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77 Cards in this Set
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- 3rd side (hint)
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What are the cell surface markers that are consistent with a diagnosis of Hodgkin's Lymphoma?
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CD15, CD30, PAX-5 gene product
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What are the 4 subtypes of classical HL?
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Nodular sclerosis
Mixed cellularity Lymphocyte rich Lymphocyte depleted |
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Which type of HL characteristically presents with an anterior mediastinal mass and HRS cells that are described as "lacunar variants"?
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Nodular sclerosis
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Which subtype of HL is associated with up to 60-70% incidence of detectable EBV DNA?
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Mixed cellularity
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Which subtype of HL has lymphocytes that exhibit the features of mantle cell lymphoma?
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Lymphocyte-rich. This type is also less likely to relapse but more likely to be fatal if it does.
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Which B-cell cell surface markers are typically absent on HL cells?
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CD20, CD45, CD79a, Surface Ig
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Which type of HL has the histologic appearance of "popcorn" or "lymphocytic and histiocytic" (L&H) cells?
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Nodular lymphocyte predominant HL.
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What are the characteristic cell surface markers in nodular lymphocyte predominant HL, and how is this different from classical HL?
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NLP HL is positive for CD20, CD79a, CD45. Unlike classical HL, the cells do not express CD 15 or CD30
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What is the expected clinical course for NLP HL?
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Prolonged survival despite frequent relapse
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What is the role of PET scanning in the managment of Hodgkin Lymphoma?
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Useful in staging, and better than prognostic index use for determing risk of relapse. In one study 97% of patients with a negative scan after two cycles of ABVD remained in relapse long-term.
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What is the standard treatment for non-bulky, low risk stage I/II HL?
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2-4 cycles of ABVD followed by involved-field radiation.
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What factors in stage I/II HL necessitate a more aggressive treatment approach?
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6 cycles of ABVD (or an augmented regimen such as BEACOPP or Stanford V) followed by radiation should be considered if:
-tumor mass >10 cm in diameter or >1/3 thoracic diameter -ESR > 50 - >3 sites of tumor involvement - B symptoms - extranodal disease |
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What is the standard treatment approach to stage III/IV HL?
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6-8 cycles of chemotherapy, usually ABVD.
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How does ABVD compare with Stanford V for advanced HL?
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In the largest Intergroup trial to compare the two regimens, presented at ASH 2010, there was no significant difference in outcomes, but Stanford V had a greater incidence of lymphopenia and neuropathy.
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How does BEACOPP compare to COPP-ABVD in advanced HL?
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It may result in improved survival as far as 10 years out (5% improvement), treatment related mortality, infertility and risk of MDS make it a questionable choice for good-risk patients. HSCT is more likely to be successful in patients treated with ABVD than those treated with BEACOPP.
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When is consolidative radiation indicated in patients with advanced stage HL?
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When there is residual (active) tumor > 5 cm in diameter, or macroscopic splenic disease.
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A 23 year old woman treated for HL has an enlarging density in the mediastinum 3 months after therapy is completed. How should this be managed?
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This is not uncommon for up to 6 months after therapy in patients younger than 25; the density may represent regenerating thymus. Serial imaging and possibly biopsy are used to sort this out and avoid unnecessary treatment.
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What percentage of patients with bulky HL in the mediastinum or retroperitoneum will have persistence of a radiographic abnormality at the conclusion of therapy?
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80%.
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How is relapse treated in Hodgkin Lymphoma?
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High-dose chemotherapy (ICE, CHLVPP) followed by autologous HSCT.
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What portion of patients who receive mantle radiation will be diagnosed with thyroid disease?
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About 2/3. Diseases include hypothyroidism, Graves' diseas, silent thyrotoxicosis, thyroid nodules and thyroid cancer.
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How is treatment of Nodular Lymphocyte predominant HL different from that of classical HL?
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Involved field radiation is first line (similar to follicular lymphoma). Recurrence can be treated the same way if it is outside the previous radiation field.
Single-agent Rituxan has a response rate of 70-100%. Extensive disease can be treated with ABVD or Rituxan. |
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Describe Castleman disease.
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Castleman's disease (giant or angiofollicular lymph node hyperplasia, lymphoid hamartoma, angiofollicular lymph node hyperplasia) is an uncommon lymphoproliferative disorder that can involve single lymph node stations or can be systemic. It must be distinguished from reactive lymph node hyperplasia and malignancies. It is a very rare disorder characterized by non-cancerous growths that may develop in the lymph node tissue at a single site or throughout the body. It involves hyperproliferation of certain B cells that often produce cytokines. While not officially considered a cancer, the overgrowth of lymphocytes with this disease is similar to lymphoma. IL-6 secretion plays a role in some but not all cases. There is an association with HHV-8.
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What is the cell surface marker expression pattern in follicular lymphoma?
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CD10 and CD20 positive, BCL-2 and 6 positive (usually on these last two).
CD5 and CD23 are negative. |
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What is the most common translocation and gene product in follicular lymphoma?
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t(14;18) producing BCL-2
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What is the treatment strategy in stage I or non-bulky stage II follicular lymphoma?
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Involved or extended field radiation.
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What are some of the indications for initiating therapy in patients with follicular lymphoma? (7 possible)
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1. max LN dia. 7 cm
2. > 3 LN sites > 3 cm dia. 3. spleen > 16 cm 4. pleural effusion 5. compressive symptoms 6. circulating lymphoma cells 7. cytopenias |
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What percentage of patients with follicular lymphoma will transform to DLBCL?
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30-40% overall, 3% annual rate. Clinically, this usually occurs as the rapid growth of a single LN/region.
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When follicular lymphoma transforms to DLBCL, how is this treated?
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R-CHOP. Less than 10% are cured, so high-dose therapy followed by HSCT is advised.
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What is the role for HSCT in treatment of relapsed/refractory NHL?
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There is a significant PFS improvement and a marginal OS improvement when autologous HSCT is compared with conventional salvage chemotherapy.
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How does allogeneic HSCT compare to autologous HSCT in relapse/refractory NHL?
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Transplant-related death is about double for allogeneic. Autologous is associated with a higher risk of dying from recurrence, whereas allogeneic is associated with a higher risk of dying from GVHD, infection and vaso-occlusive disease.
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List the 3 types of marginal zone lymphomas.
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MALT
nodal marginal zone lymphoma primary splenic marginal zone lymphoma |
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What are the characteristic cell surface markers in MZL?
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CD20 and CD23 positive.
CD5 and CD10 negative. |
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What is the most common translocation in extranodal cases of MZL?
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t(11;18). This fuses API2 and MLT.
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Marginal zone lymphomas are commonly associated with what other type of disease entity?
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Autoimmune conditions such as Sjogren syndrome or Hashimoto Thyroiditis.
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MALT lymphomas are commonly associated with what infectious agent?
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H. pylori in stomach MALT.
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How is MALT lymphoma of the stomach treated?
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With triple therapy to eradicate H. pylori. This results in a durable response in 60% of cases.
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Which MALT tumors of the stomach are likely to be resistant to antibiotic therapy, and how are they treated instead?
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MALT with t(11;18); they can be treated with radiation, single agent Rituxan, or combination chemo plus Rituxan.
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What is the treatment and prognosis for nodal marginal zone lymphomas?
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Treated the same as follicular lymphoma, but respond relatively poorly.
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How does splenic marginal zone lymphoma typically present?
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Peripheral blood and bone marrow involvement and a massively enlarged spleen. Circulating lymphocytes with "villous" projections may be confused with hairy cell leukemia.
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How is splenic marginal zone lymphoma treated?
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Splenectomy can be very effective. Rituxan, fludarabine and combination chemo are also used but are not curative.
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Lymphoplasmacytic lymphomas (e.g. WM) tend to present with an IgM spike and possibly hyperviscosity. What are the four other significant complications?
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1. neuropathy
2. amyloidosis 3. cryoglobulinemia 4. cold agglutinins |
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What infectious disease has a strong association with lymphoplasmacytic lymphoma?
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Hepatitis C, especially for patients with mixed cryoglobulinemia.
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Lymphoplasmacytic lymphomas are associated (50%) with what translocation, and what is the associated gene product?
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t(9;14). The gene product is PAX-5.
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What agents are used in the treatment of lymphoplasmacytic lymphoma?
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Nucleoside analogs (cytarabine), alklyating agents (cyclophosphamide), Rituxan, lendalidomide, bortezomib
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Bill W - lucky - lots of options.
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What is the prognosis for patients with lymphoplasmacytic lymphoma?
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Median survival is 5-10 years, relapses are inevitable, most die from the disease. Prognosis is worse for patients who transform to large-cell NHL (24% of cases).
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What are the characteristic cell surface marker findings in DLBCL?
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CD19 and CD20 positive. CD5 and CD23 negative.
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How is limited stage DLBCL managed?
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CHOP-R for 3 cycles followed by radiation.
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How is advanced-stage DLBCL managed?
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CHOP-R.
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Why is a bone marrow biopsy necessary prior to initiating treatment for relapsed DLBCL?
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10-25% of patients with late recurrence will have an indolent (follicular) subtype.
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How is relapsed/refractory DLBCL managed?
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HSCT or high-dose combination chemo, or single agent Rituxan if performance score is poor.
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What is the characteristic cell surface marker pattern in mantle cell lymphoma?
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CD5 and CD20 positive (like CLL) but negative for CD23 (unlike CLL).
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What is the common translocation and gene product in mantle cell lymphoma?
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t(11;14) resulting in overexpression of cyclin D1
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Which organ system is commonly involved in mantle cell lymphoma at presentation?
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GI; may present with diffuse bowel involvement (lymphomatouspolyposis)
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What chemotherapy regimen is commonly used in treatment of mantle cell lymphoma?
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HyperCVAD.
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What is the role of HSCT in mantle cell lymphoma?
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Autologous HSCT after induction with HyperCVAD (or similar) can produce long-term DFS.
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What chemotherapy regimen is commonly used in relapsed mantle cell lymphoma?
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FCR + mitoxantrone with Rituximab maintenance.
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What is the approved use for bortezomib in mantle cell lymphoma?
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Relapsed or refractory.
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What are the cell surface/histologic findings in lymphoblastic lymphoma?
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CD7 positive, cytoplasmic CD3, Tdt, and TCR rearrangement positive.
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What is the distinction between T-ALL and lymphoblastic lymphoma?
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T-ALL has >25% lymphoblasts in the marrow, lymphoblastic lymphoma less than 25%.
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What are the high-risk factors in lymphoblastic lymphoma?
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-elevated LDH
-bone marrow involvement -CNS involvement |
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What is the standard treatment approach to lymphoblastic lymphoma?
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-HyperCVAD
-IT chemo and testicular radiation -mediastinal radiation is controversial. |
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What is the prognosis for patients with lymphoblastic lymphoma?
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If there are no high-risk factors (LDH, marrow or CNS involvement), the disease is cured in 80% of patients and relapses after 1 year are rare. High-risk patients relapse and the 5-year survival is 20%.
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What is the role of HSCT in lymphoblastic lymphoma?
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Autologous HSCT in second remission is better than other salvage options.
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What is the cell surface marker pattern and histologic appearance of Burkitt lymphoma?
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mature B-cell pattern: CD10, monoclonal surface IgM, CD19, CD20, CD22, BCL6, CD38, CD43.
The histologic appearance is of a "starry sky", from macrophages phagocytising apoptotic tumor cells. |
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What is the characteristic translocation in Burkitt lymphoma?
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t(8;14) resulting in the c-Myc oncogene. t(2;8) and t(8;22) also occur.
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An HIV-positive patient presents with an ileocecal mass. What hematologic malignancy would you suspect?
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Burkitt lymphoma.
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What chemotherapy regimens are used commonly in Burkitt lymphoma?
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R-HyperCVAD or CODOX-M/IVAC
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What is the prognosis for patients with Burkitt lymphoma?
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CR is 85-95%, long term DFS 50-80% for patients treated with high-dose therapy, consolidation, maintenance and CNS prophylaxis.
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What is a "double-hit" lymphoma?
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One that has both DLBCL and Burkitt features, with both c-Myc and Bcl-2 rearrangements. These are aggressive and are treated as Burkitt lymphoma.
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What is the general treatment approach to primary CNS lymphoma?
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High-dose MTX +/- WBRT. Radiation is deferred in older patients due to high incidence of leukoencephalopathy.
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How do patients with angioimmunoblastic PTCL usually present?
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B symptoms and autoimmune features including hypergammaglobulinemia and positive Coombs test.
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Which PTCL commonly presents in young men as splenomegaly and thrombocytopenia with a fatal hemophagocytic syndrome?
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Hepatosplenic T-cell lymphoma
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What is the characteristic cell surface marker pattern for anaplastic large cell lymphoma?
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CD2, CD4, CD5, CD30
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2 and 5 are there, just like the translocation t(2;5)
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What is the genetic aberration associated with nodal ALCL?
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t(2;5) leading to NPM-ALK. This is associated with a good prognosis.
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How is ALCL treated and what is the response rate?
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CHOP can cause remission in more than 70% of patients, with 50-80% surviving at 5 years.
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What is the prognosis for ALK-negative ALCL of the skin?
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Favorable prognosis even with conservative measures (excision, radiation).
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What are the treatment options for advanced CTCL?
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-extracorporeal photopheresis
-IFNa -denileukin diftitox -vorinostat (HDAC) -romidepsin (HDAC) |
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