Lymphoma - Margaret Wheless
Background
- Malignancy of lymphoid cells comprising multiple different cancers of the immune system depending on the subtype and stage of development of cell/tissue involved
- Characterized by lymphadenopathy & constitutional “B” symptoms:
- Fevers, drenching night sweats and weight loss
- Branch point of diagnosis:
- Hodgkin: 10% - superficial, nodal disease with orderly spread
- Non-Hodgkin: 90% - diffuse, nodal and extranodal disease with noncontiguous spread
Evaluation
- B symptoms; pruritus (10-15% of pt with HL); history of radiation
- Lymphadenopathy: painless, firm, fixed, >1cm
- Exam: head & neck, tonsils, axilla, testes, liver, spleen
- CBC, CMP, LDH, Uric Acid
- HBV, HCV, HIV, EBV, Quant gold, Treponemal Ab, ANA
- Imaging: CXR most will eventually need PET-CT; MRI brain if neuro symptoms
- Diagnosis requires tissue
- Excisional Lymph node biopsy (surg onc. Consult)
- Core biopsy (CT guided procedure consult)
- Ann Arbor Staging System: I. 1 LN region
- II. >2 LN regions, same side of diaphragm
- III. LN regions on both sides of diaphragm
- IV. Dissem dz w/ 1+ extralymphatic organ
Non-Hodgkin Lymphoma (NHL)
Background
- 65 y/o, M>F, 85-90% B-cell
- Associated with immunodeficiency (HIV, post-transplant), autoimmune disease (both the disease and immunosuppression), infection (EBV, HTLV-1, H pylori, HCV, Borrelia, C
- psittacosis, Coxiella)
- Stratify into:
- Good prognosis: waxing/waning LAD, cytopenias, hepatomegaly or splenomegaly
- Poor prognosis: Aggressive presentation: fever, night sweats, weight loss, TLS
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Good Prognosis |
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Diagnosis |
Key Features |
Workup/Treatment |
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Follicular Lymphoma
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20-30% of NHL
T (14; 18) bcl-2 over expression; CD19+, 20+, 10+, CD5-, CD23-
Grading based on # large cells 1: <25%, 2: 25-50%, 3. >50% (grade 3= closer to DLBCL) |
Watchful waiting Treatment:
Excision or radiation (stage I) Bendamustine-R (B-R) or RCHOP (> I) |
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Marginal Zone Lymphoma |
Usually located in the GI, thyroid, orbit, lung, breast, salivary glands
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Treatment:
Extranodal MALT:
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Mycosis Fungoides
Sezary syndrome
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Skin plaques with atypical CD4+ cells
May progress to Sezary syndrome aka T cell leukemia |
Workup: Skin biopsy
Treatment: Light therapy, topical steroids, superficial radiation |
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Aggressive/Poor Prognosis |
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Diagnosis |
Key Features |
Workup/Treatment |
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DLBCL
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~25% of NHL; CD20+, CD45+ CD5+ confers the worst prognosis Can arise as transformation from low- grade lymphoma (Richter’s transformation)
60% present with advanced disease Risk factors for CNS disease: involvement of testicle, paranasal sinus, epidural space, bone marrow or >1 extra nodal sites + ↑LDH CNS disease needs IT and systemic MTX |
If PET/CT reveals BM disease then no need for biopsy
Tx: RCHOP vs DA-R-EPOCH (double hit)
Use IPI prognostic scoring |
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Mantle Cell Lymphoma |
7% NHL; CD20+, CD200+ T(11;14) =↑Cyclin D1 M>F; avg age ~50-60s
Most present with diffuse disease Blastoid variant more often involves CNS |
Dx: pathology from bx Use MIPI prognostic tool
Treatment: Aggressive: R-hyperCVAD-M/ara-C Poor PS: BR & Rituximab |
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Double or Triple Hit Lymphoma |
Similar to DLBCL with combination of bcl-2, bcl-6, or myc aberrations conveying double or triple hits
Very aggressive needs intrathecal ppx even if no evidence of CNS disease |
PET/CT for staging; if no evidence of BM involvement on PET, will need biopsy Tx: Da-R-EPOCH vs R hyperCVAD/MA vs R-CODOX-M/ IVAC |
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Burkitt Lymphoma |
MYC translocation - most commonly t (8;14) with path showing classic “starry-sky” Rapidly growing tumors HIV: CNS relapse is 30-50% if no CNS ppx
1. Endemic (African jaw); often EBV/CD21+ 2. Sporadic (in the US): median age 30 at dx; present w/ abdominal masses & metastasis 3. Immunodeficiency-related (often seen with CD4 counts >200); more often involve LN, CNS, and BM |
R-EPOCH vs R-CODOX-M/IVAC Everyone gets CNS prophylaxis
TLS prophylaxis for all pts
Everyone will need TTE before anthracycline-based chemo |
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Lymphoblastic Lymphoma |
Express TdT+ (cyclin D1 -) Aggressive; often present with mediastinal mass and CNS involvement Consider empiric allopurinol before starting chemotherapy |
Treated like acute leukemias
Will need LP with IT chemo |
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Anaplastic Large Cell Lymphoma (ALCL) |
< 2% of NHL Primary cutaneous variant can arise from existing mycosis fungoides and age >40 |
Use IPI prognostic tool
Treatment: check CD30 +/- status - CD30+: BV(brentuximab)+ CHP (cyclophosphamide, doxorubicin, prednisone) CD30-: CHOP + etoposide |
Hodgkin Lymphoma (HL)
Background
- Bimodal Distritbution: 15-35 y/o and >50; M>F
- CD15+, CD30+ (Reed Sternberg cells “owl eyes”)
- Associated with EBV in immunocompromised pt
- 4 classical types
- Nodular sclerosing (60-80%): young adult, F>M, low stage, mediastinal LAD, collagen bands
- Lymphocyte rich (5%): best prog
- Lymphocyte depleted (<1%): worst prognosis, older, M>F, disseminated, HIV+, diffuse positive and RS cells
- Mixed (15-30%): older, M>F, intermediate prognosis
- 1 nonclassical (5%): nodular lymphocyte predominant, peripheral LN
Presentation
- Lymphadenopathy (usually contiguous), generalized pruritus, Incidental mediastinal mass
- B-Symptoms: fevers, night sweats, weight loss
Evaluation
- IPS negative prognostic calculator: albumin <4, hemoglobin <10.5, male, stage IV by Ann Arbor, age>45, WBC count> 15K, lymphocyte <8%
Management
- Stage I-II: ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) ± RT
- Stages III-IV: ABVD x6 or escalated BEACOPP (bleo, etop, doxo, cyclophos, vinc, procarbazine, prednisone)
Additional Information
- Pts will need TTE prior to chemo given the use of anthracycline
- Increased risk of secondary cancers (~25% of pt with 40% increased risk over next 40 years)