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Lymphoma

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Lymphoma, Lugano Classification for Staging Lymphoma, Deauville Scoring of PET-CT in Lymphoma Surveillance

  • See Also
  1. Non-Hodgkin's Lymphoma
  2. Hodgkin's Lymphoma
  3. HIV related Lymphoma
  • Epidemiology
  1. Incidence (U.S. 2019): 82,000 new cases (4.7% of all new U.S. cancers)
    1. Lymphoma will account for 21,000 deaths (3.5% of all cancer deaths) in 2019
  • Pathophysiology
  1. Cell neoplasia residing in Lymphoid Tissue
  2. Reticuloendothelial organ infiltration
  • Types
  1. Hodgkin's Lymphoma
    1. Localized disease with contiguous nodal spread
    2. Typically involved supradiaphragmatic nodes
    3. Mediastinal involvement
    4. Curability >75%
    5. Five Year Survival: 86%
    6. Systemic Symptoms (Fever, Night Sweats, weight loss)
  2. Non-Hodgkin's Lymphoma
    1. Non-contiguous nodal spread
    2. Extranodal involvement
    3. Abdominal and Bone Marrow involvement
    4. Curability <25%
    5. Five Year Survival: 72%
    6. Systemic symptoms may occur with more advanced disease
  • Risk Factors
  • Infection Mechanisms
  1. Direct Lymphocyte transformation
    1. Epstein-Barr Virus or Mononucleosis (Burkitt Lymphoma, Hodgkin Lymphoma)
    2. Human T-Cell LeukemiaVirus Type 1 or HTLV1 (T-Cell Lymphoma)
  2. Immunosuppression
    1. HIV related Lymphoma (Hodgkin Lymphoma and Non-Hodgkin Lymphoma)
  3. Chronic Antigenic Stimulation (Non-Hodgkin Lymphoma)
    1. Helicobacter Pylori
    2. Chlamydia psittaci
    3. Campylobacter jejuni
    4. Campylobacter coli
    5. Borrelia Burgdorferi (Lyme Disease)
    6. Hepatitis C
  • Risk Factors
  • Other
  1. Inherited Immunodeficiency Disease
    1. Klinefelter's Syndrome
    2. Chediak-Higashi Syndrome
    3. Ataxia Telangiectasia syndrome
    4. Wiskott-Aldrich Syndrome
    5. Common Variable Immunodeficiency disease
  2. Acquired Immunodeficiency
    1. Iatrogenic Immunosuppression
    2. Acquired Immunodeficiency Syndrome
    3. Acquired Hypogammaglobulinemia
  3. Autoimmune Disease
    1. Sjogren's Syndrome
    2. Celiac Sprue
    3. Rheumatoid Arthritis
    4. Systemic Lupus Erythematosus
    5. Dermatomyositis
  4. Chemical or drug exposure
    1. Phenytoin (Dilantin)
    2. Radiation Therapy
    3. Chemotherapy
    4. Tobacco Abuse
  5. Miscellaneous
    1. Obesity (BMI >30 kg/m2)
  • Symptoms
  1. See Hodgkin Lymphoma and Non-Hodgkin Lymphoma for more specific presentations
  2. Painless Adenopathy
    1. Variable progression depending on aggressiveness of Lymphoma
  3. Systemic symptoms (more common in Hodgkin Lymphoma and in more advanced Non-Hodgkin Lymphoma)
  • Staging
  • Lugano Classification (based on PET/CT)
  1. Stage I
    1. Single nodal group or single extralymphatic lesion
  2. Stage II
    1. Multiple nodal groups on same side of diaphragm OR
    2. Limited contiguous extralymphatic involvement
    3. May also be classified as bulky disease if <10 cm mass
  3. Stage III
    1. Multiple nodal groups on both sides of diaphragm (and may involve Spleen)
  4. Stage IV
    1. Non-contiguous extra-lymphatic involvement
  5. Additional modifiers in Hodgkin Lymphoma
    1. A: Systemic symptoms absent
    2. B: Systemic symptoms present
      1. Fever >101.3 F or 38.5 C or
      2. Drenching Night Sweats or
      3. Unintentional Weight Loss over 6 months >10% of body weight
  6. References
    1. Cheson (2014) J Clin Oncol 32(27): 3059-68 [PubMed]
  • Diagnosis
  1. Open Lymph Node biopsy
    1. Initial evaluation is often with FNA or core needle biopsy, but open biopsy is typically needed for diagnosis
    2. Morphology, immunohistochemistry and flow cytometry are used to define the Lymphoma
    3. Identifying Reed-Sternberg Cells differentiates Hodgkin Lymphoma from Non-Hodgkin Lymphoma
  2. Bone Marrow Biopsy
    1. Indicated in diffuse large B cell Lymphoma, when PET-CT is negative
  • Staging
  • Deauville Scoring based on PET/CT (for monitoring after Lymphoma treatment)
  1. Criteria
    1. Score 1: No FDG (fluorodeoxyglucose) uptake related to Lymphoma
    2. Score 2: FDG (fluorodeoxyglucose) uptake at Lymphoma site is less than or equal to the FDG uptake at mediastinum
    3. Score 3: FDG (fluorodeoxyglucose) uptake at Lymphoma site is greater than the FDG uptake at mediastinum
    4. Score 4: FDG (fluorodeoxyglucose) uptake at Lymphoma site is greater than the FDG uptake at any site in the liver
    5. Score 5: FDG (fluorodeoxyglucose) uptake at Lymphoma site is much greater than the FDG uptake at liver OR new FDG uptake site
  2. Interpretation
    1. Non-Hodgkin Lymphoma
      1. Score <=3: Complete Remission
      2. Score 4-5: Consider escalating therapy
    2. Hodgkin Lymphoma
      1. Score 1-2: No further treatment
      2. Score 3-5
        1. Additional Chemotherapy and possibly radiation
        2. Biopsy if Score 5, and if biopsy positive, then define as refractory disease
  • Imaging
  1. PET/CT Imaging
  • Management
  • Surveillance
  1. See Hodgkin Lymphoma and Non-Hodgkin Lymphoma for specific treatment protocols
  2. Reevaluation of Lymphoma after initial management
    1. See Deauville Scoring of PET-CT in Lymphoma Surveillance as above
  3. Surveillance
    1. Oncology every 3-6 months for first 2 years, then every 6-12 months for third year, and then yearly
    2. Imaging surveillance is optional in asymptomatic patients
      1. Chest XRay or CT Chest may be obtained every 6-12 months for first 2 years, then yearly for next 3-5 years
    3. Secondary complications from chemoradiation including secondary malignancies
      1. See Cancer Survivor Care
  1. Prevnar 13 followed 8 weeks later by Pneumovax 23
  2. Haemophilus Influenzae Type B Vaccine 3 dose series
    1. Start 6-12 months after successful Hematopoetic Stem Cell Transplant
  3. Influenza Vaccine annually (unless anti-B Cell agents such as Rituximab)
    1. Avoid Influenza Vaccine and Live Vaccines for 6 months after last anti-B agent
  4. See Asplenism for additional Vaccinations if indicated
  5. Immunize all household contacts (except Live Vaccines)
  6. Resume other Vaccinations (live and inactivated) after sufficient delay
    1. Six months after last anti-B Cell agent (e.g. Rituximab) OR
    2. Three months after other agents
  • Complications
  1. See Non-Hodgkin Lymphoma and Hodgkin Lymphoma for type specific complications
  2. Compression in high grade Lymphomas
    1. Superior Vena Cava Syndrome
    2. Malignant Epidural Spinal Cord Compression
    3. Malignant Pericardial Effusion
  • References
  1. Lewis (2020) Am Fam Physician 101(1):34-41 [PubMed]