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Hematology Pro > NHL > Flashcards

NHL Flashcards

1
Q

What components make up the MIPI score?

A

WALE;

WBC, Age, LDH, ECOG Status

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2
Q

What components make up the FLIPI-1 score?

FLIPI-2?

A

5LASH;
5>= Nodal areas, LDH>ULN, Age>60, Stage III-IV, Hemoglobin <120

(5yr OS %)
Low risk = 0 (80%), Intermediate = 1-2 (51%), High = 3+ (19%)

6BASH;
Largest LN >6cm, B2M>ULN, Age>60, Stage IV(BM involvement), Hemoglobin <120
Low risk = 0 (76%), Intermediate = 1-2 (49%), High = 3+ (37%)

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3
Q

What components make up the IPI?

A

APLES;

Age >60, PS 2-4, LDH>ULN, Extranodal sites >1, Stage III-IV

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4
Q

What components make up the CLL-IPI?

A

B 65, SUM 53

B2M >3.5 (2)
Age >65 (1)
Stage (Rai 1-4) (1)
Ig-UM (2)
P53 Mut (4)

Low = 0-1, Intermediate = 2-3, High = 4-6, V. High = 7-10

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5
Q

What components make up the CNS-IPI

A

APLES + Adrenal/Kidney involvement

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6
Q

What are the translocations common to Burkitt Lymphoma, and what do they involve?

A

t(8;14), t(2;8), t(8;22)

They involve MYC on Chromosome 8, IGH gene on Chromosome 14, Kappa chain on Chromosome 2 and Lambda chain on Chromosome 22

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7
Q

What is the translocation common to Follicular Lymphoma, and what does it involve?

A

t(14;18), involves IGH on Chromosome 14, and BCL2 on chromosome 18

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8
Q

What it the translocation commonly seen in Mantle cell lymphoma?

A

t(11;14) CCND1 and IGH, CyCliN D1

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9
Q

What translocation is seen in Anaplastic Large Cell Lymphoma?

A

t(2;5); NPM1 and ALK

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10
Q

What translocation is seen in Extranodal Marginal Zone Lymphoma which predicts resistance to H. Pylori treatment?

A

t(11;18) -

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11
Q

What are the Rai stages?

What are the Binet stages?

A 
Rai 0 - Lymphocytosis
Rai 1 - Lymphadenopathy
Rai 2 - Hepatosplenomegaly
Rai 3 - Anemia
Rai 4 - Thrombocytopenia

Binet A - Hb>100, Plt>100, and 2 or less clinical nodal areas involved
Binet B - Hb>100, Plt>100 and 3 or more clinical nodal areas involved
BinetC - Hb<100 or Plt <100

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12
Q

Common CLL Cytogenetic abnormalities, ranked by favorability to adverse risk

A

Del(13q) - favorable risk
Trisomy 12 - neutral risk
Del(11q) - poor prognosis
Del(17p) - worst prognosis

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13
Q

CLL Immunophenotype vs MCL Immunophenotype

A

CD5 +, CD10- for both

CLL commonly CD23+, CD200+, CD20dim and FMC7-neg

MCL commonly CD23-, CD200-, Cyclin D1 +ve

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14
Q

Hans Algorithm for subtyping DLBCL

A

CD10 + : GCB

CD10-, BCL6-: non-GCB

CD10-, BCL6+, MUM1+: non-GCB

CD10-, BCL6+, MUM1-: GCB

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15
Q

What is the difference between double/triple expressor vs double/triple hit lymphoma?

A

Double/Triple expressor has detectable MYC AND [BCL2 and/or BCL6] involvement on IHC

Double/Triple hit lymphoma has MYC AND [BCL2 and/or BCL6] involvement on FISH/Cytogenetics

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16
Q

Immunophenotype of HCL? What mutation is often seen in HCL?

A

CD11c+, CD25+, CD103+, CD123+

BRAF V600E

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17
Q

Front-line treatment of Hairy Cell Leukemia?

A

Classically Cladribine or Pentostatin

Some groups consider adding Rituximab up-front

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18
Q

Treatment options for R/R Hairy Cell Leukemia:

A

If refractory:
Try alternative purine analogue, or Vemurafenib + Rituximab

If relapsed:
>24 months: Re-treat with purine analogue previously used + Rituximab

<24 months: 
Vemurafenib + Rituximab (or Dabrafenib)
Moxetumomab Pasudotox (anti CD-22 antibody)
Alternative purine analogue
Monoagent rituximab
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19
Q

1L Treatment for Advanced stage Follicular Lymphoma

A

BR > R2 / RCHOP > RCVP

20
Q

Treatment for R/R FL:

A
  1. If relapsed within 24 months, or 12 months from monoagent Rituximab, then considered v. high risk. Should be offered salvage chemotherapy into autoHSCT
  2. If relapsed >24 months can consider:
    R2, RCHOP, BR (Ideally substitute Rituximab for Obinutuzumab based off GADOLIN and GALLIUM studies)
  3. Multiply relapsed:
    Copanlisib + Rituximab, R2, Tazemetostat, CAR-T, Clinical Trial
21
Q

What is the role of maintenance Rituximab in FL? How is it dosed?

A

It improves PFS, but makes no impact to OS

It is dosed q2-3months x 2 years (8-12 doses)

22
Q

What is the role of maintenance Rituximab in Mantle-Cell Lymphoma?

How is it dosed?

A

Maintenance Rituximab has roles in both post-autoHSCT and post-induction therapy. Post-HSCT it improves OS and PFS. Post-induction (trial was with R-CHOP) it showed OS advantage.

It is dosed after autoHSCT q2months x 3 years
It is dosed after induction therapy q2months until progression (Controversial for BR; MAINTAIN study-branch of the StIL trial demonstrated no PFS/OS advantage)

23
Q

What is the 1L treatment for transplant-eligible MCL?

What is the 1L treatment options for transplant-ineligible MCL?

A

Transplant eligible:
A cytarabine containing regimen x4-6 cycles into cytarabine-based transplant would be optimal. Maintenance rituximab after.
Options are R-CHOP/RHiDAC, R-CHOP/R-DHAPx6, BR, VR-CAP

Transplant ineligible:
BR, R-CHOP, VR-CAP

24
Q

What treatment-options are available to R/R MCL

A
  1. Ibrutinib/Acalabrutinib/Zanubrutinib (BTKi)
  2. Lenalidomide
  3. Bortezomib
  4. BR (If not used front-line)
  5. Intensive chemotherapy into alloHSCT (R-DHAP, R-ICE, R-GDP)
  6. CAR-T (Brexu-cel)
25
Q

What is the 1L treatment for Splenic Marginal Zone Lymphoma?

A

3 options:

Splenectomy, Monoagent Rituximab, BR

26
Q

What are the treatment options for R/R Splenic MZL

A 
BR (if not used up-front)
Re-treatment with mono-agent Rituximab
Ibrutinib (BTKi family)
Lenalidomide / R2
PI3Ki (Idelalisib)
27
Q

What are the treatment options for Gastric EMZL?

What are the strategies for failure after this?

A

If H Pylori positive: Trial treamtent of H. Pylori. Re-assess q6monthly with endoscopy, so long as no progression can wait up to 2 years before calling failure

If H Pylori negative, or t(11;18) then:
A) If all disease sites can be contained in one radiation field can consider IFRT
B) If above not true consider Monoagent rituximab, BR, R-CHOP, R-CVP

For R/R:
C) Consider targeted agents such as BTKi, PI3Ki, or Lenalidomide +/- Rituximab

28
Q

Name the purported etiologic agent with these types of Extra-nodal marginal zone lymphoma:

Ocular:
Salivary Gland:
Thyroid:
Lung:
Stomach:
Small Intestine:
Skin:
Splenic:
A 
Ocular: Chylamydophyla Psittica
Salivary Gland: Sjogrens Syndrome
Thyroid: Hashimoto's thyroiditis
Lung: Sjogrens Syndrome / LIP
Stomach: Helicobacter Pylori
Small Intestine: Campylobacter Jejuni
Skin: Borrelia Burgforderi 
Splenic: Hepatitis C
29
Q

What non-classical (Not in CLL-IPI) risk factors influence risk?

A

CD38 and ZAP70 expression are both deleterious and increase risk. They are surrogates for IGHV-UM status. IGHV-UM does not change with disease evolution, so only needs to be done once.

30
Q

What are the criteria to treat CLL?

A

Lymphocyte doubling rate >6 months, or >50% increase over 2 months (BUT ABSOLUTE LYMPHOCYTOSIS MUST BE >30)
Splenomegaly with spleen >6cm Below Costal Margin
Lymphadenopathy >10cm
Cytopenias from CLL marrow involvement
Autoimmune phenomena from CLL not-responsive to steroids
Constitutional symptoms from CLL

31
Q

What are the 1L treatment options for CLL, and who should receive what?

A

Ibrutinib:
Should be given to patients with IGHV-UM or TP53 +ve

Venetoclax - Obinuzutumab (12 months):
Can be given to patients not needing BTKi

Fludarabine Cyclophosphamide Rituximab:
Consider in young (<65) patients with IGHV-Mutated. Can be curative in some patients, or at-least v. prolonged disease control

Chlorambucil (+/- Rituximab):
Essentially no role anymore except v. frail. Consider adding Rituximab if you think can tolerate

BR:
Can be used in older (>65) patients with IGHV-Mutated disease. VO was better than BR in

32
Q

What are the options for R/R CLL?

A
  1. BTKi (Acalabrutinib, Ibrutinib, Zanubrutinib)
  2. Venetoclax + Rituximab (2 years)
  3. Idelalisib + Rituximab or Duvelalisib + Ritux
  4. AlloHSCT
  5. Chemoimmunotherapy (select patients only)
33
Q

Name 3 chemotherapies that cause MAHA

A
  1. Gemcitabine
  2. Oxaliplatin
  3. Proteosome inhibitors
  4. Pentostatin
  5. Mitomycin
  6. Bevacizumab
  7. Sunitinib and Ponatinib
34
Q

What is PTLD, how is it diagnosed?

A

PTLD occurs typically from EBV reactivation in the setting of suppressed cytotoxic T-cell function post transplant.

It is diagnosed on histology. In a patient post-transplant 2/3 diagnostic criteria are needed to call PTLD:
Distorted nodal architecture from lymphoproliferative disease
Mono/Oligoclonal B-cells
EBV +ve

Any 2/3 makes the diagnosis.

The subclasses of PTLD are division between Early Lesions (non-malignant) vs Malignant (late lesions).

Early: Benign Polyclonal Lymphoproliferation, and Florid Follicular Hyperplasia
Malignant: Monomorphic PTLD (DLBCL, BL, PCN, PTCL-NOS), Polymorphic PTLD (Architecture distortion, poly/monoclonal B-cells, usually EBER+ve, does not meet criteria for a WHO B/T/NK cell neoplasm)

NOTE: Small lymphocytic disorders (FL, SLL, MZL) are NOT PTLD.

35
Q

How do we treat PTLD?

A

Division between Early Lesion and Monomorphic/Polymorphic.

In general the steps are:

  1. Reduce immunosuppression
  2. Rituximab (if CD20+ve)
  3. Rituximab +/- Chemotherapy

In patients with monomorphic/polymorphic disease patients may require advancement to 2 or 3 pending on how they are doing. They are expected to respond less to just reduction of IS, and if PS is poor from malignancy, or anticipated to worsen to point they won’t tolerate chemotherapy soon, you may choose to treat more immediately more aggressively. NB: PTLD can present like cHL. Treat like cHL if this occurs.

36
Q

1L Treatment options for WM/LPL

A
  1. BR (Omit Ritux first cycle if IgM >40g/L) (mPFS in StIL was 70 months vs 28 in RCHOP)
  2. Bortezomib, Rituximab, Dexamethasone (mPFS in one study 43months)
  3. Cyclophosphamide, Rituximab, Dexamethasone (mPFS 34 months)
  4. Ibrutinib, Acalabrutinib, Zanubrutinib (I: estimated PFS at 5 yrs 54%, A:estimated PFS at 2 yrs 90%, Z: too early)
    (May use Rituximab with Ibrutinib if desired)

All are acceptable up-front agents. For frail patients consider BTKi monotherapy.

Rituximab maintenance numerically improved PFS in StiL-MAINTAIN, but was not statistically significant. Can discuss with patient re: addition or not. May have benefit in patients >60yo in subgroup analysis of StiL-MAINTAIN

37
Q

Treatment of R/R WM

A
  1. Patients relapsing >3yrs from frontline treatment can consider re-treatment with the same agents
  2. BTKi
  3. Cyclo/Dex/Ritux
  4. Bortez/Dex/Ritux, CyBorD-R, PI+Ritux
  5. Purine analogues (FR, Cladribine-Ritux, FCR) - only for multiply relapsed patients with good PS
  6. AutoHSCT - Only for young fit patients who have multiply relapsed disease. DO NOT PERFORM ALLO.
38
Q

Alemtuzumab; What are the toxicities? What monitoring is needed? What prophylaxis is needed

A

Highly potent immunosuppressive antibody against CD52.

Toxicities: Infusion reaction, Cytopenias, Immunosuppressive +++, May provoke autoimmune disease ANYWHERE, vigilent monitoring. May increase risk of 2dry malignancies. PML possible. Stroke possible.

Monitoring: Weekly CMV + treatment if starts to spike

Prophylaxis: PJP + VZV prophylaxis. If HepB CAb positive needs prophylaxis against reactivation.

39
Q

HIV-related lymphomas/LPD

A 
cHL
DLBCL
BL
Primary effusion lymphoma
Plasmablastic lymphoma
Primary CNS lymphoma
40
Q

EBV-related lymphomas

A 
cHL
PTLD
Primary CNS Lymphoma
DLBCL
Extranodal NK/T cell lymphoma
Burkitt Lymphoma
Plasmablastic Lymphoma + Primary Effusion Lymphoma
41
Q

HHV-8 Related Diseases

A

HHV +ve multicentric Castleman’s Disease
Kaposi’s Sarcoma
Primary Effusion Lymphoma

42
Q

Primary CNS Lymphoma - HIV Related;

Treatment options

A

1L. High-dose Methotrexate + ART
1L-alt. Temozolomide and Rituximab if not fit for HD-MTX
2L: WBRT, Focal irradiation + Lenalidomide,

43
Q

Primary CNS Lymphoma: Staging

IELSG (International Extranodal Lymphoma Study Group)

A
  1. MRI Brain
  2. LP with cytology
  3. Slit-lamp/Ophthalmology exam (15-25% involvement of eyes)
5 criteria: CLAPD
CSF Protein - Elevated
LDH - Elevated
Age - >60
PS - >2
Deep brain structures involved (cerebellum, brainstem, corpus callosum, basal ganglia)

There is also a MSKCC scoring system:
Good risk Age <50
Int risk Age >50 but KPS >70
High risk Age >50, KPS <70

44
Q

Primary CNS Lymphoma Treatment - Immunocompetent patients

A

HD-MTX is backbone. Rituximab should be considered for all patients as well. Based off results of RCT MATRix (Methotrexate, AraC, Thiotepa, Rituximab) is an excellent 1L choice. Consolidation with AutoHSCT, using Thiotepa based conditioning (not BEAM/Carmustine based)

Induction:

  1. MATRix (HD-MTX, Ara-C, Thiotepa, Rituximab)
  2. R-MT (Rituximab, HD-MTX, Temozolomide)
  3. R-MVP (Rituximab, HD-MTX, vincristine, and procarbazine)
  4. R-MVBP (Rituximab, HD-MTX, teniposide, carmustine (BCNU), and prednisone)

Consolidation with Thiotepa/Busulfan/Cyclophos based AutoHSCT if fit.

10-15% are primary refractory and 50% of patients responding will relapse
R/R:
1. Rechallenge with HD-MTX
2. WBRT
3. BTKi (Ibrutinib)
4. Lenalidomide + Rituximab
45
Q

Secondary CNS Lymphoma: Treatment options

A

Typically a high-grade lymphoma like DLBCL/BL

  1. R-CHOPM (R-CHOP with HD-MTX) + Thiotepa-based autologous HSCT
  2. HyperCVAD / MA
  3. R-CODOX-M/IVAC
  4. MATRix/R-ICE

Consider autologous HSCT in those fit enough to receive

R/R Therapies:

  1. Ibrutinib - particularly ABC-subtypes
  2. Lenalidomide + Ritux
  3. PD-L1/PD-1 inhibitors

Palliative:

  1. Steroids
  2. WBRT
46
Q

CNS-IPI

A

APLES + Adrenal/Renal involvement

47
Q

Differences between HCL and HCL-v

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

Hematology Pro (5 decks)

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