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Lymphoma

Danielle Fishman

Background

  • Classically characterized by lymphadenopathy and constitutional “B” symptoms: fevers, drenching night sweats and weight loss
  • Hodgkin (10%): superficial, nodal disease with orderly spread
    • Bimodal distribution: 15-35 years and >50 years; M>F
    • CD15+, CD30+ (Reed Sternberg cells “owl eyes”)
    • Associated with EBV in immunocompromised pt
  • Non-Hodgkin (90%): diffuse, nodal and extranodal disease with noncontiguous spread
    • Average 65 years, M>F, 85-90% B-cell
    • Associated with immunodeficiency (HIV, post-transplant), autoimmune disease, infection (EBV, HTLV-1, H pylori, HCV, Borrelia, C psittacosis, Coxiella)

General Evaluation

  • History: B symptoms, pruritus (10-15% of pt with HL), history of radiation
  • Physical Exam
    • Evaluate head and neck, tonsils, axilla, testes, liver, spleen
    • Lymphadenopathy: painless, firm, fixed, >1cm
  • Lab tests:
    • CBC, CMP, LDH, uric acid, phosphorus
    • Consider HBV, HCV, HIV, EBV, Quant gold, treponemal Ab, ANA
  • Imaging:
    • CT chest, abdomen, pelvis
    • Most will eventually need PET-CT. MRI brain if neuro symptoms
  • Consider LP for NHL with high risk of CNS involvement or presence of neurological symptoms
    • Risk factors: Burkitt, lymphoblastic, testicular involvement, double/triple hit
    • Multiple LPs may be required to diagnose CNS lymphoma
  • Diagnosis requires tissue
    • Excisional lymph node biopsy: Surgical Oncology consult
    • Core biopsy: CT guided procedure consult
    • Of note, steroids may impact value of biopsy results
  • Lugano Classification: staging of lymphoma
    • One - 1 LN region or single extra lymphatic organ/site without nodal involvement
    • Two - >2 LN regions, same side of diaphragm
    • Three - LN regions on both sides of diaphragm
    • Four - Disseminated disease w/ 1+ extralymphatic organ
  • Hodgkin:
    • IPS negative prognostic calculator: albumin <4, hemoglobin <10.5, male, stage IV disease, age>45, WBC count> 15K, lymphocyte <8% of WBC count
  • Non-Hodgkin:
    • Good prognosis: Follicular, marginal zone, mycosis fungoides/Sezary syndrome
    • Poor prognosis: DLBCL – can arise from low grade lymphoma (Richter transformation); Double/Triple hit: bcl-2, bcl-6, or myc aberrations; mantle cell, Burkitt, lymphoblastic lymphoma, and anaplastic large cell lymphoma

General Management:

  • ECG and TTE to establish pre-chemotherapy cardiac function—many chemo regimens with anthracyclines
  • Daily labs: CBC, TLS, LDH
  • TLS prophylaxis: mIVF, allopurinol

Common Chemotherapy Regimens for Lymphoma:

Regimen Components Use
R-CHOP rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone NHL
R-EPOCH etoposide plus the drugs above (dosing is different) NHL (Double/Triple hit)
Hyper-CVAD cyclophosphamide, vincristine, doxorubicin, and dexamethasone NHL
HD-MTX + R high dose methotrexate + rituximab Primary CNS Lymphoma
ABVD doxorubicin, bleomycin, vinblastine, dacarbazine HL