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42 Cards in this Set
- Front
- Back
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what proportion of NHL are MCL?
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6%
mnemonic M=13, C=3, L=12 --> 28 --> check difference is 6 |
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what are the key points for mantle cell lymphoma (MCL)?
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older adults; intermediate-grade uncurable refractory lymphoma with disseminated disease at presentation, with generalized lymphadenopathy, common splenomegaly and often early leukemic phase
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what is the M:F ratio in MCL?
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M:F = 4:1
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what is the median age in MCL?
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60 years
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under what name is mantle cell lymphoma (MCL) also known?
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intermediate lymphocytic lymphoma
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what are the frequencies of lymphadenopathy and splenomegaly in MCL?
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generalized lymphadenopathy in 90%
splenomegaly in 60% hepatomegaly in 30% |
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what is the cytogenetic abnormality associated with MCL?
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t(11;14)
mnemonic 11 plus median survival (3) equals 14 |
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what is the prognosis in MCL?
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not curable with conventional chemotherapy, with a median survival of 3 years
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what are typical immunophenotypic findings in MCL?
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B-cell markers: CD5+, CD23- and CD10- (cALLa)
overexpression of cyclin D1 |
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what are the therapy principles in MCL?
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CHOP or CVP +/- rituximab
or hyper-CVAD comprises hyperfractionated cyclophosphamid, vincristin, doxorubicin and dexamethasone alternating with high-dose methotrexat and cytarabin |
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what percentage of NHL are diffuse large B-cell lymphoma?
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30%, thus DLBCL most common of the NHL
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what are the therapy principles for DLBCL?
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R-CHOP
salvage regimens with DHAP (dexamethasone, cisplatin and cytarabine), ESHAP (etoposide, methylprednisolone, cisplatin and cytarabine), or M-BACOD (Methotrexat, bleomycin, Adriamycin=doxorubicin, cyclophosphamid, oncovin and dexamethasone) HA = high dose ara-C P = Platinol, Cisplatin mnemonic: the name's too long, CHOP it off! |
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what are risk factors for DLBCL?
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Sjoegren syndrome
solid organ or bone marrow transplant recipient HIV infection |
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what are common extranodal sites of DLBCL?
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gastrointestinal tract
bone testis central nervous system |
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what other lymphomas are threated like DLBCL?
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primary mediastinal large B-cell-lymphoma (PMBL)
primary effusion lymphoma (PEL) = body-cavity-based B-cell lymphoma (BCBL) mnemonic: too long as well --> CHOP it off! |
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what is the median age in primary mediastinal (thymic) large B-cell lymphoma (PMBL)?
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30 years
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what is the course in PMBL?
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aggressive
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what is the M:F ratio in PMBL?
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slightly more common in women, in contrast to most other types of lymphoma
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what are the symptoms in PMBL?
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superior vena cava syndrome is common as is dyspnoea due to compression of the airways and/or pleural effusion
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what are the therapy principles in PMBL?
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treatment is similar to those for DLBCL with R-CHOP (cyclophosphamide, hydroxydaunorubicin, vincristin, prednisone) or MACOP-B (methotrexate, adriamycin=doxorubicin, cyclophosphamide, oncovin, prednisone and bleomycin)
involved-field radiation is commonly administered |
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what are subtypes of DLBCL?
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ABC, PMBL and GCB (germinal-center B-cell-like), all histologically indistinguishable
special subtypes associated with oncogenic herpesviruses are the immunodeficiency-associated large B-cell lymphoma and the primaly effusion lymphoma |
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what is a common extranodal site involved in MCL?
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gastrointestinal tract, also known as lymphomatoid polyposis
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what oncogene is characteristically expressed in Burkitt lymphoma?
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c-myc, but not in Burkitt-like lymphomas
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which subtypes of Burkitt lymphoma exist?
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endemic african, nearly all associated with EBV-infection
sporadic, 20% associated with EBV-infection Aids-associated, 30-40% associated with EBV-infection |
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what are characteristic genetic abnormalities in Burkitt lymphoma?
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reciprocal translocation of the c-MYC gene on chromosome 8, the translocation partner usually is the IgH locus, or less commonly the Ig kappa or Ig lambda ligh-chain loci
most with t(8;14), much less common with t(8;22) or t(2;8) |
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what are the therapy principles in Burkitt lymphoma?
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high-dose combination chemotherapy with CNS prophylaxis
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what is the median age in Burkitt lymphoma and Burkitt-like lymphoma?
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30 years in Burkitt, 55 years in Burkitt-like lymphoma
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which NHL is most common in children?
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Burkitt lymphoma
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which is the most common site involved in Burkitt's lymphoma?
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gastrointestinal tract
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which other aggressive NHL is similar morphologically to Burkitt's lymphoma?
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DLBCL
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what are the characteristics of endemic Burkitt lymphoma?
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presents as jaw tumor with eventual spread to extranodal sites, particularly the bone marrow and leptomeninges
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what are the standard therapy principles for Burkitt lymphoma in children?
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either ALL-like approach or shorter duration, dose-intensive combination chemotherapy with CNS prophylaxis
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what is the association between Burkitt lymphoma and immunosuppression?
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only associated with HIV infection, but not other forms of immunosuppression
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what are the key points in Burkitt's lymphoma?
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high-grade NHL with three clinical types
peripheral lymphadenopathy is rare and while bone marrow involvement is common in late stages leukemic phases are very rare |
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what are the key points for DLBCL?
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typically presents as rapidly enlarging mass at a nodal or extranodal site, bone marrow involvement is uncommon and leukemic phase is rare
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what are the key points for Burkitt lymphoma?
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truly high-grade lymphoma most often with manifestation at extranodal sites, involvement of the bone marrow and leukemic phases are uncommon
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in what age group is Burkitt's lymphoma mainly found?
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children and young adolescents
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what is the prognosis in Burkitt's lymphoma?
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responds well to intensive chemotherapy, most children and young adults can be cured, while older adults have a much more guarded outcome
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what are subtypes of DLBCL associated with oncogenic herpesviruses?
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immunodeficiency-associated large B-cell lymphoma in the setting of severe T-cell immunodeficiency (HIV, allogeneic bone marrow transplantation), with infection of the neoplastic cells with EBV
Primary Effusion Lymphoma (PEL) with malignant pleural or ascitic effusion with infection of the tumor cells with KSHV/HHV-8 |
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what is the cell of origin in DLBCL?
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germinal-center or post-germinal-center B cell
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what is the cell of origin in mantle cell lymphoma?
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naive B cell
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what is the fusion gene created in MCL?
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t(11;14) with cyclinD1-IgH fusion gene
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