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NCCN > CLL > Flashcards

CLL Flashcards

1
Q

CLL diagnosis requires presence of monoclonal B lymphocytes _______ x 109/L in peripheral blood

A

≥5 x 109/L

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2
Q

Clonality of B cells should be confirmed by

A

Flow cytometry

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3
Q

Presence of lymphadenopathy and/or splenomegaly with monoclonal B lymphocytes ≤5 x 109/L in peripheral blood

A

SLL

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4
Q

TRUE OR FALSE

Biopsy is generally not required.

A

TRUE

Biopsy is generally not required.

If diagnosis is not established by flow cytometry, then proceed with lymph node biopsy.

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5
Q

Diagnostics that are informative for prognostication and/or therapy determination

A
  • FISH to detect: +12; del(11q); del(13q); del(17p)
  • TP53 sequencing
  • CpG-stimulated metaphase karyotype for complex karyotype (CK)
  • Molecular analysis to detect: Immunoglobulin heavy chain variable region gene (IGHV) mutation status
  • Beta-2-microglobulin
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6
Q

Features of Monoclonal B-cell lymphocytosis (MBL)

A
  • Absolute monoclonal B-lymphocyte count <5 x 109/L
  • All palpable lymph nodes <1.5 cm
  • No anemia
  • No thrombocytopenia
  • No palpable organomegaly
  • No constitutional symptoms

Persists more than 3 months

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7
Q

Categories of MBL

A
  • Low-count MBL (<0.5 x 109/L) that rarely progresses to CLL
  • High-count MBL (0.5 – 4.9 x 109/L) that can progress to CLL requiring therapy at a rate of 1% to 2% per year.

Observation is recommended for all individuals with MBL

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8
Q

Typical immunophenotype of CLL:

A

CD5+, CD23+, CD43+/-, CD10-, CD19+, CD20 dim, sIg dim+, and cyclin D1-

Some cases may be sIg bright+ or CD23- or dim
Some MCL may be CD23+

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9
Q

Marker that may distinguish CLL from mantle cell lymphoma (MCL)

A

CD200

CLL: CD200+

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10
Q

May be helpful in suspected cases of MCL that are cyclin D1-negative

A

LEF1 and SOX11

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11
Q

When are CT scans warranted

A

Evaluation of symptoms of bulky disease or for the assessment of risk for TLS prior to initiating venetoclax

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12
Q

Treatment for SLL/Localized (Lugano Stage I)

A

Locoregional involved-site radiation therapy (ISRT) (if indicated)

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13
Q

Indications for treatment:

A
  • Significant disease-related symptoms:
  • Fatigue (severe)
  • Drenching night sweats
  • Unintentional weight loss (≥10% in previous 6 months)
  • Fever without infection
  • Threatened end-organ function
  • Progressive, symptomatic, or bulky disease (spleen >6 cm below costal margin, lymph nodes >10 cm)
  • Progressive thrombocytopenia
  • Progressive anemia
  • Steroid-refractory autoimmune cytopenias

Absolute lymphocyte count (ALC) alone is not an indication for treatment in the absence of leukostasis, which is rarely seen in CLL.

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14
Q

Select patients with mild, stable cytopenia _______________ may continue to be observed.

A
  • ANC <1000/μL
  • Hgb <11 g/dL or
  • Platelet <100,000/μL)

Other causes of anemia/ thrombocytopenia (eg, autoimmune disorders, vitamin/iron deficiency) should be excluded.

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15
Q

TRUE OR FALSE

IGHV mutation status changes over time and analysis should be repeated if previously done prior to initiation of treatment.

A

FALSE

IGHV mutation status does not change over time and analysis does not need to be repeated if previously done prior to initiation of treatment.

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16
Q

Unfavorable Prognostic Variables:

A
  • del(17p)
  • del(11q)
  • Mutated TP53
  • IGHV ≤2% mutation
  • Complex Karyotype

Intermediate: +12, Normal
Favorable: del(13q) (as a sole abnormality), TP53 Wild-type; IGHV >2%

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17
Q

TRUE OR FALSE

Immune-mediated cytopenias are not the basis for these stage definitions.

A

TRUE

Immune-mediated cytopenias are not the basis for these stage definitions.

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18
Q

SLL STAGING SYSTEM
Lugano Modification of Ann Arbor Staging System
(for primary nodal lymphomas)

A

Limited
Stage I: One node or a group of adjacent nodes
Stage IE: Single extranodal lesions without nodal involvement
Stage II: Two or more nodal groups on the same side of the diaphragm
Stage IIE: Stage I or II by nodal extent with limited contiguous extranodal involvement

Advanced
Stage III: Nodes on both sides of the diaphragm; Nodes above the diaphragm with spleen involvement
Stage IV: Additional non-contiguous extralymphatic involvement

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19
Q

Rai System

A
  • 0: Lymphocytosis, lymphocytes in blood >5 x 109/L clonal B cells and/or >40% lymphocytes in the bone marrow
  • I: Stage 0 with enlarged node(s)
  • II: Stage 0–I with splenomegaly, hepatomegaly, or both
  • III: Stage 0–II with hemoglobin <11.0 g/dL or hematocrit <33%
  • IV: Stage 0–III with platelets <100,000/mm3
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20
Q

Binet System

A
  • A: Hemoglobin ≥10 g/dL and, Platelets ≥100,000/mm3 and, <3 enlarged areas
  • B: Hemoglobin ≥10 g/dL and Platelets ≥100,000/mm3 and ≥3 enlarged areas
  • C: Hemoglobin <10 g/dL and/or Platelets <100,000/mm3 and any number of enlarged areas
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21
Q

Laboratory hallmarks of TLS:

A
  • High potassium
  • High uric acid
  • High phosphorous
  • Low calcium
  • High LDH
22
Q

Consider TLS prophylaxis for patients with the following risk factors:

A
  • Patients receiving treatment with venetoclax, CIT, lenalidomide, and obinutuzumab
  • Progressive disease after small-molecule inhibitor therapy
  • Bulky lymph nodes
  • Spontaneous TLS
  • Elevated white blood cell (WBC) count
  • Pre-existing elevated uric acid
  • Renal disease or renal involvement by tumor
23
Q

Centerpiece of treatment includes:

A
  • Rigorous hydration
  • Management of hyperuricemia
  • Frequent monitoring of electrolytes and aggressive correction (essential)
24
Q

First-line and at retreatment for hyperuricemia

A

Low-Risk Disease:
Allopurinol or febuxostat beginning 2–3 days prior to CIT and continued for 10–14 days

Intermediate-Risk Disease (Stage I/II and LDH <2X ULN):
Allopurinol or febuxostat
OR
Rasburicase if renal dysfunction and uric acid, potassium, and/or phosphate >ULN

High-Risk Disease (Stage III/IV and/or LDH ≥2 X ULN):
Rasburicase

25
Q

Treatment for Autoimmune Cytopenias

A
  • Corticosteroids
  • Rituximab
  • IVIG
  • Cyclosporin A
  • Splenectomy
  • Eltrombopag, or romiplostim (ITP), or
  • BTKi-based therapy for steroid-refractory or recurrent AIHA
26
Q

Patients with CLL/SLL have a higher risk of developing secondary cancers, including:

A

Melanoma and non-melanoma skin cancers

Annual dermatologic skin screening is recommended.

27
Q

Recommended for prevention of thromboembolic events in patients receiving lenalidomide:

A

Aspirin 81 mg PO daily if platelets above 50 x 1012/L

28
Q

Reaction seen on those taking Lenalidomide

Painful lymph node enlargement or lymph node enlargement with evidence of local inflammation, occurring with treatment initiation; may also be associated with spleen enlargement, low-grade fever, and/or rash

A

Tumor Flare Reactions

29
Q

Treatment for Tumor Flare Reactions

A
  • Steroids (eg, prednisone 25–50 mg PO daily for 5–10 days)
  • Antihistamines for rash and pruritus

Prophylaxis:
* Consider in patients with bulky lymph nodes (>5 cm)
* Steroids (eg, prednisone 20 mg PO daily for 5–7 days followed by rapid taper over 5–7 days)

30
Q

BTKi interruption before surgery

Minor surgical procedure:
Major surgical procedure:

A

Minor surgical procedure: 3 days before
Major surgical procedure: 7 days before

31
Q

Preferred Regimens for CLL/SLL Without del(17p)/TP53 Mutation

A
  • Acalabrutinib ± obinutuzumab (category1)
  • Venetoclax + obinutuzumab (category 1)
  • Zanubrutinib (category 1)

OTHER
* Ibrutinib (category 1)
* Ibrutinib + obinutuzumab (category 2B)
* Ibrutinib + rituximab (category 2B)
* Ibrutinib + venetoclax (category 2B)

Based on the results of the phase III randomized studies (ELEVATE-TN, SEQUOIA, and CLL14)

Ibrutinib under “other recommended regimens” is based on the toxicity profile.

32
Q

Considered regimen for IGHV-mutated CLL in patients aged < 65 y without significant comorbidities

Without del(17p)/TP53 Mutation

A

FCR (fludarabine, cyclophosphamide, rituximab)

33
Q

Considered regimens when BTKi and venetoclax are not available or contraindicated or rapid disease debulking needed

Without del(17p)/TP53 Mutation

A
  • Bendamustine + anti-CD20 mAb
  • Obinutuzumab ± chlorambucil
  • High-dose methylprednisolone (HDMP) + anti-CD20 mAb (category 2B; category 3 for patients <65 y without significant comorbidities)
34
Q

A non-covalent (reversible) BTKi used for resistance or intolerance to prior covalent BTKi therapy

A

Pirtobrutinib

Covalent BTK Inhibitors: Acalabrutinib, ibrutinib, and zanubrutinib

35
Q

Chimeric antigen receptor (CAR) T-cell therapy for R/R CLL after prior BTKi and Venetoclax-based regimens

A

Lisocabtagene maraleucel (CD19-directed)

36
Q

Preferred Regimens for CLL/SLL With del(17p)/TP53 Mutation

A
  • Acalabrutinib ± obinutuzumab
  • Venetoclax + obinutuzumab
  • Zanubrutinib

CIT is not recommended since del(17p)/TP53 mutation is associated with low response rates.

Ibrutinib under “other recommended regimens” is based on the toxicity profile.

37
Q

Considered regimens when BTKi and venetoclax are not available or contraindicated or rapid disease debulking needed

With del(17p)/TP53 Mutation

A
  • HDMP + anti-CD20 mAb
  • Obinutuzumab

CIT is not recommended since del(17p)/TP53 mutation is associated with low response rates.

38
Q

TRUE OR FALSE

A baseline cardiovascular risk assessment should be considered prior to initiation of covalent BTKi.

A

TRUE

A baseline cardiovascular risk assessment should be considered prior to initiation of covalent BTKi.

39
Q

TLS Risk with Venetoclax

A

Low: All lymph nodes <5 cm AND Absolute lymphocyte count (ALC) <25 x 109/L

Medium: Any lymph node 5 cm to <10 cm OR ALC ≥25 x 109/L

High: Any lymph node ≥10 cm OR ALC ≥25 x 109/L AND any lymph node ≥5 cm

For patients with CrCl <80 mL/min and medium tumor burden, consider management as high risk for TLS.

40
Q

Prophylaxis for TLS with Venetoclax Use

A
  • Low: Oral hydration (1.5–2 L); Allopurinol
  • Medium: Oral hydration (1.5–2 L) and consider additional intravenous hydration; Allopurinol
  • High: * Oral hydration (1.5–2 L) and intravenous hydration (150–200 mL/h as tolerated); Allopurinol or febuxostat; Consider rasburicase if baseline uric acid is elevated

Start allopurinol or xanthine oxidase inhibitor 2 to 3 days prior to initiation of venetoclax.

For patients at risk of TLS, monitor blood chemistries at 6–8 hours and at 24 hours at each subsequent ramp-up dose.

41
Q

TRUE OR FALSE

“Accelerated CLL,” “CLL with expanded proliferation centers,” and CLL/PL (defined on HT-1) are not considered Richter transformation, but are associated with more aggressive disease and poorer outcome.

A

TRUE

“Accelerated CLL,” “CLL with expanded proliferation centers,” and CLL/PL (defined on HT-1) are not considered Richter transformation, but are associated with more aggressive disease and poorer outcome.

42
Q

Suggested CIT regimens for RT to DLBCL

A
  • Dose-adjusted EPOCH-R
  • HyperCVAD
  • OFAR (oxaliplatin, fludarabine, cytarabine, rituximab)
  • RCHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone)
  • Venetoclax + RCHOP (category 2B)

Generally managed with treatment regimens recommended for DLBCL

However, these regimens typically result in poor responses and optimal first-line therapy is not established

43
Q

__________ gene usage is associated with poor outcomes regardless of the IGHV mutation status

A

VH3-21

44
Q

Most common cytogenetic abnormalities

A
  • Del(13q) (55%)
  • del(11q) (18%)
  • trisomy 12 (16%)
  • del(17p) (7%)
  • del(6q) (7%)

Del(13q) as a sole abnormality was associated with favorable prognosis and the longest median survival (133 months) after chemoimmunotherapy.

45
Q

Prognostic tool that is based on the cytogenetic abnormalities detected by FISH, IGHV mutation status, and CD38 expression.

A

Integrated CLL Scoring System (ICSS)

46
Q

Prognostic tool based on TP53 and IGHV mutation status, serum beta-2 microglobulin concentration, clinical stage, and age

A

International Prognostic Index for CLL (CLL-IPI)

47
Q

Prognostic tool that predicts the likelihood of disease progression to need treatment in patients with early-stage CLL and stratifies patients with early-stage CLL into three risk groups with significantly different TTFT

A

International Prognostic Score for Early-Stage CLL (IPS-E)

48
Q

TRUE OR FALSE

Venetoclax-based combination regimens offer a fixed-duration treatment with a treatment-free remission period.

A

TRUE

Venetoclax-based combination regimens offer a fixed-duration treatment with a treatment-free remission period.

Fixed-duration treatment with venetoclax-based combination regimens also results in higher rates of uMRD, which is an independent predictor of improved survival.

49
Q

Clinical trials of CLL

A
  • ELEVATE-TN trial: acalabrutinib ± obinutuzumab
  • CLL14 study: Venetoclax + Obinutuzumab
  • SEQUOIA: Zanubrutinib
  • RESONATE-2: Ibrutinib
  • iLLUMINATE: Ibrutinib + obinutuzumab
  • E1912 and FLAIR: Ibrutinib + rituximab
  • CAPTIVATE, CLARITY: Ibrutinib + venetoclax
  • BRUIN: Pirtobrutinib
  • MURANO: VenR
  • TRANSCEND CLL 004: Lisocabtagene maraleucel
50
Q

RESPONSE DEFINITIONS AFTER TREATMENT FOR CLL/SLL

CR

A
  • LN: None ≥1.5 cm in longest dimension
  • Spleen size <13 cm; liver size normal
  • Constitutional symptoms: none
  • Circulating lymphocyte count: Normal
  • Platelet count: ≥100,000/μL
  • Hemoglobin: ≥11 g/dL (untransfused and without erythropoietin)
  • Marrow: Normocellular, no CLL cells, no B-lymphoid nodules
  • Neutrophils without growth factors: ≥1500/μL
51
Q

Features associated with TTO in patients with CLL

A
  • High WBC count
  • mutated IGHV
  • Positive DAT
  • ZAP70 positivity

NCCN (13 decks)

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