SET10 Flashcards
Can bortezomib be used in Mantle Cell?
Yes, if they have failed at least one prior therapy
What is a double hit lymphoma?
mutations in bcl2 (confers anti-apoptotic features) and myc (confers proliferation); can also have bcl2 (bcl = B Cell Lymphoma)
What is the pathophysiology of follicular lymphoma?
increased transcription of bcl2 leads to inhibition of apoptosis
What is a good maintenance regimen for follicular lymphoma in CR?
Maintenance rituximab (Rituximab q2 month) x 2 years (PRIMA study)
What is lymphomatous polyposis?
When a polyp biopsy reveals lymphoma
An age greater than what is a poor risk score for both the FLIPI (follicular) and the DLBCL IPI?
Age >60
What is considered the standard of care for patients with grade I-II follicular lymphoma?
Observation (despite it being indolent, tends to recur with time so you want to be judicious w/ applying early tx); should be asymptomatic i.e. without constitutional sx, cytopenias, low bulk
What lymphoma often has pancytopenia but has a lymphocytosis and has splenomegaly?
Splenic Marginal Zone Lymphoma
What is the difference in tx for high stage follicular lymphoma at time of diagnosis vs. stage I-II
Not so paradoxically, you observe at high stage (III-IV) but can actually treat with ISRT for low stage I-II
What are the roles of bcl2 and myc in “Double Hit Lymphomas”?
bcl2 confers anti-apoptotic features; myc confers marked proliferation
Name some options that are available for chemotherapy when follicular lymphoma DOES require tx
R-CHOP, R-CVP, R-Bendamustine, and even Rituximab alone (RESORT trial)
About 5% of pt w/ DLBCL will experience CNS relapse, what are 4 risk factors for CNS relapse?
Paranasal sinus location, testes location, HIV lymphoma, and epidural involvement (Can then ppx w/ HD MTX 3g/m2; or intrathecal MTX or intrathecal Ara-C)
What translocation often implies lack of benefit to H. pylori therapy for MALT lymphoma?
t(11;18)
How do you decide whether to do BR or R-CHOP for mantle cell lymphoma?
BR can be better for indolent mantle cell forms whereas more aggressive ones should get R-CHOP; also BR better tolerated and does not contain anthracycline so that is good if poor heart fxn
When is follicular lymphoma pathologically defined as grade IIIb?
If there are >15 centroblasts per hpf (or solid sheets of centroblasts)
What is the optimal dosing of ibrutinib in mantle cell lymphoma?
500 mg daily
What is often implicated in MALT lymphoma?
H. pylori (even if Bx neg, should still do a noninvasive test such as urea breath or stool ag)
What is the FLIPI for Follicular Lymphoma?
Low risk is 0-1 features, intermediate is 2, poor risk is 3 or more; Age >60, Stage III or IV, Hgb <12, LDH > upper limit of normal, >5 or more lymph node sites
Which type of follicular lymphoma most commonly occurs in concert with DLBCL?
Grade IIIb follicular lymphomas; less likely to be CD10+, less likely to be BCL2+, and often lack t(14;18)
What is a form of radioimmunotherapy that can be given to follicular lymphoma w/ advanced stage who are in first CR?
Ibritumomab tiuxeta (Zevalin); this is a monoclonal IgG1 ab (Ibritumomab) to which a chelator (tiuxetan) is added to yttrium-90
What do you do for a patient with follicular lymphoma that is rapidly enlarging?
You should repeat a biopsy to check for transformation to DLBCL
When should you consider transformation of a follicular lymphoma?
if the LDH acutely rises, new B symptoms develop, or if one site begins growing OOP to the others
What low grade lymphomas often have HCV ab positivity and light chain restriction?
Splenic Marginal Zone Lymphoma
What is a common way that splenic marginal zone lymphoma presents?
It is a low grade lymphoma often associated with HCV ab +; it leads to splenomegaly, pancytopenia but WITH A LYMPHOCYTOSIS and bone marrow involvement; usually do NOT see LAD
What is the IPI for DLBCL?
Low risk is 0-1 features, intermediate is 2, poor is 3 or more; Age >60, performance status >2, >1 extranodal site, stage III/IV dz, and elevated LDH
What has the OS benefit been for autologous transplant (HDT ASCR) in patients with follicular lymphoma in first remission?
No survival benefit
What is the most aggressive form of mantle cell lymphoma?
Blastoid variant
What is the usual immunophenotype of splenic marginal zone lymphoma?
CD5-, CD20+, CD22+ (negative for CD25 and CD103 which are usually positive in Hairy Cell Leukemia)
How are grade IIIb follicular lymphomas often different from other lymphomas?
They are usually CD10 neg, less likely to be BCL2 +, and often lack t(14;18); also they often OCCUR IN CONCERT WITH DLBCL and are tx w/ R-CHOP
When should you consider treatment for splenic marginal zone lymphoma?
If constitutional sx, early satiety (d/t splenomegaly), or if cytopenias develop would then consider splenectomy, Rituximab or Rituximab and chemo; also since often associated with HCV, if Tx HCV then lymphoma may regress
What is the classic translocation in follicular lymphoma and what happens?
t(14;18), juxtaposes bcl-2 next to the IgH locus
What is the maintenance treatment of Mantle Cell Lymphoma?
Rituximab (usually); may especially confer benefit if Bendamustine/Rituximab is used as induction therapy
What is ibritumomab tiuxeta?
It is a monoclonal ab adhered to a chelator to which yttrium-90 is attached; used as a radioimmunotherapy in pt w/ high grade follicular lymphoma in first CR
What cells are key when discussing the pathologic grade of a follicular lymphoma?
centroblasts
