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Hematology > NHL > Flashcards

NHL Flashcards

1
Q

4 factors in the MIPI score

A

Age, ECOG, LDH, WBC (for mantle cell)

MIPI-c adds Ki67

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2
Q

Factors in the FLIPI score

A

“No-lash”

Nodal sites >/= 5
LDH above ULN
Age >60
Stage III/IV
Hemoglobin <120

0 to 1= “low risk” with a 10y OS of 70%.
2= “intermediate risk”, 50%.
≥ 3 =“high risk” with a 10 year overall survival of 35%.

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3
Q

Factors in the FLIPI-2 score

A

“B-bash”, where s is “size”

B2-MG above ULN
BM involvement
Age >60
Largest node >6cm
Hemoglobin <120

0=low risk
1-2=intermediate
3-5=high risk

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4
Q

Name 3 chemotherapies that cause MAHA

A

Bevacizumab
Bortezomib
Carfilzomib
Gemcitabine
Mitomycin
Oxaliplatin
Pentostatin
Sunitinib

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5
Q

What are the two trials which showed superiority of BR over R-CHOP or R-CVP for indolent lymphomas?

Name side effect that was more common with bendamustine-ritux than RCHOP/RCVP

A

StIL Trial (2013): noninferiority BR vs CHOP-R for indolent/MCL
Primary: PFS
BR had better PFS and less toxic effects (peripheral neuropathy, paresthesia, alopecia, myelosuppression)

BRIGHT Trial (2014): BR vs CHOP-R/CVP-R indolent/MCL
Primary: CR
BR had better CR and ORR
BR had more n/v and drug-hypersensitivity reactions, but LESS peripheral neuropathy/paresthesia and alopecia, myelosuppression/neutropenia
5 year follow up showed greatest improvement in PFS seen in mantle cell group

Side effect that was more common with BR than RCHOP/RCVP…Rash/Skin reaction

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6
Q

What mutation is common in Waldenstrom Macroglobulinemia, the same one of which can also be acquired in patients treated with G-CSF?

A

MYD88 (seen in >90% of patients with WM) L265P
CXCR4 (30-35% of patients with WM, worse response to Ibrutinib)

G-CSF mechanism in part is down regulation of CXCR4.

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7
Q

Name 4 treatment strategies for PTLD

A

1.Reduce immunosuppression
2.Single agent rituximab
3.Chemotherapy (CHOP) if fails to achieve CR with IST
5.If localized disease, can get surgery or localized XRT
6.Donor T cells if donor ebv+
7. Antiviral if primary EBV infection (cidofovir) Not sure if this is treatment or prevention

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8
Q

Diagnosis of PTLD

A

Biopsy or EBV >30,000 and and one of:
Splenomegaly
Mass
LAD
B lymphocytosis or kappa/lambda predominance
Unexplained fever> 38.5, if fever is the only symptom, then EBV must be >300,000.

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9
Q

Name 3 malignancies associated with HHV8

A

Kaposi Sarcoma
Castleman’s Disease
Primary Effusion Lymphoma
HHV8 positive DLBCL, NOS (new in WHO 2016)

*stain is LANA-1

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10
Q

Factors in IPI for DLBCL

A

“APLES”
Age >60
PS (ECOG) >/= 2
LDH elevated
Extra-nodal side >1
Stage III or IV

 0-1 is Low
 2 is Low-intermediate
 3 is High-intermediate
 4-5 is High

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11
Q

Other than IPI, 8 poor prognostic factors in DLBCL

A
  1. CNS involvement
  2. Testicular involvement
  3. BM involvement
  4. Double/triple expressor BCL2 and c-MYC
  5. Double HIT
  6. Triple HIT
  7. Bulky disease (node >10cm)
  8. ABC subtype cell origin
  9. Baseline immunocompromised host- HIV, low CD4 count
  10. Active infection at diagnosis delaying chemotherapy
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12
Q

Explain role of PD1 & PD1L interaction

A

PD-L1 on the tumor cell binds to PD-1 receptor on the cytotoxic T cell –> inhibition of T cell mediated anti tumor effect leading to loss of cytotoxic functionality and absence of cell-dependent cytotoxicity and apoptosis.

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13
Q

3 subtypes of DLBCL by gene expression profiling

A
  1. Germinal center B cell (more BCL-2, m-TOR)
  2. Activated B cell (more NFKB)
  3. Mediastinal large B cell (elevated , PDL1, PDL2)
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14
Q

You have a patient with DLBCL who has completed 6 cycles of R-CHOP and has a negative PET scan at the end of therapy. What surveillance imaging schedule would you recommend at this point?

A

No surveillance if post PET negative

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15
Q

MOA of Nivolumab

A

PD1 inhibitor

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16
Q

What were the treatment arms and conclusion of the ECHELON 2 trial in PTCL?

A

ECHELON-2 trial (Lancet 2019)
o Is A+CHP better than CHOP for CD30+ PTCL?
o Incl: CD30+ PTCL (75% Anaplastic large cell lymphoma) measurable disease, ECOG ≤2, not cutaneous, no CNS disease
o median PFS 48.2 mo in A+CHP vs 20.8 in CHOP.

*brentuximab vedotin, cyclophosphamide,
doxorubicin, and prednisone (A+CHP)

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17
Q

What is the flow cytometry pattern for T-LGL?

A

CD3, CD8, CD16, CD57 positive, CD4 negative.

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18
Q

3 drug classes of antiemetics

A
  1. ondansetron - 5HT3 receptor antagonist
  2. olanzapine – dopamine antagonist
  3. aprepitant – NK1 (neurokinin) antagonist/reduces substance P
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19
Q

5 poor prognostic factors in primary CNS lymphoma. ie. PCNSL prognostic score

A

“APLES”
1. Age >60
2. Performance, ECOG >/= 2
3. LDH (Elevated)
4. Elevated protein levels in CSF
5. Site-deep lesion in the brain

0-1=low
2-3 = int
4-5 = high

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20
Q

What are 3 cytotoxic agents to treat CNS lymphoma? What are options for R/R PCNSL?

A
  1. Cytarabine
  2. MTX
  3. Thiotepa
  4. Rituximab
    (MATRiX)
  5. Ifophospamide

If r/r: Temozolamide plus ritux (PD-1 inhibitors (nivolumab), ibrutinib, lenolidamide, WBRT, Pemetrexed)

If ASCT: conditioning (thiotepa and busulfan).

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21
Q

Define monoclonal B cell lymphocytosis. What is the distinction between high and low count and the significance for pts.

A

Monoclonal population of B lymphocytes <5x10’9 detected on flow cyto of peripheral blood.

Low count-<0.05x10’9 monoclonal B cells (marginal increased risk of transformation to CLL, and increased infx and secondary malignancy)

High count-2.0-5.0x10’9 monoclonal B cells. ~1%/yr risk of progression to CLL, 3x increased risk of infxn/malignancy.

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22
Q

What is the most common cytogenetic/molecular abnormality in gastric MALT?

A

t(11:18) API2/MALT1
1. Predicts poor response to treatment with H pylori eradication alone
2. LESS likely to transform to DLBCL

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23
Q

Which of the NHLs are non-FDG avid?

A

Non-FDG-avid: CLL/SLL, LPL, marginal zone

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24
Q

Immunophenotype of follicular lymphoma. What is the oncogene and it’s function? What is the translocation?

A

Follicular: CD5-, CD10+
Pan B-cell antigens (CD19, CD20, CD79a), CD21 +sIG
Oncogene: BCL2, anti-apoptosis
t(14;18), IgH/BCL2

Others:
t(2;18) – kappa LC promoter to BCL2
t(18;22) – lambda LC promoter

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25
Q

Immunophenotype of MCL. What is the oncogene and it’s function? What is the translocation?

A

MCL:CD5+,CD10-, CD20+/sIG+, FMC7+, CD23-, CD200-
SOX11 (+ in classic, - in leukemic variant)
Oncogene: CyclinD1, cell cycle regulator
t(11;14), CCND1/IGH

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26
Q

Classic immunophenotype of DLBCL. What is the oncogene and it’s function? What is the most common translocation?

A

CD5–, CD10+, CD23–, CD25–, CD45+, CD56–, CD103–
Pan B-cell antigens (CD19, CD20, CD22, CD79a) +k/lambda restricted
t(14;18), BCL2, anti-apoptosis.
t(3;14) , BCL6, anti-apoptosis.
(can also have c-MYC)
*CD5+ or EBER expression are poor prognostic markers

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27
Q

Classic immunophenotype of MZL. What is the most common translocation? What is the significance?

A

o CD5–, CD10– (can be +), CD23– (CD 21, CD 35)
o Pan B-cell antigens (CD19, CD20, CD22)
o Often trisomy 3 or t(11;18) [API2-MALT], latter worse response to H.pylori Tx.

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28
Q

Classic immunophenotype of LPL. What is the oncogene and it’s function?

A

o Usually CD5–, CD10– and CD23–, CD 138+
o Pan B-cell antigens (CD19, CD20, CD22, CD79a)
o Surface IgM (rarely IgG or IgA)
o Variable CD11c, CD25, CD38

MYD88, proliferative enhancer

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29
Q

Classic immunophenotype of HCL. What is the oncogene and it’s function?

A

CD103+, CD11c+, CD25+, CD123+, CD22+
CD5-, CD10-, CD 23-
Pan B-cell markers: CD19, CD20, CD22 are bright
BRAF V600E, proliferation (activating mutation of MAPk signaling)

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30
Q

Factors in rIPI for DLBCL

A

still “APLES”

Age >60
PS (ECOG) >/= 2
LDH elevated
Extra-nodal side >1
Stage III or IV

Splits into 3 groups: very good=0, good= 1-2, and poor= 3-5

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31
Q

Very elderly/frail pt with DLBCL. 2 treatment options. What are some supportive adjuvants before starting or during chemo?

A
  1. R-mini-CHOP with dose-reductions of 50% for the cyclophosphamide, doxorubicin, and vincristine; and prednisone 40mg/m2
  2. Rituximab single-agent (35% RR)
  3. Palliation +/- radiation if localized disease

*older women have reduced rituximab clearance, resulting in higher serum levels and prolonged exposure times. (no data of changing R dose base on sex currently)

Adjuncts:
 steroid pre-phase (3-7days of 100mg prednisone daily) prior to doing mini-R-CHOP. (improved ECOG and tolerance of cyc1)
 Consider giving an extra rituximab loading dose prior to the first cycle of CHOP
Suggest G-CSF prophylaxis with Pegfilgrastim x1 on day 3 or 4

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32
Q

What is the CNS-IPI score?

A

Age >60
Elevated LDH
ECOG 2-4
Stage III-IV
>1 extranodal site
kidney or adrenal involvement

o 0-1 – low risk (~3%)
o 2-3 – intermediate risk (~4%)
o 4-6 – high risk (~12%)
• If high risk should get high-dose systemic MTX, usually day 15 of 21 of R-CHOP, alternating cycles. (total of 3 infusions)

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33
Q

o Indications for ASCT after R-CHOP (4-6 cycles) for DLBCL?

A

• PET+ after 6 cycles of R-CHOP (change in SUVmax <66% from baseline)
• High CNS IPI score

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34
Q

Tx options of c-MYC re-arranged DLBCL (No BCL2 or 6)?

A

 DA-EPOCH-R or Magrath

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35
Q

Tx options of double/triple expressor DLBCL? Double or triple hit?

A

Double expressor:
o RCHOP ± HDMTX after cycles 2/4/6

• Double/triple hit:
o DA-EPOCH-R + HD MTX after 2,4 or 3,6
o RCODOX-M/IVAC
o R-HyperCVAD/MA (rituximab plus hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone alternating with methotrexate and cytarabine).

*no benefit has been shown for auto-SCT, however high risk so watch carefully!

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36
Q

Options for Tx of r/r DLBCL, transplant and non-tx eligible?

A

Tx candidate:
-Salvage (eg. R-DICEP, RGDP or R-MICE)—>auto SCT
(repeat imaging after 2 cycles to document chemosensitivity)
- Salvage chemo to CAR-T

Non-TX candiate: (prognosis <6mos)
• R-GDP (gemcitabine, Dex, cisplatin)
• R-DHAP, ICE, CEPP, MEP
Symptom control with IFRT
-Polatuzumab vedotin (Ab drug conjugated that targets CD79b) + BR or BG
-Selinexor single agent

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37
Q

You diagnosis a man with primary CNS lymphoma based on MRI and sterotactic brain biopsy. What are your next investigations to help with staging? What are two things that you might look for on PE that wouldn’t otherwise look for typically?

A
  1. Lumbar puncture (elevated protein)
  2. LDH

PE:
1. Slit examination
2. Testicular exam (need a testicular ultrasound)

Treat with MATRix
Relapse, Temozolamide plus ritux (PD-1 inhibitors (nivolumab), ibrutinib, lenolidamide, WBRT, Pemetrexed)

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38
Q

Treatment of primary mediastinal B-cell lymphoma?

A

o ASH says DA-EPOCH-R is standard of care without radiation!

 75% cure rate, and OS >90%

 IFRT should probably be only used for:
• Bulky disease at diagnosis (>10cm) that do not respond well to chemo (ie. <50% response)
• Patients with positive end-of-treatment PET (may consider delaying the PET until 6 weeks post, instead of 3-4 weeks post)

Relapsed:
Auto transplant, pembrolizumab

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39
Q

Pt with follicular lymphoma, limited stage (IA or perhaps contiguous IIA). After discussion with the pt you decide to treat with observation (over RT). How often will you follow and when will you perform imaging next?

A

o If watchful waiting, then clinical follow-up q3-6m and CT CAP at 1yr post-Dx
 If still not meeting treatment criteria after 1 year, then a CT at 2yr post-Dx can be considered

40
Q

Treatment of advanced stage follicular lymphoma? What are options for frail pts with ++comorbidities?

A

o Bendamustine + Ritux or G (6 cycles of 28d)
 Add maintenance therapy with rituximab 375mg/m2 every 3 months for 2 years (8 doses)

o Limited life-expectancy from comorbidities, or do not want IV:
 PO chlorambucil + Ritux

If ok with IV:
Single agent Ritux x4 then observation (or Ritux weekly x 4, then q2months for another 4 doses total).

41
Q

Relapsed follicular lymphoma >2 years from last treatment. What are potential Tx options?

Of note, if relapse <2yr—>auto SCT

A

 If ≥5yr remission, can try initial treatment again

-CHOP- O or R
-Lenalidomide +O or R
-bendamustine +Obinutusumab better than bendamustine alone regarding PFS in Gadolin trial (use proph abx as inc infections)
-stem cell transplantation (auto)
-Tazemetostat (EZH2 wild type or unknown relapsed/refractory disease in patients who have no satisfactory alternative treatment options)
-Copanlisib
-CarT

 Ritux maintenance should only be used once in the course of the disease (either first remission or first relapse)!

Ps. No data for ritux maintenance for FL after BR, our rational is extrapolated from benefit (PFS, EFS, but not OS) post R-CHOP and post BR in MCL, LPL, MZL.

42
Q

What is the GELF criteria for treating follicular lymphoma?

A

o Involvement of ≥3 nodal sites, each with a diameter of >3cm (FLIPI sites)
o Any nodal or extranodal tumor mass with diameter of ≥7cm
o Presence of B symptoms
o Risk of local compressive symptoms that may result in organ compromise
o Cytopenias (WBC <1.0, PLT <100)
o Leukemia phase (>5.0 x10⁹/L circulating malignant cells)
o Splenomegaly (>16cm on CT)
o Pleural effusion or peritoneal ascites

43
Q

How is gastric MALT staged?

A

Staging is usually done by Lugano system for GI lymphomas:

Summary: I gastric, II nodes around GI tract, IIE serosal/adjacent invasion, no III, IV distance.

 Early (Stage I/II)
• I: Single primary lesion or multiple non-contiguous lesions, but must be confined to GI tract
• II: extends beyond GI tract into the abdomen
o II1: involves local nodes
o II2: involves distant nodes
o IIE: penetrates the serosa to involve adjacent organs or tissues
 There is no Stage III
 Stage IV: disseminated extranodal involvement or concomitant supra-diaphragmatic nodal involvement

44
Q

Pt with gastric MALT. You prescribe H.pylori eradication with Omeprazole, clarithromycin, + metronidazole (or amoxicillin ). What is the next step in management? (ie. when do you repeat EGD?).

There is still evidence of H.pylori. What now?

What do you counsel the pt on when the MALT lymphoma will regress?

A

 Repeat EGD with Bx for lymphoma and H pylori at 3 months, and then q6m for 2 yrs, then q1y for 3 yrs.

 Can repeat treatment once if H pylori persists

 The MALT lymphoma may regress slowly over 12-18 months once H pylori is eradicated. If persists–>RT

45
Q

Gastric MALT, but H.pylori is negative. What is the treatment?

A

RT

46
Q

Gastric MALT, stage IIAE or greater. How do you treat?

A

-Treat as advanced low-grade lymphoma, eg. BR
-Plus eradicate H Pylori if positive

47
Q

Splenic Marginal Zone Lymphoma who was managed expectantly, but has now developed poor prognostic signs (low Hg, plt, LDH, LAD). What is first line therapy?

A
  1. Ritux weekly x 4,
    –>If they respond (at least PR), then do maintenance ritux q3 months for 2 years
  2. If non-response or progressive disease–> splenectomy.
  3. B-R if additional nodal disease, extensive BM involvement, or non-operative candidate–>maintenance ritux q3m for 2 years

*Screen all for HCV (if +, may regress with Hep C therapy)

48
Q

What are 3 lymphomas associated with the HCV?

A
  1. SMZL
  2. LPL
  3. Nodal MZL
  4. DLBCL (less often)
49
Q

What are 3 lymphomas associated with the EBV?

A
  1. HL
    2.Burkitt
  2. PTLD
  3. Extranodal NK/TC lymphoma
  4. DLBCL
  5. 1* CNS lymphoma in HIV
  6. Lymphomatoid granulomatous
    8.Angioimmunoblastic T cell lymphoma
50
Q

Which bacteria is associated with each of the following:
1. Gastric MALT
2. Ocular MALT
3. Cutaneous MALT
5. Intestinal MALT lymphoma

A
  1. Gastric MALT=H.pylori
  2. Occular MALT=Chlamydia psittaco
  3. Cutaneous MALT=Borrelia burgdorferi
  4. Intestinal MALT lymphoma= Camplyobacter jejuni
51
Q

What is the distinction between LPL and WM?

*note, this is controversial

A

WM has bone marrow involvement and is accompanied by an IgM monoclonal protein of any size.

52
Q

What are 5 treatment indications for LPL? (hint: there are 5 specific features of paraprotein-related complications)

A
  1. Constitutional symptoms
  2. Progressive LAD or HSM
  3. Cytopenias: Hg<100, or plt <100
  4. Progression to a high-grade lymphoma
  5. Clinical features of paraprotein-related complications:
    -Peripheral neuropathy
    -Amyloidosis
    -Symptomatic cryoglobulinemia,cold agglutinins
    -Renal impairment (eg. MGRS)
    -Hyperviscosity
  • IgM level is not an indication!
53
Q

What is moxetumomab and what disease is it used to treat?

A

Anti-CD22 immunotoxin medication for the treatment of adults with relapsed or refractory hairy cell leukemia who have received at least two prior systemic therapies, including treatment with a purine nucleoside analog.

  • serious adverse reaction is HUS
54
Q

What are 3 treatment options for burkitt lymphoma, specifically, what common treatment is NOT adequate?

A
  1. R-CODOX-M/IVAC
  2. DA-EPOCH-R + HD MTX after 2,4 or 3,6
  3. R-HyperCVAD/MA

*Cannot give R-CHOP, inadequate for burkitt!
*All patients need CNS prophylaxis

55
Q

3 options for LPL treatment? Fit and co-morbid.

A
  1. BR with maintenance Ritux
  2. Bortezomib, dexamethasone, and rituximab
  3. Cyclophosamide, dexamethasone and Rituximab

For elderly/highly comorbid:
1. Ibrutinib with or without rituximab (if old or comorbidities)
2. Single agent Ritux (especially if not that symptomatic)

*always want to add Ritux if possible

*Ritux can precipitate IgM flare (can PLEX or steroid pre-tx before starting R)

56
Q

WM relapsed after BR + Ritux maintenance, not ASCT candidate and high co-morbidity index, what treatment do you suggest?

A

*If >3 years from initial treatment can re-trial a front line option if able to tolerate

Otherwise:
Ibrutinib + Ritux
Zanibrutinib
Acalabrutinib
Any front line options not used (Bortezomib/Cyclo + Dex/Ritux)

Very frail:
Single agent Ritux
Single agent Benda

–>Auto-Tx (not great evidence, only if young/fit and multiple relapsed)

57
Q

Mantle cell lymphoma, what are 4 good prognostic factors? (other than MIPI)

A
  1. Non-nodal presentation (ie. leukemic/indolent subtype)
  2. Predominantly hypermutated Ig heavy chain variable regions
  3. Non-complex karyotypes
  4. SOX11 negative on IHC
  5. Ki 67<10%

In these pts can consider watchful waiting, in all others treatment!

Other options are: Single agent Ritux, ibrutinib, lenolidamide.

58
Q

Mantle cell lymphoma, what are 4 poor prognostic factors?

A
  1. High burden nodal disease
  2. Ki-67 ≥20-30%
  3. Blastoid histology
  4. P53, SOX11 or Notch1 mutation, gene expression profiling and altered microRNA signature
59
Q

2 treatment options for high risk MCL if transplant eligible. (high MIPI, high ki67%). What is an option if they relapse after auto-SCT?

A
  1. BR x 3 cycles, then RC (cytarabine) x 3 cycles, then Auto-SCT transplant
    –> cytarabine improves PFS although no OS
  2. R-HyperCVAD
  3. R-CHOP x6 or RCHOP x3 alternating with R-DHAP x3 is an alternative for initial chemo regimen)—> we don’t use, as not change in OS and more toxicity
  4. Auto-SCT
    oMaintenance rituximab q2 months x 3 years post-auto-SCT and if PR after R-CHOP regimen (no great data for maintenance after BR)

–>If relapse after auto-SCT, can try allo-SCT or CAR-T

60
Q

2 treatment options for high risk MCL if transplant ineligible.

A
  1. B-R induction x6 cycles, then prolonged Ritux maintenance q2mo until progression (up to maximum 4 years)

*Maintenance Ritux after BR is controversial
2. Ibrutinib
3. Single agent Ritux
4. IFRT alone if ≥70 years old or significant comorbidities
5. Lend +Ritux

61
Q

What is the treatment of Burkitt’s lymphoma?

A

Aggressive combo chemo (eg. R-CODOX-M/IVAC) with proph IT chemo

R-CODOXM (Rituximab, cycophosphamide, vincristine, doxorubacin, Methotrexate)
IVAC- Ifosfamide, Etoposide, Cytarabine (intrathecal MTX).

IT chemo (cytarabine/MTX)

Others: DA-EPOCH-R HD with MTX after 2,4 or 3,6, HYPERCVAD-R/MA

62
Q

Testicular lymphoma treatment?

A

R-CHOP x 6 + CNS prophylaxis with HD MTX, RT to contralateral testes (primary tested is removed to make diagnosis).

Often express PDL1 and MYD88 (allows entry into sanctuary site)

63
Q

What prophylaxis would you give for Alemtuzumab? What are 3 things you are concerned about?

A
  1. Infusion rxn
  2. CMV reactivation
  3. Cytopenias
  4. Auto-immune thyroiditis

1.Septra DS 3x/week
2. Acyclovir 400 BID (or Valacyclovir) x 2 months post-chemotherapy
3. If HBsAg or HBcAb+, Lamivudine until 6 months post-therapy
4. CMV DNA qweekly –> Ganciclovir if positive infection
(start 1 week before, continue 2 months after)

64
Q

What are 3 lymphomas associated with HIV infection?

A

Burkitt lymphoma
DLBCL (immunoblastic variant) (CD4 usually <100)
Primary CNS lymphoma (CD4 usually <50)
Plasmablastic lymphoma
Primary effusion lymphoma
Hodgkin Lymphoma (CD4 usually >200)

65
Q

3 translocations seen in Burkitt’s lymphoma

A
  1. t(8;14), c-myc/IgH
  2. t(2:8), kappa light chain/IgH
  3. t8;22), lamda light chain/IgH
66
Q

2 translocations seen in anaplastic large cell lymphoma, ALK +

A

t(2;5) – (~75%) NPM-ALK fusion, acts as constitutively active TK
t(1;2) (~15%) TPM3-ALK fusion

67
Q

Classic immunophenotype of HCL variant.

A

Variant HCL: CD103+/11c+/CD25-/CD123-/CD22+

BRAF negative!

68
Q

Factors in the age-adjusted IPI score

A

For pts <60, removes ENS

ECOG >/=2
LDH elevated
Stage III/IV

69
Q

What is lymphomatoid granulomatosis?

A

Extranodal, predominantly pulmonary (90%), angiocentric &/or angiodestructive EBV(+) B-cell LPD

-Spectrum of histological grade and clinical aggressiveness, which is related to the proportion of large B cells (neoplastic B-cells are EBV positive)

-Usually young pts (30-40)

-RFs: HIV, Wiskott Aldrich (“tie” thrombocytopenia, immunodeficiency and eczema), HSCT

-Tx as DLBCL if high grade, high spontaneous remission if low grade.

70
Q

6 forms of PTLD according to the 2017 WHO

A

Non-destructive PTLD (“early disease”, more EBV association, usually milder)
1. Plasmacytic hyperplasia
2. Infectious mononucleosis-like PTLD
3. Florid follicular hyperplasia.

Destructive PTLD
1. Polymorphic PTLD
2. Monomorphic PTLD
3. Classic Hodgkin lymphoma-like PTLD

71
Q

What 3 lymphomas, when diagnosed in the post transplant setting are not considered PTLDs?

A

Small B cell lymphoid neoplasms (eg, follicular, small lymphocytic lymphoma) and MALT lymphomas arising in the post-transplant setting are not considered PTLD.

72
Q

What is the treatment of primary effusion lymphoma?

A

Usually CHOP +/- R.
-No R if CD 20- (obviously) or if CD4 <50 because risk of infxn is too high.
- Usually, pt population is immunocompromised–>HSCT, HIV, elderly, kaposi’s.

No methotrexate with an effusion!

73
Q

2 clinical differences between NHL with and without a concurrent diagnosis of HIV.

A

With HIV:
1. More likely CNS or other extranodal regions (rectum, body cavity)
2. More likely to be advanced stage
3. More likely to be associated with other viruses (HCV, EBV, HHV8)
4. More PCNSL, PEL.

74
Q

What are 3 options for the treatment of relapsed HCL?

A
  1. if >24 months since relapse (and especially >60mos) can re-trial nucleoside analogue +/- Ritux. Either Cladrabine or pentostatin
  2. vemurafenib, dabrafenib +/- Ritux (skin rash, arthralgias, arthritis, or secondary skin tumors)
  3. Moxetumomab pasudotox + psuedomonas exotoxin (anti CD-22)
  4. Old: Splenectomy, interferon-alpha
75
Q

What are 5 differences clinically (or by flow) for HCL and hairy cell variant?

A

Compared to HCL, HCL variant has:
1. Higher WBC
2. Less/no monocytopenia
3. Less likely to get dry tap (less reticulin fibrosis)
4. CD 25, CD 123, annexin A1 negative
5. BRAF V600E mutation is not present
6. Less responsive to cladrabine

76
Q

According to the Gallium study, G-CHOP (Obinutuzumab) had better PFS than R-CHOP. However there were 2 side effects which were seen more commonly in the G-CHOP group. What were these?

A
  1. Neutropenia
  2. Infection
77
Q

What are 5 potential treatment options for relapsed refractory MCL?

A
  1. Ibrutinib, Acalabrutinib, Zanubrutinib (+/- Ventoclax)
  2. Lenolidamide +Ritux
  3. BR (if not used in first line)
  4. CAR-T (with CD8+ selection, to not include any leukemic mantle cells, bruxu-cel)
  5. Auto (if not done in first line), Allo-if failed auto.
78
Q

When would you consider allo-SCT for MCL?

A

a. First remission only if: TP53-mutated, blastoid variant, or high risk MIPI (full myeloablative conditioning).
b. Relapsed MCL (1 or 2 dn relapse)
i. >1year following ASCT (reduced intensity conditioning for alloSCT if prior ASCT)
ii. If no prior ASCT (full myeloablative conditioning pre-AlloSCT)

79
Q

3 WHO sub-types of MZL.

A
  1. Extranodal MZL (MALT)
  2. Nodal MZL (behaves like follicular)
  3. Splenic MZL (rule out HCV!)
80
Q

What does MALT stand for? Other than GI, skin and ocular, what are 3 other common locations?

A

mucosa-associated lymphoid tissue.

  1. Salivary glands (Sjogrens)
  2. Lung
  3. Thyroid (Hashimoto’s)
  4. Breast
81
Q

Which lymphoma replaces red pulp of the spleen? Which replaces white pulp?

A

HCL-RED pulp (red head)–> dilated sinusoids filled with blood (blood lakes)
SMZL- White (white margins)

82
Q

3 frontline treatments for LPL/ Waldenstroms (after Tx of hyperviscosity if present)

A
  1. Benda/Ritux
  2. ibrutinib +/- Ritux (may respond worse in CXCR4)
  3. Bortezomib/Dex/ritux
  4. Cyclophos/dex/Ritux
83
Q

5 common side effects of Bendamustine?

A
  1. Hypersensitivity reaction, especially rash.
  2. Myelosuppression
  3. GI–>N/V/D (+ constipation), dyspepsia
  4. HSV/VZV reactivation
  5. Headache
  6. Cough
  7. Hypertensive crises
84
Q

Which flow cyto marker is the hall mark of germinal center B-cell lymphomas?

A

CD10

85
Q

In the Stil trial, of the indolent NHL subtypes, which group showed no benefit with the addition of maintenance rituximab?

A

MZL and MALT

86
Q

What is the dose of “high dose methotrexate”?

A

3500mg/m2 IV

87
Q

What are 3 lymphomas that arise from the germinal centre?

A
  1. Hodgkins
  2. Follicular
  3. DLBCL
  4. Burkitt
88
Q

What are 3 lymphomas that are POST germinal centre?

A
  1. Marginal/MALT
  2. LPL
  3. CLL/SLL (some)
  4. Plasma cell myeloma
89
Q

What are 2 lymphomas that are PRE germinal centre?

A
  1. Mantel cell lymphoma
  2. Some CLL/SLL
90
Q

What are the 3 variants of MCL?

A
  1. Blastoid, ki67>90%
  2. Aggressive ki67 >25%
  3. Indolent ki67 ~10% (behaves like CLL)
91
Q

2 mutations which confer a good prognosis in T-cell lymphomas and 2 mutations which confer a poor prognosis?

A

Good
1. Anaplastic large cell, ALK positive
2. DUSP22

Bad
1. Anaplastic large cell, ALK negative
2. TP63
3. Triple negative

92
Q

Primary management of PTCL NOS, angioimmunoblastic TCL, ALK neg ALCL?

A
  1. CHOEP + auto-SCT (may use CHOP if older >60)
  2. BV-CHP (according to Echelon-2), no vincristine if CD30, increased PFS or OS
93
Q

Frontline option for NK-T cell lymphoma?

A

SMILE + RT (always RT for nasal type if it is localized)

94
Q

Options for relapsed, refractory T cell lymphoma

A
  1. Pralatrexate (targeted anti-folate)
  2. HDAC inhibitor- Romidepsin, Belinostat
  3. P13Ki (Duvelisib)
  4. Lenolidamide
  5. Brentuximab (if CD30+)
95
Q

2 treatment options for advanced mycoses fungoidies?

A
  1. Brentuximab if CD 30+
  2. Mogamulizumab
  3. Pembrolizumab

Hematology (20 decks)

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