Non Hodgkin Lymphoma Flashcards

1
Q

HTLV-1 virus is associated with what type of cancer?

A

Adult T cell lymphoma/leukemia

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2
Q

What measurement is considered to be bulky disease on imaging?

A

7.5cm

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3
Q

What are the flow cytometry results for DLBCL? If positive for CD5 what should it be differentiated from?

A

CD 19, CD20, CD10, BCL6, and IRF4/MUM1. If positive for CD5 must differentiate from blastoid variant of MCL which is cyclin D1+

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4
Q

What are the determinants of the IPI score in DLBCL?

A

Age>60, elevated LDH, ECOG of 2 or more, Stage III/IV dx, more than 1 extranodal site.

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5
Q

What are the treatment options for Stage I non-bulky DLBCL?

A

You can give 3 cycles of R-CHOP, do a PET next, and then if it is Deauville 1-3 you can give 1 more cycle (4 cycles total) or give ISRT.

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6
Q

What are the two concerns for testicular lymphoma?

A

They have a high rate of contralateral relapse and they have a high rate of CNS metastasis.

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7
Q

Which one does better: Germinal center vs ABC type?

A

Germinal center does better.

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8
Q

What is the recommendation for Stage I Bulky DLBCL?

A

You give R-CHOP for 6 cycles + ISRT

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9
Q

What is the Deauville score for a complete response in DLBCL?

A

1-3

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10
Q

If a patient with non-bulky Stage I disease did not have a complete response to treatment what is the best next step?

A

If they had a Deauville score of 4 they can receive 1-3 additional cycles of RCHOP +/- ISRT or ISRT alone.

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11
Q

What are the options for treatment for stage II disease with extensive mesenteric involvement and stage III/IV disease as initial tx?

A

Besides R-CHOP you can use POLA-RCP (IPI of 2 or more) and DA-EPOCH

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12
Q

In DLBCL what are the indications for CNS prophylaxis?

A

Testicular lymphoma, renal/adrenal gland involvement, high grade B cell lymphoma NOS with MYC rearrangement, primary cutaneous DLBCL leg type, Stage IE DLBCL of the breast.

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13
Q

What is the mechanism of action for Polatuzumab?

A

It’s a drug/antibody conjugate. It binds to CD-79 and then releases MMAE which inhibits mitosis (by binding microtubules).

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14
Q

What chemo options are available for an elderly patients with a low PS for DLBCL?

A

R-mini CHOP, R-CDOP, RGCVP, RCEPP

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15
Q

What are some risk factors for CNS prophylaxis (think of the criteria in the score calculation)?

A

Age>60, elevated LDH, PS>1, Stage III/IV disease, more than 1 extranodal involvement

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16
Q

What are the options for CNS prophylaxis?

A

High dose MTX given during or after chemo or IT MTX with or w/o cytarabine.

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17
Q

What are the flow markers for Primary Mediastinal B Cell Lymphoma?

A

C19, CD20, CD30 (seen in 80% of patients), lack Igs on cell surface. Also + for TRAF and c-REL. Neg for CD5 and CD10.

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18
Q

What is the primary treatment option for Primary Mediastinal B Cell Lymphoma?

A

DA-EOPCH-R. You can consider R-CHOP w/RT but it has a higher relapse rate.

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19
Q

What is the tx approach for relapsed/refractory primary mediastinal B cell Lymphoma? What if they don’t respond to that initial treatment?

A

Chemoimmunotherapy followed by auto-SCT if responsive to chemo (if greater than 12 months). If less than 12 months can consider CAR-T. If they don’t respond to chemo you give CAR-T or Pembro. It says also you can consider Nivolumab +/- Brentuximab

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20
Q

What is the typical patient age wise and sex wise that presents with Primary Mediastinal B-Cell Lymphoma?

A

Median age is 35 and it has a female predominance.

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21
Q

What are the second line options for those that will proceed to transplant for DLBCL?

A

DHA, GDP, ICE all with Rituximab. DHA-(dexamethasone, cytarabine, platinum)

Other options: ESHAP w/retux, GemOx +Ritux, MNE

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22
Q

With Axi Cel and Liso Maraleucel in the second line setting what was the benefit of this drug vs chemoimmunotherapy and transplant?

A

There is a EFS and OS benefit!

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22
Q

For those that experience disease relapse with DLBCL within one year what is the tx and why?

A

These patients are treated with CAR-T (Axi Cel and Liso Maraleucel). These patients have very poor prognosis compared to those who relapse after 1 year.

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23
Q

Selinexor is used in what setting and what is the mechanism of action?

A

It is used in the third line setting. It is a XPO1 inhibitor (nuclear export protein)

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24
Q

Tafasitamab plus lenalidomide is used in what setting? And what is the mechanism of action?

A

Tafasitimab is used in the second line setting for DLBCL for those who are not eligible for auto SCT. Tafasitimab is a CD-19 monoclonal antibody.

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25
Q

Loncastuximab is used in what setting of DLBCL and what is the mechanism of action?

A

It is used in the third line setting. It is a antibody drug conjugate, it binds to CD-19 and releases SG3199 which crosslinks DNA.

26
Q

What is the flow phenotype for Burkitts Lymphoma?

A

sIg+, cIg+, Cd20+, CD10+, TdT neg, Ki 67>95%, BCL6+, BCL2-

27
Q

What is the underlying translocation in Burkitts that fuels uncontrolled cell growth?

A

c-MYC translocation with Ig heavy or variant light chain gene so (8;14), (2;8), (8;22).

28
Q

What are the clinical features of Burkitts that predict for a poor prognosis?

A

Age greater than 40, LDH 3 or more times the upper limit, ECOG PS 2 or more, and CNS involvement.

29
Q

What is the treatment of Burkitts Lymphoma in less than 60 years of age?

A

CODOX-M, Hyper-CVAD, DA-EPOCH-R. Also given w/ IT MTX and cytarabine and systemic high dose MTX.

30
Q

What chemo regimen do you give patients 60 years or older with Burkitts?

A

DA-EPOCH-R

31
Q

In CD30+ sCD3 peripheral T cell lymphoma (anaplastic T cell lymphoma) what medication was found to be associated with a PFS and OS benefit?

A

Brentuximab vedotin when swapped with Vincristine w/CHOP regimen.

32
Q

What is the flow phenotype in Adult T cell leukemia/lymphoma?

A

CD2, CD4, CD5 positive, CD 8 negative and CD 25+.

33
Q

What is the mechanism of action of Brentuximab?

A

Binds CD30 expressing cells. Releases MMAE which binds microtubles and inhibits mitosis.

34
Q

For adult T cell leukemia/lymphoma specifically the lymphoma subtype you use what treatment?

A

DA-EPOCH or CHP with Brentuximab. You can use CHOP in those that can’t tolerate the above regimens or lack CD30.

35
Q

For the acute and chronic/smoldering types of adult T cell lymphoma what treatment do you use?

A

Zidovudine and interferon therapy.

36
Q

What is the mechanism of action of Mogamulizumab?

A

Monoclonal antibody targeting CCR4, which is epxressed in 90% of cases of ATLL.

37
Q

For the acute and lymphoma subtype of Adult T cell lymphoma/leukemia what treatment should they receive after?

A

Allogeneic SCT due to high relapse rate.

38
Q

What are the options for refractory PTCL-NOS? Can you remember the mechanism of action?

A

Pralatrexate (folate analogue), Romidepsin (HDAC inhibitor), Belinostat (HDAC inhibitor), Brentuximab (if not already received and CD30 greater than 10%). Initial tx-CHOP, CHOEP, DA-EPOCH

39
Q

What consolidative therapy are PTCL-NOS patients offered?

A

HDC/auto-SCT

40
Q

In essence when looking at the GELF criteria in Follicular Lymphoma what is the final takeaway?

A

Any patient who is symptomatic (has B symptoms, splenomegaly, cytopenias) or has extensive disease (3 or more nodal sites greater than 3cm) or nodal/extranodal greater than 7 cm will need treatment (bulky disease).

41
Q

What are the tx options for Stage I or Stage II contiguous Follicular Lymphoma?

A

Asymptomatic-observation
Symptomatic-ISRT alone (preferred). You can also give ISRT with Rituximab or Obintuzumab +/-chemotherapy. OR you can do anti-CD 20 with chemo only.

42
Q

What are the tx options for Stage II non-contiguous disease in Follicular Lymphoma?

A

Asymptomatic-observation
Symptomatic-Rituximab or Obintuxumab +/- chemotherapy +/-ISRT for local palliation.

43
Q

What are the chemo options for advanced stage Follicular Lymphoma?

A
  1. CHOP w/Rituximab or Obintuzumab
  2. CVP w/ Rituximab or Obintuzumab
  3. Bendamustine w/Rituximab or Obintuzumab
  4. Lenalidomide+Rituximab
  5. Observation for those w/no indication for tx
44
Q

What is the tx of choice for low burden dx in asymptomatic Follicular Lymphoma

A

You can either observe (preferred) or give 4 doses of Rituximab w/o maintenance and retreat when they develop dx again or you can give maintenance (no diff in OS).

45
Q

When thinking about Follicular Lymphoma and who needs treatment what are the two things to look for?

A

Are they symptomatic and what burden of disease do they have (high/bulky disease-7cm vs low).

46
Q

What is the benefit of Obintuzumab plus Bendamustine in relapsed/refractory FL?

A

It has an OS benefit.

47
Q

In general what are the options for refractory FL in the second line setting?

A

Basically all the agents you can use in the first line setting.

48
Q

What are the options in the third line setting for FL?

A

Copanlisib, Tazemetostat.
CAR T-Axi Cel and Tisalucel
BITE therapy-Mosunetuzumab

49
Q

What is the flow phenotype for FL? What is the chromosome translocation?

A

CD 10, CD20, BCL-2, BCL-6, CD 23 +/-
t(14,18)

50
Q

Grade 3B FL should be treated as and regarded as?

A

DLBCL.

51
Q

What is the immunophenotype of MZL? What key mutation separates this from Waldenstroms?

A

CD 20, CD79a, CD 19, CD 5 and 10 neg, CD 23 neg, cyclin D1 neg
MYD 88 mutation.

52
Q

What mutation in MALT lymphoma predicts resistance to H. Pylori therapy? In addition to antibiotics what do you do?

A

t(11;18)
Besides antibiotics you also give these patients ISRT or Rituximab.

53
Q

If the flow phenotype is consistent with MZL, but is positive for CD 5 what do you use to differentiate from MCL?

A

Positive for cyclin d1 and t(11;14).

54
Q

What is the antibiotic therapy used for MALT Lymphoma?

A

PPI plus 2 antimicrobials (Charithromycin, Amoxicillin)
Quadruplet therapy-PPI, tetracycline, Bismuth, and flagyl

55
Q

How long after treatment in MALT lymphoma will they need to be biopsied again?

A

3 months.

56
Q

What virus can be assoc with Splenic Marginal Zone Lymphoma and can resolve with treatment for that?

A

Hepatitis C

57
Q

What are the treatment principles of splenic MZL?

A

Look for Hep C, if positive then treat Hep C. If neg then give Rituximab. Can consider splenectomy in young healthy fit patients (Cat 2B Rec).

58
Q

What in the flow markers help you differentiate Splenic MZL from HCL, CLL, FL, and MCL?

A

The lack of CD5 rules out CLL and MCL. The lack of CD 10 rules out FL. And the lack of CD 103 rules out HCL. Of note it is positive only for CD20, CD23 +/-, CD43 +/-, IgM, and CD22

59
Q

What are the second line tx options for MZL? Just have a general idea of how to approach these patients.

A

R-CHOP, R-CVP, BR, BO (obintuzumab), Acalabrutinib, Zanabrutinib, Ibrutinib (due to toxicity profile use the others first), Len/Ritux

60
Q

What are regimens to use for fit patients as initial treatment for MCL?

A

Nordic regimen, R-CHOP/RDHAP, R-HCVAD followed by auto SCT then Ritux maintenance for 3 years

61
Q

What are the second line options for MCL?

A

BTK inhibitors, Len-Ritux, Venetoclax (in third line setting), Chemoimmunotherapy, CAR-T (has a PFS and OS benefit).

62
Q

What flow markers are positive in Hairy Cell Leukemia?

A

CD19, CD20, CD22, but remember these-CD25 and CD103 and CD11c.

63
Q

What are less intense regimens you can offer an older patient with Mantle Cell Lymphoma?

A

Bendamustine/Rituximab, V(Bortezomib)R/CAP, R-CHOP, Lenalidomide/Rituximab