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