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121 Cards in this Set

  • Front
  • Back
Where does Non-Hodgkin's Lymphoma originate?
In the cells and organs of the immune system
In what cells does N-H lymphoma originate?
Undifferentiated lymphoid cells
T or F: N-H lymphoma is thought to result from genetic deviation that influences cell proliferation, differentiation, & cell death.
True
Recurrent, nonrandom chromosome translocations are seen in what disease's cytogenetic studies?
NHL
What immunodeficiency syndromes are associated with a higher risk of NHL?
1. Wiskott-Aldrich
2. Ataxia-telangiectasia
3. Other inherited or acquired immunodeficiencies
What role does the EBV virus have regarding NHL?
EBV is thought to precede or occur along with B-cell transformation (assoc. with Burkitt's lymphoma)
Why is histologic classification important in NHL?
It helps to identify the malignant cell of origin, therefore aiding in the understanding of the biologic behavior of the tumor.
How is Non-Hodgkin's lymphoma divided?
Into T-cell and B-cell lymphomas; subdivisions are specific to precursor characteristics
What four classifications does the WHO divide NHL into?
1. Lymphoblastic lymphoma (LL)
2. BL & Burkitt-like lymphoma (BLL)
3. Diffuse large B-cell lymphoma
4. Anaplastic large-cell lymphoma (ALCL)
T or F: LL accounts for one-third of all childhood lymphomas
True
What cell line accounts for 85-90% of all LL?
Immature Tcells undergoing differentiation in the thymus
What cell line accounts for only 10-15% of all LL?
only 10-15% express the phenotype of pre-B cells, along w/multiple cytogenetic abnormalities
What physical sign do 70% of T-cell LL patients present with at diagnosis?
Mediastinal mass
What symptoms occur in relation to the presence of a mediastinal mass?
Compression symptoms: dyspnea, stridor, wheezing, dysphagia, & swelling of the head & neck.
What area is one most likely to find lymphadenopathy in a LL patient?
Neck
Supraclavicular
Axillary
T or F: T-cell LL does not typically spread to the BM or CNS or gonads.
False: It commonly spreads to those areas
If a T-cell LL patient has >25% blasts in their bone marrow, what is their diagnosis?
Acute Lymphoblastic Leukemia (ALL)
How does B-cell origin LL typically present in terms of overall disease?
Localized disease of bone, lymph nodes, and skin
T or F: BL and BLL account for about 50% of all childhood NHL.
False: They account for about 40%
How is BL characterized in terms of chromosomal abnormalities?
Translocation is t(8;14;q24;q11) - involves gene for immunoglobulin heavy chain.
What are the common sites for disease in BL or BLL?
Abdomen and pelvis
What are some common presenting signs in BL or BLL?
1. rapidly enlarging mass
2. abdominal pain
3. intussusception
4. intestinal obstruction/perforation
What is associated with improved outcome in relation to the BL or BLL tumor?
Complete excision is indicated
T or F: Peripheral lymph nodes, bone and bone marrow are other sites of BL or BLL disease
True
What percentage of BL or BLL patients have CNS involvement?
approximately 10% of all cases
Where does endemic BL occur?
Africa and New Guinea
What percentage of childhood cancer does NHL account for in Africa & New Guinea?
50% of all childhood cancers are NHL
What distinctive features is endemic BL associated with?
Jaw involvement
EBV infection
How is Diffuse large B-cell lymphoma identified under a microscope?
1. basophilic cytoplasm
2. large nuclei
3. prominent nucleoli
T or F: Diffuse large B-cell lymphomas can be seen at genetic level.
True: they are a heterogeneous group
T or F: The mediastinal tumors in diffuse large B-cell lymphoma are molecularly the same as primary mediastinal tumors
False: the are molecularly different
T or F: Disseminated disease has better outcomes that those with mediastinal tumors.
False: pt's w/mediastinal tumors have superior outcomes to those w/disseminated disease
What is expressed on a cellular level in one-half to two-thirds of the cases of diffuse large B-cell lymphomas?
Surface immunoglobulin
What B-cell markers are expressed in diffuse large B-cell lymphoma?
CD19
CD20
CD22
CD79a
How do pediatric cases differ than adults?
Pedi cases of diffuse large B-cell lymphoma differ in distinct subtypes & translocations
Where doe 2/3 of all patients with diffuse large B-cell lymphoma present with disease?
Abdomen
Which disease is lymph node involvement more common; BL or Diffuse large B-cell lymphoma?
Diffuse large B-cell lymphoma
What sites of disease are more uncommon in Diffuse large B-cell lymphoma?
Bone marrow
Mediastinal
CNS
What percentage of all pediatric large-cell lymphomas does Anaplastic Large-cell lymphoma (ALCL) account for?
30-40%; They account for only 8-12% of pediatric NHL.
What cell features does ALCL have under the microscope?
anaplastic features: kidney-shaped nuclei often w/eosinophilic region near the nucleus.
T or F: ALCL often express null cell or T-cell antigens.
True
What antigen is also expressed but not as often in ALCL?
CD30 (Ki-1)
What does 90% of pediatric ALCL cases feature?
anaplastic lymphoma kinase (ALK) protein gene rearrangements
What is associated with a worse prognosis in ALCL?
Lack of ALK expression
What are the common presenting symptoms of ALCL?
peripheral, intrathoracic, or intrabdominal lymph nodes
Is bone involvement common in ALCL?
Yes, and is a primary site for disease
What organ involvment combination carries a poor prognosis in ALCL?
Bone Marrow
Lymph nodes
AND Skin
T or F: CNS involvment in ALCL is common
False: CNS involvement is rare
What is the incidence of lymphomas in developed countries?
Ranked 3rd after leukemia & brain tumors
15% of all childhood cancers
What is more common in children less than 10year; NHL or Hodgkin's disease?
Non-Hodgkin's Lymphoma
T or F: NHL is uncommon in children younger than 5 years old
True: incidence increased steadily in children older than 10years
T or F: Hodgkin's disease incidence is almost twice of NHL in children btwn 15-19yrs
True
What immunedeficiencies have and increased incidence of lymphoma in children?
1. Wiskott-Aldrich
2. SCIDs
3. X-linked lymphoproliferative
4. Ataxia-telangiectasia
5. AIDS
T or F: There is an increased incidence of NHL in children w/immunosuppression after solid organ & SCT.
True: particularly after T-cell depleted SCT
Is NHL more common in males or females?
3-4x higher in males in kids younger than 15yrs.
What are the 5 sites of clinical presentation of lymphoma?
1. Abdomen
2. Mediastinum
3. Head & Neck
4. CNS (rare)
5. Bone marrow
What will almost 2/3 of lymphoma patients clinically present with?
Locally advanced or metastatic disease
What are common presenting symptoms of lymphoma in the abdomen?
Abdominal pain, n/v, change in bowels, abd distention, palpable mass, intussusception, obstructive jaundice, and GI bleed.
What are common presenting symptoms of lymphoma in the mediastinum?
Dysphagia, subtle cough, wheezing, stridor, dyspnea, orthopnea, pericardial effusion, SVC syndrome w/distended neck veins, head & neck edema, inferior vena caval obstruction, pericardial tumor, or cardiac tamponade
What are common presenting symptoms of lymphoma in the head & neck?
Cervical lymphadenopathy, jaw swelling, unilateral tonsillar enlargement, nasal obstruction, snoring, rhinorrhea, or cranial nerve palsy.
What are symptoms associated with rare CNS lymphoma?
HA, vomiting, irritability, or papilledema
What are symptoms associated with lymphoma in the bone marrow?
pallor, anemia, thrombocytopenia
What are some other presentation sites for lymphoma?
Breast, testicular, skin, pharyngeal, nasopharyngeal, adrenal gland, epidural, thyroid, salivary gland, orbital, bone, muscle, or lung parenchyma
Why is it important to obtain diagnostic testing quickly if lymphoma is part of a differential diagnoses?
Due to the rapidity of tumor growth;
Prevent certain deadly conditions that are a result of a tumor mass
What is the fastest growing tumor in humans?
BL = Burkitt's Lymphoma; with a doubling time of 24hours
In what 2 types of lymphoma does tumor lysis syndrome typically occur in children?
1. BL = Burkitt's Lymphoma
2. Precursor Tcell lymphoblastic lymphoma
Regarding lymphoma - what needs to be determined before treatment is started?
The extent of disease
T or F: It is sufficient to obtain a unilateral BMA & Biopsy in suspected lymphoma patients
False: Bilateral is standard
What is the diagnosis if a BMA has greater than 25% blasts?
Acute leukemia
Why is an open biopsy of the tumor recommended for suspected lymphoma patients?
It permits sufficient tissue sampling for histology, morphology, cytogenetics, immunophenotyping, molecular and enzymatic studies
What alternative is there for diagnostic testing if the patient is unstable?
Fine needle aspiration of mass, pleural fluid, or CSF often contains malignant cells.
What 2 types of lymphoma often presents with fluid positive for malignant lymphoma cells?
LL = Lymphoblastic Lymphoma
BL = Burkitt's Lymphoma
Why is a chest CT especially important when making a lymphoma diagnosis?
Assesses airway prior to any invasive procedure in pt. w/mediastinal mass
If CNS disease is suspected, what diagnostic tests should be included in the workup?
LP to assess CSF, MRI to detect disease or endoscopy
What is the 5-year event free survival rate for children with NHL early stage?
85-90%
What is the 5-year event free survival rate for children with NHL advanced-stage disease?
70-90%
What has pediatric NHL historically been classified according to?
Murphy Ann Arbor System
What staging system is commonly used today?
St. Jude's Children Research Hospital
What therapy has increased overall survival for LL & BL?
CNS-directed therapy
What therapy has NOT increased overall survival for NHL?
Radiation therapy
What non-therapy improvements have increased overall survival for NHL?
1. Classification systems (biology, immunology, molecular biology)
2. Imaging and staging
3. Advances in supportive care
4. Application in chemotherapy
What qualifies "limited-stage" disease in NHL?
one or two masses on one side of the diaphragm
How many different stages of NHL are there?
4 stages
According to St. Jude Children's Research Hospital Staging System
What does initial treatment for NHL focus on?
Managing presenting symptoms assoc. w/BM invovlement, mediastinal or intrabdominal mass, hyperuricemia, tumor lysis
What purpose does surgery serve with NHL diagnosis?
necessary for staging & resection in emergent situations; also complete resection w/isolated tumor.
Why would chemotherapy be delayed after a complete resection of an isolated NHL tumor?
Surgery increases risk for fistula and perforation post Op.
Is radiation a routine therapy for NHL?
No, has no "routine" role. Exception is w/emergency situation - airway blocked by tumor, spinal obstructions.
What NHL diagnosis is likely to recieve Cranial XRT?
Tcell Lymphoblastic Lymphoma
T or F: Chemotherapy is the primary therapeutic modality regardless of stage or site(s) of disease in NHL
True - tumor responds to many different chemotherapy agents
What meds are used for CNS prophylaxis in NHL patients?
1. MTX
2. Cytarabine
3. Both of above
T or F: Children w/Stage I or II have excellent prognosis
True - 5-year disease free survival of 85-95%
What 3 types of NHL are treated similarly?
1. BL
2. BLL
3. Large B-cell lymphoma
* Bcell histology all similar
What is the typical general type & length of treatment for Stages I and II?
Multiagent intensive chemo for 3-6 months (approx. 90% survival rate)
What chemotherapy is used typically for Stages I and II of NHL?
Cytoxan (cyclophosphamide)
Vincristine (Oncovin)
MTX
Prednisone
Adriamycin
What chemotherapies do the newer NHL protocols contain?
1. High dose MTX in initial therapy
2. Other agents (VP16, Ifos, HD AraC)
T or F: Lymphoblastic lymphoma (LL) is treated w/regimens similar to ALL protocols.
True - 3 phase multi-agent protocol; w/CNS prophylaxis
What 3 phases are part of LL treatment? & How long does it last typically?
1. Induction
2. Consolidation
3. Maintenance
- 15 to 36 months
What is the overall survival rate for LL patients with limited disease? extensive disease?
LL limited disease = 80-90%
LL extensive disease = 60-80%
What is a recently identified sub-type of NHL? & what is standard therapy?
- ALCL (Anaplastic large cell lymphoma).
- No standard therapy determined yet.
What is the overall survival rate of ALCL? & what treatment protocols are typically used?
- Overall survival ALCL = 80-90%
- Protocol similar to ones used for Bcell lymphomas
What treatment for ALCL has been effective for Stages I and II?
3 cycles of Cyclophos, Doxo, VCR, & prednisone
Is radiation used to treat Stages I and II of ALCL?
No.
When is CNS prophylaxis used for NHL in Stages I and II?
Used in most NHLs except those disease where CNS spread is uncommon & those w/minimal disease not involving head/neck/epidural areas
What does CNS prophylaxis entail for NHL in Stages I and II (limited-stage disease)?
- MTX alone
OR
- MTX & AraC
While CNS directed therapy has improved overall survival in LL & BL; has radiation?
No
What stages are classified as advanced-stage disease?
Stage III and IV.
REad page 22 for Staging System for NHL.
Stages I through IV.
What NHL diagnoses can be classified as advanced-stage diseases?
1. Burkitt's lymphoma (BL)
2. Lymphoblastic Lymphoma (LL)
3. Large-cell lymphoma w/recurrent disease
What chemotherapy is used to treat Burkitt's lymphoma?
1. Cyclophosphamide
2. High dose MTX
3. Cytarabine
4. more recently - Ifos & VP16 in advance stages of BL
In patients with BL treated for more than 6 months, what is the survival rate?
80% are cured
In advance-staged LL what is treatment and 5-year disease free survival rate?
2 year multi-agent chemo similar to high risk ALL treatment w/IT chemo & cranial XRT
- DFS = 80%
What chemotherapy is used to treat advance-staged Large-cell lymphoma and recurrent disease?
1. VCR (Vincristine)
2. Prednisone
3. Doxo; Substituting MTX after cumulative dose is reached.
What has been said to likely increase the survival rate of Large-cell lymphomas & recurrent disease?
The addition of monoclonal antibiodies such as Rituximab, to chemo regimens.
What is the common thought regarding Large-cell lymphoma and treatment in regards to aggressiveness?
Many feel a more aggressive approach to limited-stage disease is warranted & should be treated w/same intensive & prolonged regimens as advanced-staged diseases.
How has the common held belief mentioned above effected treatment regimens?
Chemo's initially used for salvage therapy and used for initial therapy
What NHL patients are candidates for SCT?
Those w/responsive-relapsed disease, even if NOT in complete remission.
What is a NHL patients 5-yr DFS if SCT is warranted?
50% DFS at 5-years.
T or F: SCT conditioning regimens may or may not include TBI for relapsed disease
True - no clear-cut advantage has been determined either way
T or F: ALCL has favorable outcomes with autologous transplant. And allogeneic transplant.
False - poor outcome w/AUTO

ALLO - being explored d/t Graft VsTumor effect
What chemo regimens are used in the case of relapsed ALCL?
Single-agent Vinblastine (VCR, Cis-retinoic acid, alpha-interferon)
What are the 4 goals of nursing assessment and intervention in relation to NHL?
Pt. will experience minimal complications r/t 1. lymphoma; 2. treatment.
Pt. & family will 3. be adequately informed of diagnosis, disease, & treatment; 4. adequately cope w/diagnosis.