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4. HDS Multisystem > Lymphoma > Flashcards

Lymphoma Flashcards

1
Q

Lymphoma

Overview

A

Tumors of malignant lymphoid cells

  • Two types:
    • Non-Hodgkin’s Lymphoma (NHL)
    • Hodgkin’s Lymphoma (HL)
  • Categorized by location:
    • Nodal ⇒ Lymph nodes
      • Present as enlarged non-tender lymph nodes (>2 cm)
    • Extra-nodal ⇒ Non-lymphoid organs including bone marrow
      • Present w/ symptoms related to the site of involvement
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2
Q

Non-Hodgkin’s Lymphoma (NHL)

Clinical Presentation

A
  • Lymphadenopathy and/or organomegaly
  • “B” symptoms ⇒ systemic
    • Weight loss, fevers, night sweats
  • ↑ lactate dehydrogenase (LDH)
    • High grade, advanced stage
  • Monoclonal proteins in serum and/or urine
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3
Q

Non-Hodgkin’s Lymphoma (NHL)

Subtypes

A
  • B-cell ⇒ 90%
    • Diffuse Large B-Cell Lymphoma ⇒ 31%
    • Follicular Lymphoma ⇒ 22%
    • Marginal Zone Lymphoma ⇒ 8%
    • Small Lymphocytic Lymphoma ⇒ 7%
    • Mantle Cell Lymphoma ⇒ 6%
  • T/NK-cell ⇒ 10%
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4
Q

NHL Subtypes

B-Cell Stages

A
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5
Q

B-Cell Carcinomas

Immunophenotypic & Histological Dx

A
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6
Q

Non-Hodgkin’s Lymphoma (NHL)

Immunophenotype Algorithm

A
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7
Q

Non-Hodgkin’s Lymphoma (NHL)

Grading

A

Grading is according to clinical behavior:

  • Low grade ⇒ Survival is 5 to 7 years
  • Intermediate grade ⇒ Survival is 1 to 3 years
  • High grade ⇒ Survival is several months to 1 year
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8
Q

Non-Hodgkin’s Lymphoma (NHL)

Staging

A

Staging is the same as for HL:

Lugano Staging

The higher the stage, the worse the survival

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9
Q

Small Lymphocytic Lymphoma/Chronic Lymphocytic Leukemia (SLL/CLL)

Overview

A

Lymphoma / leukemia composed of small clonal B-cells

  • M > F
  • Age > 50 yrs
  • Considered low grade, but incurable
  • SLL and CLL are morphologically, phenotypically and genotypically indistinguishable
    • Differ only in degree of peripheral blood lymphocytosis
    • > 5K peripheral WBC ⇒ CLL
  • SLL is widespread at presentation
  • SLL is 7% of NHL
  • CLL is the most common leukemia in western world
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10
Q

SLL/CLL

Diagnostic Studies

A
  • Biopsy:
    • Mature B-cells are small, round, low grade, with soccer ball appearance
    • Smudge cells are pathognomonic ⇒ remnants of cells that lack any identifiable cytoplasmic membrane or nuclear structure
  • Flow cytometry:
    • ⊕ for CD5, CD19, CD20, CD23, kappa light chain
    • ⊖ Cyclin D1
  • FISH: Negative
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11
Q

Mantle Cell Lymphoma (MCL)

Overview

A

Lymphoma of intermediate-sized clonal B-cells

  • M > F
  • Age > 60 years
  • Often disseminated at presentation
  • Bone marrow involvement ± leukemic phase
  • Intermediate to poor prognosis
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12
Q

MCL

Translocation

A

t(11;14) (q13;q32) is characteristic of MCL

Juxtaposes cyclin D1 gene on 11q13 next to Ig heavy chain gene on 14q32 ⇒ over-expression of cyclin D1 (cell cycle regulatory protein)

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13
Q

MCL

Diagnostic Studies

A
  • Biopsy:
    • Intermediate sized B-cells found in the mantle zone
  • Phenotype:
    • ⊕ for CD5, CD19, CD20, lambda light chain, cyclin D1
    • ⊖ CD23
  • Cytogenetics:
    • t(11;14) (q13;q32) is characteristic of MCL
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14
Q

Follicular Lymphoma

Overview

A

Lymphoma composed of neoplastic cells that resemble the normal germinal center B-cells

  • F > M
  • Age > 50
  • Mimics the follicular architecture of normal lymphoid tissues
  • Frequently presents with diffuse lymphadenopathy
  • Frequently involves bone marrow ± leukemic phase
  • Low-grade
  • Long survival (70%) but rarely curable
  • t(14;18) (q32;q21) is characteristic of FL
    • Bcl-2 @ 18q21 → IgH @ 14q32 ⇒ Bcl-2 over-expression
  • Minority of cases have a rearrangement of 3q27 (Bcl-6)
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15
Q

Follicular Lymphoma

Diagnostic Studies

A
  • LN follicular structure similar to normal without lighter center
  • ⊕ CD10, CD19, CD20, kappa light chain
  • ⊕ Bcl-2 / Bcl-6
  • t(14;18)(q32;q21)
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16
Q

MALT Lymphoma

Overview

A

Extra-Nodal Marginal Zone Lymphoma

  • F > M, Median age 60
  • Associate with epithelial sites:
    • Stomach - most common
    • Small intestine, colon and rectum
    • Ocular adnexa
    • Skin
    • Thyroid
    • Lung
  • Some are associated with infections:
    • Gastric ⇒ H. Pylori
    • Ocular ⇒ Chlamydia psittaci
    • Small intestine ⇒ Campylobacter jejuni
    • Cutaneous ⇒ Borrelia burgdorferi
  • Some are associated with genetic translocations
    • Clonal IgH rearrangement
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17
Q

MALT Lymphoma

Diagnostic Studies

A

⊕ CD20

⊖ for CD10, Bcl-6

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18
Q

Lymphoma

Infectious Agents

A
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19
Q

Burkitt Lymphoma

Overview

A

Lymphoma composed of intermediate-sized immature B-cells.

  • High mitotic rate
  • Numerous admixed MΦ
  • High-grade
  • Poor survival if not treated aggressively
  • Curable if treated with high-dose regimens
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20
Q

Burkitt Lymphoma

Translocations

A

Translocations involving cMYC locus on 8q24 are characteristic of BL:

t(8;14) (q24;q32) ⇒ MYC/IGH

t(8;22) (q24;q11) ⇒ MYC/LAMBDA

t(2:8) (p11;q24) ⇒ MYC/KAPPA

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21
Q

Burkitt Lymphoma

Diagnostic Studies

A
  • ⊕ Ki-67 (100%)
  • ⊕ for CD10, CD19, CD20, kappa light chain
  • ⊕ Bcl-6
  • ⊖ Bcl-2
  • t(8;14) (q24;q32) ⇒ most common
  • Starry sky appearance on histology
  • ± EBERs (EBV-encoded RNAs)
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22
Q

Endemic

Burkitt Lymphoma

A
  • Children in equatorial Africa and New Guinea
  • Head and neck tumors
  • ⊕ EBV (100%)
  • Common locations:
    • Jaw, facial bones (50%)
    • CNS
    • Distal ileum/cecum, omentum, gonads, kidneys, long bones, thyroid, salivary glands, breasts, ± bone marrow
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23
Q

Sporadic

Burkitt Lymphoma

A
  • Mostly children and young adults, but also elderly
  • ⊕ EBV (30% cases)
  • Common locations:
    • CNS
    • Distal ileum/cecum, abdomen, gonads, kidneys, breasts, LN, ± bone marrow ± leukemic phase
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24
Q

Immunodeficiency-associated

Burkitt Lymphoma

A
  • HIV ⊕ patients
    • CD4 counts may still be high
  • ⊕ EBV (30-40% cases)
  • Common locations:
    • CNS
    • LN, bone marrow ± leukemic phase
25
Q

Diffuse Large B-Cell Lymphoma

Overview

A
  • Most common type of NHL
    • M > F
    • Age > 50, but can occur at all ages
  • Often presents as isolated mass
  • Extranodal sites are common
  • Many have BCL6 (3q27) rearrangements
  • Intermediate grade
  • Higher cure rate than low grade lymphomas
26
Q

Diffuse Large B-Cell Lymphoma

Diagnostic Studies

A
  • Large B-cells with cytoplasmic rim
  • ⊕ for CD20, Bcl-2, Bcl-6, Ki67 (50%)
  • t(3;14)(q27;q32)
27
Q

Mycosis Fungoides

A
  • Most common type of cutaneous T-cell lymphoma
  • ⊕ for CD2, CD3, CD4, CD5, CD7, CD8
  • Uncommon subtype: < 1% NHL
  • M > F, Adults / elderly
    • Black males have higher risk
  • Indolent course with slow progression
    • Patches → plaques → nodular lesions in skin
  • Associated w/ Sezary syndrome
28
Q

Sezary Syndrome

A
  • Erythroderma
  • Generalized lymphadenopathy
  • Clonally related neoplastic T-cells in the skin, lymph nodes and peripheral blood ⇒ called Sezary cells
  • Oncology equivalent of a burn
29
Q

Anaplastic Large Cell Lymphoma, ALK ⊕

Overview

A

T-cell lymphoma with variable morphology

  • M > F
  • Predilection for young patients (< 30 yrs)
  • Most common pediatric T-cell lymphoma
  • Lymph nodes and extranodal sites (skin is frequent)
  • Good prognosis compared to other T-cell NHL or diffuse large B-cell lymphoma
30
Q

Anaplastic Large Cell Lymphoma (ALCL), ALK ⊕

Genetics

A
  • Rearrangements of ALK gene on 2p23 are characteristic of ALCL, ALK ⊕
  • Most common ⇒ t(2;5) (p23;q35)
  • ALK is a tyrosine kinase
  • Result in over-expression of a fusion tyrosine kinase that is constitutively active
31
Q

Non-Hodgkin’s Lymphoma (NHL)

Characteristic Genetic Abnormalities

A
32
Q

Non-Hodgkin’s Lymphoma (NHL)

Treatment Principles

A

Almost all lymphomas are sensitive to tx with:

  • Chemotherapy
  • Radiation therapy
  • Immune therapy (monoclonal antibodies)
  • Treatment plan often includes combination of above modalities
  • Treatment usually requires administration of several cycles given at fixed intervals
33
Q

Non-Hodgkin’s Lymphoma (NHL)

Common Tx Agents

A

A common regimen is “R-CHOP”

Cyclophosphamide, Doxorubicin, Vincristine, Prednisone, rituximab

  • Alkylators
    • Cyclophosphamide, chlorambucil
  • Anthracyclines
    • Adriamycin
  • Corticosteroids
  • Anti-metabolites
    • Methotrexate, fludarabine
  • Anti-mitotic agents
    • Vincristine, vinblastine
  • Monoclonal antibodies
    • Rituximab (anti-CD20), alemtuzumab (anti-CD52)
  • Targeted therapies:
    • Venetoclax – Bcl-2 (CLL/SLL)
    • Ibrutinib – Bruton’s TK (CLL, MCL)
    • Idealisib – PI3K (CLL)
    • Tazametostat – EZH2 (FL)
    • Selinexor – XPO1 (DLBCL)
    • Tafasitamab – CD19 (DLBCL)
34
Q

Rituximab

MOA

A
35
Q

Non-Hodgkin’s Lymphoma (NHL)

Prognosis

A
  • Higher grade lymphomas:
    • Require more aggressive therapy
    • Are more likely to be cured than lower grade lymphomas
  • Higher incidence of relapse after initial successful treatment of low-grade lymphomas
  • Using optimal combos of tx modalities:
    • Many patients with NHL can be cured
    • Almost all can experience long-lasting meaningful remissions
  • Immediate and long-term side effects are comparable to HL
36
Q

Hodgkin’s Lymphoma (HL)

Epidemiology

A
  • 30% of all lymphomas
  • 0.7% of all new cancers
  • ~ 8,500 new cases/year (US)
  • Bi-modal age distribution
    • First peak 15-35 years
    • Second peak > 55 years
  • Male : Female = 2 : 1
37
Q

Hodgkin’s Lymphoma (HL)

Characteristics

A
  • Group of lymphoid malignancies
  • Neoplastic giant cells are the minority (1-5%) of the tumor and are referred to as Reed Sternberg cells
  • Non-neoplastic inflammatory cells are the majority (95-99%) of the tumor
38
Q

Hodgkin’s Lymphoma (HL)

Clinical Features

A
  • Adenopathy without other symptoms
  • Spreads first to anatomically contiguous nodes
    • Painless
  • Can be associated with splenomegaly and hepatomegaly
  • Nodal pain with EtOH intake
  • Constitutional symptoms are associated with higher stage disease and certain subtypes:
    • Fever ± Night Sweats
    • Weight loss, anorexia
    • Fatigue
    • Intense pruritus (25%)
    • Cutaneous anergy is present in most patients
      • Arises in a single node or chain of nodes
        • Cervical 60-70%
        • Axillary 6-12%
        • Mediastinal 6-11%
39
Q

Hodgkin’s Lymphoma (HL)

Lab Features

A
  • CBC:
    • Normochromic, normocytic anemia
    • Neutrophilia
    • Eosinophilia
    • Lymphopenia with loss of cell-mediated immunity
  • Erythrocyte sedimentation rate (ESR) often ↑
  • LDH occasionally ↑ (less so than NHL)
40
Q

Hodgkin’s Lymphoma (HL)

Subtype Classification

A
  • Classical (95% of cases)
    • Characterized by the Reed-Sternberg cell and variants
    • Morphological Subtypes:
      • Nodular sclerosis
      • Mixed cellularity
      • Lymphocyte-rich
      • Lymphocyte-depleted
  • Nodular Lymphocyte Predominant (5% of cases)
    • Characterized by L&H cell
    • Morphological Patterns:
      • Nodular
      • Nodular with diffuse areas
41
Q

Classical Hodgkin’s Lymphoma (CHL)

Cytology

A
  • Reed-Sternberg cell
    • Large: 45 microns
    • Single nucleus ± multiple lobes OR multinucleated
    • Large, eosinophilic, inclusion-like nucleolus
    • Abundant cytoplasm
  • Variants
    • Mononuclear
    • Lacunar
42
Q

Reed-Sternberg Cells

Immunophenotype

A

⊕ for CD15, CD30, PAX5

± EBV

⊖ for CD3, CD 20, CD45

⊖ EMA (Epithelial membrane antigen)

43
Q

Nodular Sclerosis Type

Classical Hodgkin’s Lymphoma (CHL)

A
  • Most common subtype: 70% of cases
  • Males = Females
  • Frequently adolescents and young adults
  • Propensity to involve mediastinal (80%), supraclavicular, cervical lymph nodes
  • EBV ⊕ in 10-40% of cases
  • Low stage at presentation
  • Systemic symptoms (40%)
  • Excellent prognosis
44
Q

Mixed Cellularity Type

Classical Hodgkin’s Lymphoma (CHL)

A
  • Second most common type: 25% of cases
  • Males > Females
  • Older age
  • Peripheral lymph nodes
  • Systemic symptoms are common
  • Advanced tumor stage at presentation
  • EBV ⊕ in 75% cases
  • Good prognosis
45
Q

Role of EBV

Classical Hodgkin’s Lymphoma (CHL)

A
  • EBV plays an etiologic role in HL
  • Most frequent in: extremes of life, developing countries, HIV infection, cervical nodes
  • EBV can be demonstrated to be present in R-S cells and is clonal
  • EBV DNA is the same in all cells of the tumor
  • Type 2 pattern of latency (immuno-stains for viral encoded proteins)
    • EBNA1 ⊕, LMP1 ⊕
    • EBNA2 ⊖, EBNA3 ⊖
46
Q

Lymphocyte-rich Type

Classical Hodgkin’s Lymphoma (CHL)

Overview

A
  • Rare: 5% of cases
  • Male > Female
  • Older patients
  • Peripheral nodes commonly involved
  • Low stage at presentation
  • EBV ⊕ in 40%
  • Good to excellent prognosis
47
Q

Lymphocyte-rich Type

Classical Hodgkin’s Lymphoma (CHL)

Histology

A
  • Vague nodularity
  • Residual germinal center elements
  • Diagnostic Reed-Sternberg cells and mononuclear variants
  • Nodules contain mostly B-cells (CD20 ⊕) and few RS cells (CD20 ⊖)
48
Q

Lymphocyte-Depleted Type

Classical Hodgkin’s Lymphoma (CHL)

Overview

A
  • Least common form: 2% of cases
  • Males > females
  • Older patients, HIV⊕ patients
  • Advanced stage at presentation
  • Systemic symptoms are common (80%)
  • Most aggressive form of HL
49
Q

Lymphocyte-Depleted Type

Classical Hodgkin’s Lymphoma (CHL)

Histology

A
  • Few lymphocytes
  • Many Reed-Sternberg cells and pleomorphic variants
  • Background of fibrosis
  • EBV ⊕ 90% of cases, especially in HIV ⊕ patients
50
Q

Hodgkin’s Lymphoma (HL)

Spread

A

Spread of HL is predictable:

Contiguous nodes → Spleen →Liver → Bone marrow and other extranodal sites

51
Q

Hodgkin’s Lymphoma (HL)

Staging

A
  • Staging
    • Physical exam
    • CT w/ contrast
    • PET/CT
    • ± Bone marrow examination
  • Implications:
    • Prognosis
    • Therapy
52
Q

Hodgkin’s Lymphoma (HL)

Additional Designations

A
  • A: No symptoms
  • B: Weight loss, fever, drenching sweats
  • X: Bulky disease
    • > ⅓ widening of mediastinum
    • > 10 cm maximum dimension of nodal mass
  • E: Involvement of a single extra nodal site, or extension to an extranodal site contiguous or proximal to known nodal site
53
Q

Hodgkin’s Lymphoma (HL)

Criteria For “B” Symptoms

A
  • Unexplained weight loss of > 10% of body weight during the 6 months before initial staging investigation
  • Unexplained, persistent, or recurrent fever with temperatures > 38°C during the previous month
  • Recurrent drenching night sweats during the previous month
54
Q

Hodgkin’s Lymphoma (HL)

Radiation Therapy

A
  • Disease ‘volume’ has to fit within radiation field
  • Side effects from radiation vary depending on:
  • Size of radiation field (larger = more side effects)
  • Sensitive non-diseased organs exposed to radiation (gonads, thyroid, lung, heart)
55
Q

Radiation Therapy

Long-term Side Effects

A
  • Secondary Malignancies with latency of 5-10 years
    Risk continues to increase for 30 years
    • Breast (women getting radiation before age 30)
    • Lung
    • Thyroid
    • Sarcomas of soft tissue and bone
  • Cardiovascular disease (coronary arteries, pericardial, myocardial)
  • Hypothyroidism
  • Sterility if gonads in radiation field
56
Q

Hodgkin’s Lymphoma (HL)

Chemotherapy

A

Most commonly used in US: “ABVD”

Adriamycin, bleomycin, vinblastine, dacarbazine

  • Immediate side effects well-known ⇒ neutropenia, alopecia, nausea
  • Sterility can often be avoided with use of modern combination chemotherapy
  • Long-term side effects increasingly recognized
    • Cardiac (anthracyclines)
    • Secondary MDS/AML
57
Q

Hodgkin’s Lymphoma (HL)

Treatment Considerations

A
  • Both radiation and chemotherapy have limitations and side effects
  • At 15 years after dx, risk of death from late effects > risk of death from HL
  • Tx needs to be tailored to deliver to least amount of toxicity with the highest likelihood of cure
  • Risk-adapted therapy via PET-CT
  • Need to consider
    • Disease factors (stage, relapse risk)
    • Co-morbidities (existing and anticipated)
    • Patient preferences
58
Q

Hodgkin’s vs Non-Hodgkin’s Lymphoma

A

4. HDS Multisystem (43 decks)

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