Hematology and Oncology Book

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Lymphoma

Aka: Lymphoma, Lugano Classification for Staging Lymphoma, Deauville Scoring of PET-CT in Lymphoma Surveillance
  1. See Also
    1. Non-Hodgkin's Lymphoma
    2. Hodgkin's Lymphoma
    3. HIV related Lymphoma
  2. 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
  3. Pathophysiology
    1. Cell neoplasia residing in lymphoid tissue
    2. Reticuloendothelial organ infiltration
  4. 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
  5. 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
  6. 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)
  7. 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)
  8. 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]
  9. 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
  10. 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
  11. Imaging
    1. PET/CT Imaging
  12. 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
  13. Prevention: Immunizations
    1. Prevnar 13 followed 8 weeks later by Pneumovax 23
    2. HaemophilusInfluenzae 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
  14. 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
  15. References
    1. Lewis (2020) Am Fam Physician 101(1):34-41 [PubMed]

Lymphoma (C0024299)

Definition (MEDLINEPLUS)

Lymphoma is a cancer of a part of the immune system called the lymphatic system. There are many types of lymphoma. One type is called Hodgkin disease. The rest are called non-Hodgkin lymphoma.

Non-Hodgkin lymphomas begin when a type of white blood cell, called a T cell or B cell, becomes abnormal. The cell divides again and again, making more and more abnormal cells. These abnormal cells can spread to almost any other part of the body. Most of the time, doctors can't determine why a person gets non-Hodgkin lymphoma.

Non-Hodgkin lymphoma can cause many symptoms, such as

  • Swollen, painless lymph nodes in the neck, armpits or groin
  • Unexplained weight loss
  • Fever
  • Soaking night sweats
  • Coughing, trouble breathing or chest pain
  • Weakness and tiredness that don't go away
  • Pain, swelling or a feeling of fullness in the abdomen

Your doctor will perform an exam and lab tests to determine if you have lymphoma.

NIH: National Cancer Institute
Definition (NCI_CDISC) A malignant neoplasm composed of lymphocytes of B- or T/NK-cell phenotype.
Definition (NCI) A malignant (clonal) proliferation of B- lymphocytes or T- lymphocytes which involves the lymph nodes, bone marrow and/or extranodal sites. This category includes Non-Hodgkin lymphomas and Hodgkin lymphomas.
Definition (NCI_NCI-GLOSS) Cancer that begins in cells of the immune system. There are two basic categories of lymphomas. One kind is Hodgkin lymphoma, which is marked by the presence of a type of cell called the Reed-Sternberg cell. The other category is non-Hodgkin lymphomas, which includes a large, diverse group of cancers of immune system cells. Non-Hodgkin lymphomas can be further divided into cancers that have an indolent (slow-growing) course and those that have an aggressive (fast-growing) course. These subtypes behave and respond to treatment differently. Both Hodgkin and non-Hodgkin lymphomas can occur in children and adults, and prognosis and treatment depend on the stage and the type of cancer.
Definition (MSH) A general term for various neoplastic diseases of the lymphoid tissue.
Definition (CSP) malignant (clonal) proliferation of B- or T- lymphocytes which involves the lymph nodes, bone marrow and/or extranodal sites; general term for various neoplastic diseases of the lymphoid tissue.
Concepts Neoplastic Process (T191)
MSH D008223
ICD10 M9590/3, C85.9
SnomedCT 115244002, 118600007, 21964009, 188695001, 188694002, 189965002, 188704004, 189959002, 134218000, 269627002, 188676008
LNC LA15685-3
English Germinoblastic Sarcomas, Germinoblastoma, Germinoblastomas, Lymphoma, Reticulolymphosarcoma, Reticulolymphosarcomas, Sarcoma, Germinoblastic, Sarcomas, Germinoblastic, Lymphoma, Malignant, Lymphomas, Malignant, Malignant Lymphomas, Germinoblastic Sarcoma, LYMPHOMA MALIGNANT, Lymphoma malignant, Malignant lymphoma NOS, Lymphoma morphology, Malig.lymphoma NOS-unspec site, Malignant lymphoma NOS of unspecified site, Lymphoma (Hodgkin and Non-Hodgkin), Lymphoma NOS, [M]Lymphoma NOS, [M]Malignant lymphoma NOS, Malignant lymphoma - category, Malignant lymphoma (category), malignant lymphoma (diagnosis), malignant lymphoma, Malignant lymphoma, no ICD-O subtype, Lymphoma [Disease/Finding], malignant lymphomas, lymphoma malignant, lymphomas, lymphomas malignant, Lymphomatous, Malignant lymphoma NOS (disorder), [M]Reticulolymphosarcoma NOS, [M]Malignant lymphoma NOS (disorder), Malignant lymphoma NOS of unspecified site (disorder), Lymphoma morphology (morphologic abnormality), Lymphosarcoma, Malignant lymphoma, no ICD-O subtype (morphologic abnormality), Malignant lymphoma, no International Classification of Diseases for Oncology subtype (morphologic abnormality), Malignant lymphoma, no International Classification of Diseases for Oncology subtype, Lymphomas, LYMPHOMA, MALIGNANT, LYMPHOMA (HODGKIN AND NON-HODGKIN), LYMPHOMA (HODGKIN'S AND NON-HODGKIN'S), MALIGNANT LYMPHOMA, Lymphoma, NOS, LYMPHOMA, Malignant lymphoma, Lymphoma (clinical), Malignant lymphoma (clinical), Malignant lymphoma (disorder), Malignant lymphoma - category (morphologic abnormality), lymphoma, germinoblastoma, reticulolymphosarcoma, Malignant lymphoma, NOS, Malignant Lymphoma, Lymphoma (Hodgkin's and Non-Hodgkin's)
Portuguese LINFOMA MALIGNO, Linfoma mligno, Linfoma maligno NE, Linfoma maligno, Linfoma NE, Germinoblastoma, Linfoma Maligno, Linfoma, Reticulolinfossarcoma, Sarcoma Germinoblástico
Spanish LINFOMA MALIGNO, morfología: linfoma maligno, no clasificado como subtipo en CIE - O, Linfoma maligno NEOM, Linfoma NEOM, Linfoma maligno, linfoma maligno (categoría, anomalía morfológica), linfoma maligno - categoría, linfoma maligno - categoría (anomalía morfológica), linfoma maligno (categoría, anomalía morfológica) (anomalía morfológica), linfoma hodgkiniano Y/O no hodgkiniano, linfoma de localización no especificada, SAI (trastorno), linfoma, SAI (trastorno), linfoma de localización no especificada, SAI, morfología de un linfoma (anomalía morfológica), linfoma, SAI, Malignant lymphoma NOS, morfología de un linfoma, linfoma (clínico), linfoma maligno (clínico), linfoma maligno (trastorno), linfoma maligno, linfoma, morfología: linfoma maligno, no clasificado como subtipo en CIE - O (anomalía morfológica), reticulolinfosarcoma, Linfoma, Germinoblastoma, Linfoma Maligno, Reticulolinfosarcoma, Sarcoma Germinoblástico
Dutch lymfoom NAO, lymfoom maligne, maligne lymfoom NAO, maligne lymfoom, lymfoom, Germinoblastoom, Lymfoom, Lymfoom, maligne, Reticulolymfosarcoom, Sarcoom, germinoblastair
French Lymphome malin SAI, Lymphome SAI, LYMPHOME MALIGNE, Hématosarcome, Lymphome malin, Lymphome, Lymphomes
German Lymphom NNB, Lymphom boesartig, boesartiges Lymphom, boesartiges Lymphom NNB, LYMPHOM MALIGNE, Germinoblastom, Lymphom, malignes, Lymphom, Retikulolymphosarkom, Sarkom, germinoblastisches
Italian Linfoma maligno NAS, Linfoma NAS, Germinoblastoma, Linfoma maligno, Reticololinfosarcoma, Sarcoma germinoblastico, Linfoma
Japanese 悪性リンパ腫ＮＯＳ, リンパ腫ＮＯＳ, ﾘﾝﾊﾟｼｭNOS, ﾘﾝﾊﾟｼｭ, ｱｸｾｲﾘﾝﾊﾟｼｭ, ｱｸｾｲﾘﾝﾊﾟｼｭNOS, リンパ腫, 悪性リンパ腫, ゲルミノブラストーマ, リンパ腫-悪性, 細網リンパ肉腫, 胚細胞性肉腫, リンホーマ, 肉腫-胚細胞性
Swedish Lymfom
Czech lymfom, lymfom maligní, Maligní lymfom NOS, Maligní lymfom, Lymfom NOS, Lymfom, maligní lymfom
Finnish Lymfooma
Russian LIMFOMA ZLOKACHESTVENNAIA, LIMFOMA, SARKOMA GERMINOBLASTNAIA, GERMINOBLASTOMA, RETIKULOLIMFOSARKOMA, ГЕРМИНОБЛАСТОМА, ЛИМФОМА, ЛИМФОМА ЗЛОКАЧЕСТВЕННАЯ, РЕТИКУЛОЛИМФОСАРКОМА, САРКОМА ГЕРМИНОБЛАСТНАЯ
Croatian LIMFOM
Polish Immunoblastoma, Chłoniak, Mięsak immunoblastyczny
Hungarian Lymphoma, Lymphoma k.m.n., Malignus lymphoma k.m.n., Rosszindulatú lymphoma, Malignus lymphoma
Norwegian Lymfom, Malignt lymfom
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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