Non-Hodgkin's Lymphoma

Aka: Non-Hodgkin's Lymphoma, Non-Hodgkin Lymphoma, B-Cell Lymphoma, T-Cell Lymphoma, Burkitt Lymphoma, Follicular Lymphoma, Diffuse Large B-Cell Lymphoma, Precursor Cell Lymphoblastic Lymphoma, Peripheral T-Cell Lymphoma, Marginal Zone B-Cell Lymphoma, Primary Cutaneous Lymphoma

advertisement

II. Epidemiology

  1. Most common of solid hematologic malignancies
  2. Incidence: 40,000 new cases/year in United States (19.7 per 100,000 persons)
  3. Prevalence: 509,065 in United States (2013)
  4. Median age of diagnosis: 67 years old (median age at death: 76 years old)
    1. Contrast with Hodgkin's Lymphoma with typically diagnosed between ages 20 to 34 years

III. Types: General

  1. Non-Hodgkin B-Cell Lymphomas
    1. Diffuse Large B Cell Lymphoma
      1. Incidence: 7.2 per 100,000
      2. Median Age Onset: 60 to 70 years old
      3. Risk Factors include AIDS and immunomodulator medications (Biologic Agents)
      4. Five Year Survival: 63%
      5. Relapse rate: 40%
    2. Follicular Lymphoma
      1. Incidence: 3.5 per 100,000
      2. Five Year Survival: 88%
    3. Marginal Zone Lymphoma
      1. Incidence: 2.2 per 100,000
      2. Five Year Survival: 90%
    4. Precursor B Cell Lymphoma
      1. Incidence: 1.5 pcer 100,000
      2. Five Year Survival: 69%
    5. Burkitt Lymphoma
      1. Incidence: 0.4 per 100,000
      2. Five Year Survival: 64%
  2. Non-Hodgkin T Cell Lymphomas (and Natural Killer CellLymphomas)
    1. Peripheral T Cell Lymphoma
      1. Incidence: 1.2 per 100,000
      2. Five Year Survival: 58%
    2. Mycosis Fungoides (Cutaneous T-Cell Lymphoma)
      1. Incidence: 0.6 per 100,000
      2. Five Year Survival: 91%

IV. Types: Primary Cutaneous Lymphoma

  1. Primary Cutaneous T-Cell Lymphoma (CTCL, 75% of cases)
    1. See Cutaneous T-Cell Lymphoma
  2. Primary Cutaneous B-cell Lymphoma (CBCL, 25% of cases)
    1. See Cutaneous B-cell Lymphoma
    2. Primary Cutaneous Follicle Center Lymphoma (PCFCL)
    3. Primary Cutaneous Diffuse Large B-Cell Lymphoma (PCDLBCL)
    4. Primary Marginal Zone Lymphoma (PCMZL)
    5. EBV-Positive Mucocutaneous Ulcer (EBVMCU)

V. Risk Factors

  1. See Lymphoma (for risk factors common to all Lymphomas)
  2. Male
  3. White race
  4. Increasing age
  5. Family History
    1. Any first degree relative: 3.1 fold increased risk
    2. Increased risk is for the particular Lymphoma subtype, that the relative experienced
  6. Exposures
    1. Breast Implants
    2. Long-term Pesticide exposure

VI. Findings: Symptoms and Signs (differentiate from Hodgkin's Lymphoma)

  1. Presence of Lymphadenopathy draining Waldeyer's ring
  2. Non-Hodgkin adenopathy occurs anywhere (contrast with supradiaphragmatic involvement in Hodgkin Lymphoma)
    1. Epitrochlear Lymph Nodes
    2. Mediastinal, abdominal and extranodal involvement
      1. Common at presentation
  3. Chest Pain (suggests lung involvement)
  4. Systemic symptoms are more common in Hodgkin's Lymphoma

VII. Diagnosis: Pathology (Lymph Node histology)

  1. See Lymphoma
  2. Diffuse small cleaved cell
  3. Poorly differentiated Lymphocytes
  4. Absence of Reed-Sternberg Cells (contrast with Hodgkin Lymphoma)

VIII. Staging

  1. See Lymphoma

IX. Management

  1. Combination Chemotherapy and Radiation Therapy
    1. Regimens vary depending on Non-Hodgkin Lymphoma subtype
  2. Chemotherapy: CHOP
    1. Cyclophosphamide
    2. Doxorubicin
    3. Vincristine
    4. Prednisone
  3. Adjunctive agents used with CHOP
    1. Rituximab (Rituxan, known as CHOP-R when used with CHOP protocol above)
      1. Rituximab is a Monoclonal Antibody that binds CD-20 Antigen on mature B Cells
    2. Bendamustine (Bendeka)
      1. Alkylating Agent
    3. Lenalidomide (Revlimid)
  4. Surveillance
    1. See Lymphoma for general surveillance protocols

X. Complications

  1. See Lymphoma for complications shared by all Lymphomas
  2. Other cancers (related to primary cancer or its treatment)
    1. See Cancer Survivor Care
    2. Myelodysplasia
    3. Acute Leukemia
    4. Thyroid Cancer
    5. Head and neck cancer
    6. Breast Cancer
    7. Bladder Cancer
    8. Lung Cancer
    9. Skin Cancer
  3. Treatment related adverse effects
    1. Cardiomyopathy
    2. Thyroid disorders
    3. Sexual Dysfunction
  4. Paraneoplastic Syndromes (rare)
    1. Dermatomyositis
    2. Polymyositis

XI. Prognosis: International Prognostic Index

  1. Background
    1. Varies by staging
    2. Overall five year survival: 69%
  2. Criteria
    1. Age >60 years
    2. Serum LDH Increased
    3. Eastern Cooperative Oncology Performance Status >=2
      1. Ambulatory but unable to carry out activities (or limited self care only or bedridden)
    4. Ann Arbor Stage III or IV Disease
      1. Multiple nodal groups on both sides of the diaphragm (may involve Spleen) OR
      2. Noncontiguous extranodal involvement
    5. Extranodal sites >1
  3. Interpretation
    1. Score 0-1: Five year survival 73%
    2. Score 2: Five year survival 51%
    3. Score 3: Five year survival 43%
    4. Score 4-5: Five year survival 26%
  4. References
    1. (1993) N Engl J Med 329(14):987-94 [PubMed]

XII. Prevention

  1. Vaccination
    1. See Lymphoma for common Vaccinations for both Hodgkin Lymphoma and Non-Hodgkin Lymphoma

XIII. Resources

  1. Non-Hodgkin Lymphoma, CSR, 1975-2016 (NIH NCI)
    1. https://seer.cancer.gov/archive/csr/1975_2015/results_merged/sect_19_nhl.pdf
  2. Cancer Facts: Non-Hodgkin Lymphoma (NIH NCI)
    1. https://seer.cancer.gov/statfacts/html/nhl.html

XIV. References

  1. Lewis (2020) Am Fam Physician 101(1):34-41 [PubMed]

Images: Related links to external sites (from Bing)

These images are a random sampling from a Bing search on the term "Non-Hodgkins Lymphoma." Click on the image (or right click) to open the source website in a new browser window. Search Bing for all related images

Related Topics in Lymphatic Disorders

advertisement