Lymphoma Flashcards
Pediatric HL is more common than
non-Hodgkin lymphoma (NHL), with an annual incidence rate of 12.8 per 1 million children (≤19 years old)
T or F?
True
The childhood form occurs in patients age ___ years or younger
14
The childhood form of HL is
associated with ______ family size and _____ socioeconomic status.
The childhood form of HL is
associated with increasing family size and decreasing socioeconomic status.
Early and intense exposure to an infec-
tious agent has been speculated to ______ the risk for the childhood form of HL
increase
In contrast to childhood HL, young adult
HL is associated with a _____ socioeconomic status
In contrast to childhood HL, young adult
HL is associated with a higher socioeconomic status, as
found in high-income countries
Chang et al.29 demonstrated that early exposure to other children at nursery school and day care seems to _____
the risk of young adult HL, most likely by facilitating childhood exposure to common infections and promoting maturation of cellular immunity
Decrease
What are the Two distinct immunophenotypes of HL
Two distinct immunophenotypes of HL exist.
The first immunophenotype, characteristic of L&H cells, consistently expresses CD20 and J chain and does not express CD30 and CD15.
The second immunophenotype, characteristic of
HRS cells, consistently expresses CD30, frequently expresses CD15, and does not express J chain.
Classical HL is further subclassified into:
nodular sclerosing (NS) mixed cellularity (MC), lymphocyte-rich (LR), and lymphocyte-depleted (LD) histologies based on their unique morphology.
___ is the most common subtype in all age groups, it is more frequent in ado-lescents (77%) and adults (72%) than in younger children (44%)
NS
Conversely, ___ is more common in younger children (33%) than in adolescents (11%) or adults (17%).
MC
Most children (80%) present with _____
cervical lymphadenopathy
One-third of patients have one or more of the so-called _____ at diagnosis (unexplained fever >38°C with recurrent episodes during the previous month, drenching night sweats recurrent during the previous month, or weight loss of more than 10% in the
6 months preceding diagnosis).
B symptoms
The diagnosis of HL is made by _____ and is con-
firmed pathologically by the presence of HRS cells and their
mononuclear variants
The diagnosis of HL is made by lymph node biopsy and is con-
firmed pathologically by the presence of HRS cells and their
mononuclear variants
Imaging studies of the thorax include a chest radiograph
and a computed tomography (CT) scan, which alters treatment
decisions in at least ___% of patients through delineation of
radiographically inapparent disease involving subcarinal, hilar,
or cardiophrenic angle nodes, and in extranodal sites (pleura,
chest wall, or pericardium).
10%
_____ is a standard method to assess mediastinal bulk (mediastinal to thoracic ratio of ≥33%).
Using the ratio of the measurement
of the mediastinal mass to the maximum diameter of the intra-
thoracic cavity on an upright chest radiograph
Infradiaphragmatic disease is best assessed by _____
CT scan or magnetic resonance imaging (MRI)
However, several fac-
tors continue to influence the choice and success of therapy.
These factors are interrelated in that ___, _____ and _____ are frequently codependent
disease stage
bulk, and
biologic aggressiveness
On univariate analysis, what are the factors noted significant for inferior disease-free survival (DFS) and overall survival (OS).
stage IV NS HL B symptoms white blood cell count (WBC) of ≥11,500/mm3 hemoglobin ≤11.0 g/dL bulky mediastinal disease extranodal disease erythrocyte sedimentation rate (ESR) ≥50 mm per hour
By multivariate analysis, what are the factors significant for inferior DFS and OS
male gender
stage IIB, IIIB, or IV disease
WBC ≥11,500/mm3
hemoglobin ≤11.0 g/dL
______ is the most significant prognosticator of
treatment outcome
Stage of disease
defined as a mass of more than one-third of the intrathoracic diameter, is associated with an increased risk of disease recurrence, particularly when managed with radiation therapy alone.
Large mediastinal adenopathy
the third most common form of childhood can-
cer, comprising 15% of cancer diagnoses in individuals younger
than age 20 years.
Lymphoma
Worst outcome histology of HL
Lymphocyte depleted (LD)
________ evolved in an effort
to reduce therapy-related toxicities; reduced radiation dose was
combined with non–cross-resistant chemotherapy in pediatric
patients.
Combined-modality treatment programs
What does MOPP and ABVD stand for?
include MOPP (mechlorethamine, vincristine, procarbazine, and prednisone),
ABVD (doxoru-
bicin, bleomycin, vinblastine, and dacarbazine),
unfavorable disease features are?
unfavorable disease features such as more bthan three nodal sites presence of bulky mediastinal lymphadenopathy (mediastinal ratio ≥33% peripheral nodal mass ≥6 to 10 cm) extranodal extension
High risk disease are?
High-risk HL patients are those with stage IIIB and any with stage IV
What does rapid early response to therapy means?
rapid early response to therapy (>60% reduc-
tion in the tumor dimension)
Treatment failures in pediatric HL patients typically develop
within the _____ years, although late relapses have been
reported, particularly in patients with lymphocyte-predominant
HL
first 3 years
The most common site of relapse following risk-adapted
therapies remains the ________.
primary site of disease
Recommended therapy for st I, IIa favorable risk?
Recommended therapy
2–4 cycles non–cross-resistant chemotherapy (Oepa, Vamp, COpp-aBV, aV-pC).
Response-based low-dose, involved-field radiation (15 Gy to 25.5 Gy).
Other considerations:
Consider use of IFRT based on early response to chemotherapy if CR after 2 Oepa no need for RT
Recommended therapy for intermediate risk?
3–6 cycles compacted, dose-intensive, non-cross-resistant chemotherapy (Oepa/COpp, aBVe-pC) plus
low-dose, involved-field radiation (15 Gy to 25.5 Gy).
Other considerations: Early response to therapy may be considered in determining need for radiation in
those achieving CR.
For high risk, recommended therapy?
4–6 compacted, dose-intensive cycles of non–cross-resistant chemotherapy (Oepa/COpp, aBVe-pC)
plus low-dose, involved-field radiation (15 Gy to 25.5 Gy)
Other considerations: 8 cycles non–cross-resistant chemotherapy alone (BeaCOpp).
What is IFRT?
The definition of involved fields depends on the anatomy of
the region in terms of lymph node distribution and patterns of
disease extension into regional areas.
Involved fields typically should include not just the identifiably abnormal lymph nodes but also the entire lymph node region containing the involved nodes
Although IFRT remains the standard when patients are treated with combined-modality therapy, response-adapted RT is
under investigation. What is response adapted RT?
response-adapted RT, which is limited to
areas of initial bulk disease (generally defined as ≥5 or 6 cm at
the time of disease presentation) or postchemotherapy residual
disease (generally defined as ≥2 cm, or residual PET avidity), is
under investigation.
Height reduction is a potential long-term consequence of irra-
diation, most frequently with ____ Gy and most severe in
prepubertal children.
doses >20 Gy
Radiation-associated pericardial and myocardial disease are
related to :
dose (including fraction size)
volume
Complicated by anthracycline use
Increase in mortality risk, due to premature coronary artery disease and acute myocardial infarction, has been demonstrated in patients who received mediastinal radiation in doses ___ Gy before 20 years of age
> 30
Thyroid dysfunction may result from neck or upper mediastinal
irradiation and most often is manifested by an ______.
elevated serum concentration of thyroid-stimulating hormone (TSH)
Most common thyroid disturbance post RT is?
Hypothyroidism
Risk factors for premature menopause
include:
attained age,
exposure to increasing doses of ovarian radiation
increasing alkylating agent score (based on number
of agents and cumulative dose), and
diagnosis of HL
In an effort to preserve ovarian function, it is rec-
ommended that the ovaries are transposed via ______ to
a shielded area laterally or inferomedially near the uterine cer-
vix prior to the initiation of radiation.
oophoropexy
In boys irradiated to the pelvis, oligospermia is common, but
it is reversible (usually by ____ months)
18 to 24 months
_______ is the most com-
mon solid second malignant neoplasm following the treatment
of children. The increased risk of _______ is a significant
concern for women treated for HL at a younger age.
Breast cancer
_____is an anti-CD30 antibody conjugated to a synthetic antimicrotubule agent, monomethyl auristatin E, recently approved by the U.S. Food and Drug Administration for the treatment of relapsed or refractory HL, has shown excellent response in refractory HL.
Brentuximab vedotin
Evidence of ____ infection has been
demonstrated in the majority of endemic (mainly in equatorial
Africa) Burkitt tumors and in about 15% of sporadic cases (out-
side of Africa), suggesting a significant role for this virus in
lymphomagenesis through unknown mechanisms
EBV
EBV infection has also been associated with the development
of ________, a B-cell
lymphoproliferative disorder seen in patients after solid organ
or hematopoietic stem cell transplantation.
posttransplant lymphoproliferative disease (PTLD)
Pediatric NHL cases are divided into four major
histopathologic subtypes:
(a) Burkitt lymphoma (BL ), (b) lymphoblastic lymphoma (LL), ( c) diffuse large B-cell lymphoma (DLBCL), and ( d) anaplastic large cell lymphoma (ALCL).
______ is the most common site of disease in sporadic BL,
Abdomen
However, in endemic BL, ______ is the most common, and bone marrow involvement is rare.
jaw involvement
The majority (50% to 70%) of children with precursor T-cell LL present with \_\_\_\_\_\_\_
rapidly enlarging neck and mediastinal lymph-
adenopathy
MC with SVC
_______ can be locally invasive and may present with SVC
syndrome.
Primary mediastinal large B-cell
lymphoma
most frequent sites of involvement in systemic ALCL
are _____
peripheral nodes, mediastinal lymph nodes, and extra-
nodal sites such as skin, soft tissue, and bone
In NHL, ______ remain the primary means of establishing the definitive diagnosis, karyotype and molec-
ular studies will be vital for implementation of an optimal
treatment plan.
histology and immunophenotype
In NHL, If the patient’s condition does
not permit an open biopsy (e.g., a large mediastinal mass is
causing airway obstruction or compression of SVC), then use
of a less invasive means of sampling tumor cells with as mini-
mal anesthesia as possible, such as ______
percutaneous fine-needle
aspiration or examination of pleural fluid, ascites, periph-
eral blood, or bone marrow
T or False?
The use of irradiation or steroids before biopsy for respira-
tory distress may result in rapid shrinkage of the mediasti-
nal mass but may jeopardize the ability to establish a tissue
diagnosis
True
These special features of childhood NHL include:
the pre-ponderance of extranodal presentations,
the noncontiguous pattern of disease spread,
and a tendency to evolve into leukemia
and to involve the central nervous system (CNS).
Most common staging system for NHL of pediatric age group
The St. Jude Children’s Research
Hospital staging system is used most widely
The distinction between lymphoma and leukemia lies in the
percentage of malignant cells in a bone marrow aspirate.
Patients with _______% blasts in bone marrow are con-
sidered to have acute leukemia, and those with between ___% marrow involvement are considered to have stage IV
NHL.
The distinction between lymphoma and leukemia lies in the
percentage of malignant cells in a bone marrow aspirate.
Patients with more than 25% blasts in bone marrow are con-
sidered to have acute leukemia, and those with between 5%
and 25% marrow involvement are considered to have stage IV
NHL.
The most important factor in determining prognosis in child-
hood NHL is ____
stage
T or F?
COMP regimen was superior to LSA2L2 for BL
True
In God, All things are possible?
AMEN
Burkitt lymphoma/
leukemia is a rapidly growing tumor and is often associated with__________
tumor lysis syndrome (including hyperuricemia)
In NHL, _____ is reserved for emergency treatment
of mediastinal disease or symptomatic neurologic compromise
such as spinal cord compression, palliation of pain, consoli-
dation before bone marrow transplantation in patients with
recurrent disease, and treatment of overt symptomatic CNS
lymphoma at diagnosis or relapse.
Radiation
T or F
IFRT has been eliminated from the
design of advanced-stage pediatric NHL trials. Recent results of
chemotherapy-alone trials tend to support this practice
True
In NHL, For palliation, RT at total
doses as low as _____Gy fractions can result in rapid relief
from symptoms associated with such conditions as SVC syn-
drome, acute respiratory distress, spinal cord compression,
and orbital proptosis
4 Gy in 2- Gy per fraction
In NHL, Indications for cranial irradiation are currently limited to
________.
patients with overt symptomatic CNS lymphoma at diagnosis,
particularly when unresponsive to initiation of chemotherapy
or dexamethasone or when there is CNS relapse
