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4. HDS Multisystem > Lymphoma > Flashcards

Lymphoma Flashcards

(58 cards)

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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

Study These Flashcards
A
  • HIV ⊕ patients
    • CD4 counts may still be high
  • ⊕ EBV (30-40% cases)
  • Common locations:
    • CNS
    • LN, bone marrow ± leukemic phase
25
Diffuse Large B-Cell Lymphoma Overview
* **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
Diffuse Large B-Cell Lymphoma Diagnostic Studies
* Large B-cells with cytoplasmic rim * **⊕ for CD20, Bcl-2, Bcl-6, Ki67 (50%)** * **t(3;14)(q27;q32)**
27
Mycosis Fungoides
* **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
Sezary Syndrome
* 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
Anaplastic Large Cell Lymphoma, ALK ⊕ Overview
**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
Anaplastic Large Cell Lymphoma (ALCL), ALK ⊕ Genetics
* 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
Non-Hodgkin’s Lymphoma (NHL) Characteristic Genetic Abnormalities
32
Non-Hodgkin’s Lymphoma (NHL) Treatment Principles
_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
Non-Hodgkin’s Lymphoma (NHL) Common Tx Agents
**_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
Rituximab MOA
35
Non-Hodgkin’s Lymphoma (NHL) Prognosis
* _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
Hodgkin’s Lymphoma (HL) Epidemiology
* **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
Hodgkin’s Lymphoma (HL) Characteristics
* 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
Hodgkin’s Lymphoma (HL) Clinical Features
* **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
Hodgkin’s Lymphoma (HL) Lab Features
* 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
Hodgkin’s Lymphoma (HL) Subtype Classification
* **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
Classical Hodgkin’s Lymphoma (CHL) Cytology
* **Reed-Sternberg cell** * Large: 45 microns * **Single nucleus ± multiple lobes OR multinucleated** * Large, eosinophilic, inclusion-like nucleolus * Abundant cytoplasm * _Variants_ * **Mononuclear** * **Lacunar**
42
Reed-Sternberg Cells Immunophenotype
**⊕ for CD15, CD30, PAX5** **± EBV** ⊖ for CD3, CD 20, CD45 ⊖ EMA (Epithelial membrane antigen)
43
Nodular Sclerosis Type Classical Hodgkin’s Lymphoma (CHL)
* **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
Mixed Cellularity Type Classical Hodgkin’s Lymphoma (CHL)
* **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
Role of EBV Classical Hodgkin’s Lymphoma (CHL)
* **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
Lymphocyte-rich Type Classical Hodgkin’s Lymphoma (CHL) Overview
* **Rare: 5% of cases** * Male \> Female * Older patients * **Peripheral nodes commonly involved** * **Low stage at presentation** * EBV ⊕ in 40% * Good to excellent prognosis
47
Lymphocyte-rich Type Classical Hodgkin’s Lymphoma (CHL) Histology
* **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
Lymphocyte-Depleted Type Classical Hodgkin’s Lymphoma (CHL) Overview
* **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
Lymphocyte-Depleted Type Classical Hodgkin’s Lymphoma (CHL) Histology
* Few lymphocytes * **Many Reed-Sternberg cells and pleomorphic variants** * **Background of fibrosis** * **EBV ⊕ 90% of cases**, especially in HIV ⊕ patients
50
Hodgkin’s Lymphoma (HL) Spread
_Spread of HL is predictable:_ ## Footnote **Contiguous nodes → Spleen →Liver → Bone marrow and other extranodal sites**
51
Hodgkin’s Lymphoma (HL) Staging
* _Staging_ * Physical exam * CT w/ contrast * PET/CT * ± Bone marrow examination * Implications: * Prognosis * Therapy
52
Hodgkin’s Lymphoma (HL) Additional Designations
* **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
Hodgkin’s Lymphoma (HL) Criteria For "B" Symptoms
* **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
Hodgkin’s Lymphoma (HL) Radiation Therapy
* 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
Radiation Therapy Long-term Side Effects
* **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
Hodgkin’s Lymphoma (HL) Chemotherapy
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
Hodgkin’s Lymphoma (HL) Treatment Considerations
* 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
Hodgkin’s vs Non-Hodgkin’s Lymphoma

4. HDS Multisystem (43 decks)

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