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