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85 Cards in this Set
- Front
- Back
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Which cell surface markers are expressed on B-cells in early development as they begin heavy chain rearrangement?
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CD10 (aka CALLA), CD19, Tdt (terminal deoxynucleotidyl transferase).
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What are the changes in cell surface markers as B-cells begin light-chain rearrangement and expression of surface Ig?
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CD20 is expressed; CD10 and Tdt expression is lost
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Which two Ig molecules are expressed on B-cells when they are still antigen-naive?
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IgM and IgD.
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What are the two types of T-cell receptors (TCR)?
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gamma-delta
alpha-beta. All T-cells express one or the other but not both. |
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Which cell surface marker is considered definitive for T-cell identity?
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CD3, which is co-expressed with the TCR.
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Lack of CD5 on a cell that otherwise appears to be a lymphocyte suggests what?
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Likely an NK cell.
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What is significance of light-chain restriction in the workup of B-cell malignancies?
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B cells normally
express and light chains in a ratio of 2:1. A clonal expansion can be identifi ed by a marked predominance of either - or -expressing B cells. This would not be expected in a reactive process. |
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What are the 3 pan-B cell cell surface markers?
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CD19, CD20, CD22
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How is follicular lymphoma differentiated from MCL on the basis of cell surface markers?
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Follicular lymphoma is negative for CD5 and CD10; MCL is positive for CD5 and CD23.
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What are the distinguishing factors between primary cutaneous follicle center lymphoma and primary cutaneous DLBCL, leg type?
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PCFCL typically presents with solitary lesions on the head and trunk; tumor is composed of neoplastic follicle center cells including centrocytes and a variable number of centroblasts.
Primary cutaneous DLBCL is comprised of a diffuse monotonous pattern of centroblasts or immunoblasts, irrespective of site, and is considered aggressive |
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In the updated WHO classification, subcutaneous panniculitis-like T-cell lymphoma (SCPTCL) is confined to cases with what phenotype?
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Alpha-beta. The gamma-delta phenotype is listed as a subtype of SCPTCL along with primary cutaneous SCPTCL, due to the aggressive nature of these malignancies.
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Name 3 autoimmune conditions which carry an increased risk of NHL.
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RA
Hashimoto Thyroiditis Sjogren Syndrome |
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MALT lymphoma is associated with H/pylori infection. Name 3 other microbe-lymphoma associations.
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Chlamydia psittaci and orbital adnexa MALT.
C. Jejuni and immunoproliferative small intestinal disease. Borrelia burgdorferia and cutaneous MALT |
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What infectious agent is associated with primary body cavity lymphoma in HIV-positive patients?
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HHV-8
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Hepatitis C is associated with an increased risk of which lymphoma?
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lymphoplasmacytic.
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The BCL 6 gene is found most commonly in which lymphoma?
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DLBCL. BCL 6 is a good prognostic factor.
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Describe Ann Arbor stage IIIE.
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Involvement of LN regions on both sides of the diaphragm, which may also be accompanied by localized involvement of an extranodal site. IIIS would refer to the same but with the spleen involved.
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What are the 5 factors in the IPI for lymphoma?
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LDH
age > 60 PS > or equal to 2 Stage III or IV Acronym is APLES. > 1 extranodal sites |
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Describe the 5 year survival based on IPI scores for low risk and high risk.
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Low risk (0-1 factors) have a 75% chance of being alive at 5 years.
High risk (4-5 factors) have a 75% chance of being dead at 5 years. This data is based on the pre-Rituximab era. |
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What factors go into determination of the Follicular Lymphoma International Prognostic Index (FLIPI)?
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Number of extranoal sites.
LDH AGE STAGE Hgb < 10 Acronym is No-LASH. |
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Patients with which two lymphomas are recommended to have pretreatment and response assessment PET scans?
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DLBCL and HL
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How are follicular lymphomas graded?
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grade 1 (0-5); grade 2 (6-15); and grade 3 ( >15).
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What is the cell surface marker pattern in follicular lymphoma?
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CD20+, CD10+, BCL6+, BCL2+,
and CD5- |
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What is the difference between follicular lymphoma grades 3A and 3B?
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grade 3A (centrocytes present) is distinguished from grade
3B (solid sheets of centroblasts), |
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What genetic abnormalities/gene products are commonly seen in grade 3B follicular lymhoma?
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3q27 and/or BCL6 rearrangement
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Is there a proven benefit to early treatment of asymptomatic patients with follicular lymphoma?
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In the few randomized controlled
studies that are available, it has not been shown that early treatment of asymptomatic advanced-stage patients improves survival, so a common approach in such patients who also have nonthreatening, typically nonbulky disease is watchful waiting and treatment when symptoms or disease progression become apparent. |
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In an elderly patient with poor performance status and advanced stage indolent lymphoma, what single agent therapies are available?
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Oral chlorambucil, cyclophosphamide, Rituxan
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In patients with relapsed FL who are candidates for HSCT, use of what agent should be limited to avoid stem cell toxicity?
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Fludarabine.
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What restrictions are placed on the use of radioimmunotherapy in the treatment of relapsed indolent lymphoma?
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Use is limited to patients with
adequate leukocyte and platelet counts and < 25% marrow involvement by lymphoma |
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What is the MOA of bendamustine?
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It is a bifunctional agent that is an alkylator with purine analog characteristics.
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What is the characteristic cell surface marker pattern of marginal zone lymphoma?
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positive for CD20 but lack of CD5 or CD10
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MALT lympomas can occur in what tissues other than stomach and intestine?
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lung, salivary gland, periorbital or soft tissue, skin, and thyroid. Often these sites are affected by chronic infection or infl ammation,
such as Sjögren syndrome involving the parotid gland or Hashimoto thyroiditis |
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What is the translocation most commonly associated with MALT lymphoma?
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t(11;18)(q21;q21)
translocation in approximately 40% of cases and, less commonly, the t(1;14)(p22;q32) translocation. |
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How are MALT lymphomas treated when they are resistant to H. pylori eradication or are advanced?
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IFRT has been highly effective in
localized MALT lymphomas; Similar regimens as those used in follicular lymphoma, including rituximab-based combinations, can be used in patients with advanced-stage disease |
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What is the translocation associated with Nodal MZL?
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The t(11;18) and t(1;14) karyotypic
changes identifi ed in MALT are absent in nodal MZL, and no specific or recurring karyotypic anomaly has been described. |
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Why is endoscopic evaluation indicated in the workup of patients with Nodal MZL?
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A coexisting extranodal
MALT lymphoma component may be identified in up to one third of cases. |
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What is the relationship between indolent lymphoma (esp. splenic MZL) and Hepatitis C?
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Treating Hep C with pegylated interferon and ribavirin may lead to regression of the lymphoma
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What is the mechanism behind coagulopathy associated with Waldenstrom Macroglobulinemia?
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IgM binding to clotting factors,
platelets, and fibrin |
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What two factors are used in risk-stratifying patients with lymphoplasmacytic lymphoma?
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Age >65 and anemia.
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Why is vincristine omitted from chemotherapy in treatment of WM?
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Due to the disease-related neuropathy.
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What two nucleoside analogs have shown response rates of 75% in non-randomized studies in patients with WM?
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fludarabine and cladribine
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What is the danger of treating WM patients with Rituxan?
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Abrupt increases in IgM can occur following therapy, leading to hyperviscosity, especially if the pretreatment IgM level is >5,000
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WM is generally treated similarly to follicular/indolent lymphoma or CLL. What agents, other than those typically used in those malignancies, are also effective in WM?
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Lenalidomide and Bortezomib
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Why is NOT misdiagnosing hairy cell leukemia as myelofibrosis, aplastic anemia or MDS so important?
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HCL is very treatable, having a unique sensitivity to purine analogs (pentostatin or cladribine)
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Which hematologic malignancy has a mononuclear cell infiltrate with a "fried egg" appearance of a halo around the nuclei and increased reticulin and collagen fibrosis?
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Hairy cell leukemia
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What is the role or Rituxan in the treatment of HCL?
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HCL has high-density CD20 expression and is has shown up to 80% ORR as a single agent in relapsed patients.
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Why is NOT misdiagnosing hairy cell leukemia as myelofibrosis, aplastic anemia or MDS so important?
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HCL is very treatable, having a unique sensitivity to purine analogs (pentostatin or cladribine)
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Which hematologic malignancy has a mononuclear cell infiltrate with a "fried egg" appearance of a halo around the nuclei and increased reticulin and collagen fibrosis?
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Hairy cell leukemia
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What is the role or Rituxan in the treatment of HCL?
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HCL has high-density CD20 expression and is has shown up to 80% ORR as a single agent in relapsed patients.
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What is the danger of treating WM patients with Rituxan?
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Abrupt increases in IgM can occur following therapy, leading to hyperviscosity, especially if the pretreatment IgM level is >5,000
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WM is generally treated similarly to follicular/indolent lymphoma or CLL. What agents, other than those typically used in those malignancies, are also effective in WM?
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Lenalidomide and Bortezomib
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What are the differences in chromosomal aberrations between the ABC and GCB subypes of DLBCL, and what is the significance in terms of prognosis?
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Gains of 3p,
18q21-22, and losses of 6q21-22 are seen in the ABC subtype, and gains in 12q12 are observed frequently in the GCB subtype. GCB carries a better prognosis. |
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What is the standard treatment of localized DLBCL?
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3 cycles of R-CHOP
followed by IFRT is considered the standard therapy for localized DLBCL. |
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How is localized DLBCL treated?
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For most patients with non-bulky limited stage DLBCL, three cycles of R-CHOP (table 3) followed by 30 to 36 Gy of involved-field radiation. In cases where radiation may cause significant morbidity (eg, involvement of the oronasopharynx or pelvis), the dose of chemotherapy is maximized to allow delivery of a more tolerable radiation dose. For patients with bulky (>10 cm) stage I or II tumors, six cycles of R-CHOP followed by involved-field radiation rather than chemotherapy alone. For patients with DLBCL with testicular involvement, initial therapy consists of orchiectomy on the side of disease, low dose radiation therapy to the involved field and contralateral testis, systemic chemotherapy, and intrathecal chemotherapy. For patients with limited stage disease primary mediastinal large B cell lymphoma, six cycles of R-CHOP followed by involved-field radiation rather than the use of more intensive chemotherapy regimens. Patients with bulky primary mediastinal disease, pericardial effusion or pleural effusions are treated as advanced disease with eight cycles of R-CHOP.
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How is relapsed DLBCL treated?
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Patients with DLBCL that has relapsed after initial response
should receive a second-line chemotherapy regimen such as ESHAP, ICE, DHAP, GDP, or EPOCH |
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What is the typical presentation of a patient with primary mediastinal large B-cell lymphoma?
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Female, age 35, bulky anterior mediastinal mass with possible local invasion or SVC syndrome. Distant spread or BM involvement is uncommon.
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What is unusual about PMBCL at relapse?
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May occur at sites including the kidneys, adrenals, ovaries, liver, spleen and CNS.
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Which cell surface marker is helpful, if negative, in sorting Burkitt lymphoma from acute lymphoblastic leukemia/lymphoma?
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Tdt.
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What are the two most common chemotherapy regimens used for high-risk Burkitt lymphoma patients?
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CODOX-M/IVAC and HyperCVAD/methotrexate
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What two alterations in gammaglobulins can occur in MCL?
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-hypogammaglobulinemia
-monoclonal IgM gammopathy |
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What is the standard approach to chemotherapy in MCL?
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It varies widely, but older patients can be treated with R-CHOP and younger, healthier patients with more aggressive regimens like R-hyperCVAD alternating with cytarabine and MTX, followed by auto-HSCT.
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What chemotherapy options are available for relapsed/refractory MCL?
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-BR
-FCM-R -Bortezomib -Lenalidomide |
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What is the characteristic histologic finding in mycosis fungoides?
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Intraderma collections of Pautrier microabscesses.
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Sezary syndrome is characterized by erythroderma, generalized lymphadenopathy and the presence of Sezary cells in the skin, lymph nodes and peripheral blood. Additionally, what cell count criteria (at least one) must be met?
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Sezary cell count >1,000/uL, CD4/CD8 > 10, loss of one or more of the following T-cell surface markers: CD2, CD3, CD4 or CD5
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How is the cutaneous phase of MF managed?
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topical nitrogen mustard, electron-beam radiotherapy,
or cutaneous photochemotherapy with oral psoralen plus ultraviolet A (PUVA). |
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How is advanced-stage MF/Sézary syndrome managed?
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Interferon alfa, bexarotene, vorinostat, and
denileukin diftitox all have effi cacy in advanced-stage MF and Sézary syndrome |
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How does T-cell large granular lymphocytic leukemia present in terms of physical findings and cell counts?
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Typically with moderate splenomegaly, no lymphadenopathy, severe neutropenia with or without anemia. Thrombocytopenia is rare.
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How is T-cell large granular lymphocytic leukemia managed?
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cyclosporine A, chlorambucil, cyclophosphamide,
or corticosteroids can be effective. Splenectomy may also be useful in cases with an accompanying splenomegaly and or cytopenias |
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What are the pathologic and flow findings in adult T-cell leukemia/lymphoma?
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The malignant cells have a distinct
cloverleaf appearance and lack CD7, and the majority are CD4+/CD8- |
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What is the typical clinical presentation of Aggressive NK-cell leukemia?
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Bone marrow and peripheral blood involvement as well as liver and spleen; fever and constitutional symptoms; multiorgan failure; coagulopathy and hemophagocytic syndrome
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What is the standard treatment for PTCL-NOS?
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CHOP, even though it is not very effective
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What are the histologic, flow, and gene expression findings in angioimmunoblastic T-cell lymphoma?
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Key morphologic findings of AITL include an expanded
CD21+ follicular dendritic cell network and prominent arborizing high endothelial venule. The tumor cells are CD10, BCL6, and CXCL13 positive. |
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What is the characteristic chromosomal translocation in ALCL? What cell surface maker is strongly positive?
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t(2;5) resulting in the fusion gene NPM-ALK. CD30 is strongly positive.
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In addition to CD30, ALK-positive ALCL has which other histologic findings?
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ALK-positive ALCL is usually
positive for epithelial membrane antigen (EMA) and cytotoxic markers (TIA1, granzyme B, and perforin). |
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What is the prognostic significance of ALK positivity in ALCL?
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Predicts a good response to anthracycline therapy.
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What is the treatment approach to localized extranodal NK/T-cell lymphoma, nasal type?
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Radiotherapy is the most mportant treatment modality for localized disease.
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What is the significance of the gamma-delta subtype of subcutaneous panniculitis-like T-cell lymphoma?
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The gamma-delta phenotype has a far inferior prognosis (5-year OS 11% vs 82%) compared to the alpha-beta phenotype.
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What treatment approaches are used in subcutaneous panniculitis-like T-cell lymphoma?
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CHOP and immunosuppressive agents.
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Describe enteropathy-associated T-cell lymphoma in terms of demographic and disease association characteristics.
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Enteropathy-associated T-cell lymphoma is a rare, aggressive
intestinal tumor with a male predominance and occurs in the setting of celiac disease. It most commonly involves the jejunum or ileum. |
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How does CD4 count affect which chemotherapy is given to HIV+ patients with aggressive lymphomas?
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R-CHOP can be
considered in HIV patients if the CD4 count is 50; however, it should be given in conjunction with G-CSF given the high rate of infection in this population. |
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How does the timing of onsent related to EBV positivity in post-transplant lymphoproliferative disorders?
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PTLDs may arise within the fi rst 6 months of transplantation
or have a later onset beyond 1 to 2 years; the former is more commonly associated with EBV |
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What treatment approach is reasonable as a first step in management of PTLD?
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A minority of patients
will respond to a reduction in intensity of immunosuppression, particularly in polymorphic PTCL, but most require additional systemic therapy with Rituxan, chemotherapy, antivirals or anti-B-cell therapy |
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What type of lymphoma comprises most cases of primary CNS lymphoma?
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Most cases of PCNSL are DLBCLs, but rare
cases of PTCL, low-grade lymphoma, and Burkitt lymphoma have also been reported |
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Which cell surface markers are useful in distinguishing lymphocyte-depleted CHL from ALK-ALCL?
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CD30 and Pax5 are posititve in LD CHL.
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In terms of long-term sequelae, how do MOPP or BEACOPP compare?
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MOPP is associated with sterility, an increased risk
of MDS, and secondary AML. ABVD has not been associated with sterility or MDS/leukemia but does have the risks of pulmonary and cardiac toxicity from bleomycin and doxorubicin, respectively. |
