased
Lymphomas in Children
HL-Epidemiology
young adult HL and early exposure to infectious agents are associated with ___ risk?
decreased
Lymphomas in Children
HL-Epidemiology
HL is divided into classic and NLPHL.
What are the subtypes of the classic HL?
NS
MC
LR
LD
Lymphomas in Children
HL
What is the most common subtype of HL in young children?
MC
Lymphomas in Children
HL
staining immunophenotype of classic HL?
CD30 and 15+
lacks J chain
Lymphomas in Children
HL
staining immunophenotype of NLPHL?
CD20+ and J chain
CD30- 15-
Lymphomas in Children
HL
HL is associated with EBV.
What subtypes of HL are most commonly associated with it?
MC and LDHL
Lymphomas in Children
HL
BONUS:
What are the symptoms that are the results of cytokine production by HRS cells? (B symptoms)
recurrent and unexplained fevers >38.3ºC during the previous month
drenching night sweats
weight loss of more than 10% in the 6 months preceding diagnosis.
Lymphomas in Children
HL
What is the most significant prognosticator of treatment outcome?
stage
Lymphomas in Children
HL
What are four the components of CHIPS? (prognostic scoring)
fever
stage IV
albumin <3.5
large mediastinal adenopathy
Lymphomas in Children
HL
Historically, what is the first effective systemic therapy for HL?
MOPP
mechlorethamine
vincristine
procarbazine
prednison
Lymphomas in Children
HL
Historically, this combination was made to reduce ovarian failure and s-AML associated with MOPP.
COPP
cyclophosphamide substitution for mechlorethamine
Lymphomas in Children
HL
Most significant toxicity related to bleomycin
pulmonary toxicity/fibrosis
Lymphomas in Children
HL
Most organ system affected by significant toxicity related to doxorubicin
cardiac
Lymphomas in Children
HL
One approach to reduce the risk of
cardiomyopathy caused by doxorubicin, an anthracycline agent, is to limit the
cumulative dose of anthracyclines for pediatric HL patients to a maximum
cumulative of ______, particularly in the case of favorable risk disease.
250 mg/m2
Lymphomas in Children
HL
What agent has been incorporated into the
treatment regimens for advanced and unfavorable HL to intensify therapy and as
an alternative to alkylating agents to reduce gonadal toxicity?
etoposide (a topoisomerase II inhibitor)
Lymphomas in Children
HL
This drug combination was made as a gender-adapted regimen for boys by the GPOH to limit alkylator exposure
OEPA
Vincristine, etoposide, prednisone, doxorubicin
Lymphomas in Children
HL
This study following HD-DAL-90 Europe showed that omitting IFRT in complete responders in intermediate- and high-risk patients, caused a drop in EFS to 79% compared to 91% for those who did get RT.
What is this trial?
HL-95
Lymphomas in Children
HL
What is this conjugate antibody (approved in adults as post transplant maintenance) that combines an anti-CD30 antibody with a synthetic tubulin disruptor, has been evaluated for salvage
HL and certain NHLs, and is currently being evaluated for its role in the up-front
setting in the treatment of high-risk HL in a current COG study?
BV
brentuximab vedotin
Lymphomas in Children
HL
BONUS: CD20 targeted treatment
Rituximab
Lymphomas in Children
HL
RT dose varies and in part is determined by disease and chemotherapy; but in general, what is the recommended standard RT dose?
(invalid question)
21Gy/1.5/14
definitely lower than in adults.
The dose of RT prescribed is in part determined by the intensity and regimen of
chemotherapy used in the combined modality approach. Typical doses in the
current era of low dose RT are 15 to 25 Gy, with doses rarely exceeding 25 Gy in
the pediatric setting.
In North America, the COG has set
the standard dose as 21 Gy in 14 fractions.
Lymphomas in Children
HL
OARs/Toxicity
What should be the mean lung dose to reduce the risk of radiation pneumonitis?
<15 Gy
<17 Gy in adults especially those who received Stanford V
Lymphomas in Children
HL
OARs/Toxicity
Dose related to height reduction which is most severe in prepubertal children
> 20 Gy
Lymphomas in Children
HL
OARs/Toxicity
Increase in mortality risk, because of premature coronary artery disease and acute myocardial infarction, has been demonstrated in patients who received mediastinal radiation in doses _____ before 20 years of age
> 30 Gy
Lymphomas in Children
HL
OARs/Toxicity
What chemotherapy regimen is associated with a recovery of fertility after a transient azoospermia and should be used in boys if future fertility is a concern?
ABVD
(stanford V as well in adults)
COPP is gonadotoxic
Lymphomas in Children
HL
COG risk stratification
Favorable risk
I and IIA without bulk
Lymphomas in Children
HL
COG risk stratification
Intermediate risk
I to IIA with bulk
I to IIAE, I to IIB
IIB and IIIA and IVA
Lymphomas in Children
HL
COG risk stratification
High-risk
IIIB and IVB
Lymphomas in Children
HL
EuroNet Cooperative Treatment Group
TG1
IA/B and IIA without bulk ≥200 mL
ESR <30 mm/hr
Lymphomas in Children
HL
EuroNet Cooperative Treatment Group
TG2
stages IEA/B, IIEA, IIB, or IIIA and stages IA/B and IIA with bulk
≥200 mL or ESR ≥ 30 mm/h
Lymphomas in Children
HL
EuroNet Cooperative Treatment Group
TG3
IIIEB, IIIEA/B, IVA/B
Lymphomas in Children
HL
What trial treated patients witth stage I/II HL with 4 cycls of VBEP and adapted adjuvant treatment based on the response.
Good response 70% reduction - 20 Gy RT to initially involved fields.
poor responders were given1-2 OPPA.
If no response after 1 cycle - RT 40 Gy.
If response exceeded 70% - 20 Gy involved fields.
5y OS - 97.5%
5y EFS - 91.1%
French Society of Pediatric
Oncology MDH90 trial
Lymphomas in Children
HL
What trial successfully omitted RT in complete responders?
-treated patients witth early stage HL (both classic and NLPHL)
complete response ≥95% reduction & ≤2 ml of initial volume; unconfirmed CR ≥75% or <2 mL
No difference in results of chemo alone and CMT
GPOH-HD 95
Lymphomas in Children
HL
What trial successfully omitted RT in complete responders?
It was modeled after GPOH-HD 95 but excluded NLPHL.
IFRT - 19.8 Gy except early stage with complete response.
Partial responders were boosted to 30 Gy and those with residual disease >100 mL was given 35 Gy.
GPOH-HD 2002
Lymphomas in Children
HL
What trial who investigated on the omission of RT in select low-risk patients adapted PET CT to assess for response?
Results are awaiting publication.
Two hundred and eighty-seven stage IA/IIA
patients were treated with three cycles of AV–PC (doxorubicin, vincristine,
prednisone, and cyclophosphamide). Those who achieved a complete response
after three cycles received no further therapy, whereas partial responders
received 21-Gy IFRT. Two-year OS was 100%; however, 2-year EFS for those
with complete response to chemotherapy and no RT was 80% compared to 88%
of those with partial response who received IFRT (P = .11). A subset analysis
suggested the utility of interim PET-CT after one cycle (PET1) of chemotherapy
in identifying patients who may be treated without RT after three cycles of AV–
PC. Of the patients with complete response who did not receive RT, the 2-year
EFS rate for those with a positive/equivocal PET1 versus those who had a
negative PET1 was 65% compared to 87%, respectively (P = .005). Similarly, 2-
year EFS in partially responding patients with a positive/equivocal PET1 versus
a negative PET1 was 82% versus 96%, respectively (P = .047).
COG AHOD0431
Lymphomas in Children
HL
What was the first randomized trial to compare standard and response-based treatment arms in order to risk stratify selected groups of patients for omission of RT or augmentation of systemic therapy?
This trial is now followed as the standard in intermediate-risk HL.
AHOD0031
Early response to therapy was determined by CT scan, and complete response status was determined by CT and gallium or FDG-PET scanning.
Functional imaging was obtained after two cycles (at the time of early-response assessment), and as the availability of PET scanning increased, more patients were able to obtain interim PET-CT scans as well.
All 1,712 patients received two cycles of ABVE–PC followed by response assessment.
Patients with RER received two additional cycles of ABVE–PC followed by a second response assessment.
Those with a CR were randomized to receive 21- Gy IFRT or no further therapy.
Patients with a RER who did not have a CR were all assigned to receive IFRT.
SER patients were all randomized to either two additional cycles of ABVE–PC or dexamethasone, etoposide, cisplatin, an cytarabine (DECA) followed by an additional two cycles of ABVE–PC.
All SER patients received 21-Gy IFRT after chemotherapy.
Four-year EFS rates were 85% overall and 86.9% for RER patients versus 77.4% for SER patients (P < .001).
The 4-year OS for the entire cohort was 97.8% and 98.5% for RER patients versus 95.3% for the SER patients (P < .001). The 4-year EFS rate was 87.9% for RER/CR patients randomized to receive IFRT versus 84.3% for those randomized to no IFRT (P = .11).
Lymphomas in Children
HL
NLPHL is usually treated with RT alone in adults.
What treatment was considered feasible by
COG AHOD03P1 as a monotherapy for pediatric patients and is the basis of a EuroNet prospective trial.
resection/surgery
Lymphomas in Children
HL
Two drugs used in combination in the relapsed setting for higher-risk patients with first relapses.
ifosfamide and vinorelbine
For
higher-risk relapses, a combination of ifosfamide and vinorelbine for pediatric
patients in first relapse was studied by the COG (AHOD00P1). This regimen
showed a very good overall response rate (CR/PR) of 78% and achieved good
stem cell mobilization for future autologous stem cell transplant once remission
is achieved, because studies have shown that patients undergoing autologous
stem cell transplant with active disease have a worse outcome
Lymphomas in Children
NHL
What is the most common histologic subtype in children?
Burkitt lymphoma
Lymphomas in Children
NHL
In 2016, what was added as a distinct entity in the WHO classification?
pediatric follicular subtype
Lymphomas in Children
NHL
Where is the most common site of presentation of endemic BL?
Jaw
Lymphomas in Children
NHL
Where is the most common site of presentation of sporadic BL?
abdomen
Lymphomas in Children
NHL
What is the most common presentation of precursor T-cell LL?
rapidly enlarging neck and mediastinal lymphadenopathy
Lymphomas in Children
NHL
In what histology is FDG-PET documentation of BM involvement is sufficient?
DLBCL
Lymphomas in Children
NHL
What is the most commonly used staging system?
Murphy’s
and
IPNHL
Lymphomas in Children
NHL
BL
What is the standard chemotherapy for BL (based on FAB/LMB96?
cyclophosphamide, vincristine, prednisone, high dose methotrexate,
doxorubicin, and cytarabine
(CVPMAA)
Lymphomas in Children
NHL
BL
What targeted treatment (as in adults) is presently investigated in pediatric BL and DLCBL as an addition to FAB/LMB96 or as upfront therapy.
Rituximab
Lymphomas in Children
NHL
Treatment options (chemo) for primary mediastinal B-cell lymphoma
R-CHOP
DA-EPOCH-R
