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MALIGNANT HEME > SET10 > Flashcards

SET10 Flashcards

1
Q

Can bortezomib be used in Mantle Cell?

A

Yes, if they have failed at least one prior therapy

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2
Q

What is a double hit lymphoma?

A

mutations in bcl2 (confers anti-apoptotic features) and myc (confers proliferation); can also have bcl2 (bcl = B Cell Lymphoma)

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3
Q

What is the pathophysiology of follicular lymphoma?

A

increased transcription of bcl2 leads to inhibition of apoptosis

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4
Q

What is a good maintenance regimen for follicular lymphoma in CR?

A

Maintenance rituximab (Rituximab q2 month) x 2 years (PRIMA study)

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5
Q

What is lymphomatous polyposis?

A

When a polyp biopsy reveals lymphoma

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6
Q

An age greater than what is a poor risk score for both the FLIPI (follicular) and the DLBCL IPI?

A

Age >60

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7
Q

What is considered the standard of care for patients with grade I-II follicular lymphoma?

A

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

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8
Q

What lymphoma often has pancytopenia but has a lymphocytosis and has splenomegaly?

A

Splenic Marginal Zone Lymphoma

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9
Q

What is the difference in tx for high stage follicular lymphoma at time of diagnosis vs. stage I-II

A

Not so paradoxically, you observe at high stage (III-IV) but can actually treat with ISRT for low stage I-II

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10
Q

What are the roles of bcl2 and myc in “Double Hit Lymphomas”?

A

bcl2 confers anti-apoptotic features; myc confers marked proliferation

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11
Q

Name some options that are available for chemotherapy when follicular lymphoma DOES require tx

A

R-CHOP, R-CVP, R-Bendamustine, and even Rituximab alone (RESORT trial)

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12
Q

About 5% of pt w/ DLBCL will experience CNS relapse, what are 4 risk factors for CNS relapse?

A

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)

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13
Q

What translocation often implies lack of benefit to H. pylori therapy for MALT lymphoma?

A

t(11;18)

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14
Q

How do you decide whether to do BR or R-CHOP for mantle cell lymphoma?

A

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

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15
Q

When is follicular lymphoma pathologically defined as grade IIIb?

A

If there are >15 centroblasts per hpf (or solid sheets of centroblasts)

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16
Q

What is the optimal dosing of ibrutinib in mantle cell lymphoma?

A

500 mg daily

17
Q

What is often implicated in MALT lymphoma?

A

H. pylori (even if Bx neg, should still do a noninvasive test such as urea breath or stool ag)

18
Q

What is the FLIPI for Follicular Lymphoma?

A

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

19
Q

Which type of follicular lymphoma most commonly occurs in concert with DLBCL?

A

Grade IIIb follicular lymphomas; less likely to be CD10+, less likely to be BCL2+, and often lack t(14;18)

20
Q

What is a form of radioimmunotherapy that can be given to follicular lymphoma w/ advanced stage who are in first CR?

A

Ibritumomab tiuxeta (Zevalin); this is a monoclonal IgG1 ab (Ibritumomab) to which a chelator (tiuxetan) is added to yttrium-90

21
Q

What do you do for a patient with follicular lymphoma that is rapidly enlarging?

A

You should repeat a biopsy to check for transformation to DLBCL

22
Q

When should you consider transformation of a follicular lymphoma?

A

if the LDH acutely rises, new B symptoms develop, or if one site begins growing OOP to the others

23
Q

What low grade lymphomas often have HCV ab positivity and light chain restriction?

A

Splenic Marginal Zone Lymphoma

24
Q

What is a common way that splenic marginal zone lymphoma presents?

A

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

25
Q

What is the IPI for DLBCL?

A

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

26
Q

What has the OS benefit been for autologous transplant (HDT ASCR) in patients with follicular lymphoma in first remission?

A

No survival benefit

27
Q

What is the most aggressive form of mantle cell lymphoma?

A

Blastoid variant

28
Q

What is the usual immunophenotype of splenic marginal zone lymphoma?

A

CD5-, CD20+, CD22+ (negative for CD25 and CD103 which are usually positive in Hairy Cell Leukemia)

29
Q

How are grade IIIb follicular lymphomas often different from other lymphomas?

A

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

30
Q

When should you consider treatment for splenic marginal zone lymphoma?

A

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

31
Q

What is the classic translocation in follicular lymphoma and what happens?

A

t(14;18), juxtaposes bcl-2 next to the IgH locus

32
Q

What is the maintenance treatment of Mantle Cell Lymphoma?

A

Rituximab (usually); may especially confer benefit if Bendamustine/Rituximab is used as induction therapy

33
Q

What is ibritumomab tiuxeta?

A

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

34
Q

What cells are key when discussing the pathologic grade of a follicular lymphoma?

A

centroblasts

MALIGNANT HEME (12 decks)

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