Hodgkin's Disease

BASICS

DESCRIPTION
A malignant disease of the lymphoid tissue, caused by the transformation of an uncertain progenitor cell to the pathognomonic Reed-Sternberg cell. Disease spreads to contiguous lymphoid tissue, and eventually to non-lymphoid tissue.
  • Rye classification-based on pathologic findings (frequency by percent):
    • Lymphocyte predominant (2-10)
    • Mixed cellularity (20-40)
    • Lymphocyte depleted (2-15)
    • Nodular sclerosis (40-80)
  • System(s) affected: Hemic/Lymphatic/Immunologic
  • Genetics:
    • First degree relatives - 3 times risk
    • Siblings of younger patients - 7 times risk
  • Incidence/Prevalence in USA:
    • 3.1/100000
    • About 7200 new cases expected in 1999
    • Incidence is lower in underdeveloped countries
  • Predominant age:
    • Bimodal age distribution
      • Early peak in mid to late 20s
      • Later peak around 60-70
    • Rare under age 5
  • Predominant sex: Male > Female (1.4:1)
SIGNS AND SYMPTOMS
  • Asymptomatic lymphadenopathy (usually cervical or supraclavicular)
  • Fever (Pel-Ebstein pattern)
  • Night sweats
  • Weight loss
  • Fatigue
  • Anorexia
  • Unexplained itching
  • Alcohol-induced pain
CAUSES
  • Unknown; EB virus may play a role
RISK FACTORS
  • Immunodeficiency (inherited or acquired)
  • Autoimmune disorders
  • HIV infection

DIAGNOSIS

LABORATORY
  • CBC
  • Chemistry profile
  • ESR
  • Liver function tests
  • Renal function tests
  • HIV (if risk factors present)

Drugs that may alter lab results: Phenytoin may produce pseudolymphoma
Disorders that may alter lab results: N/A

PATHOLOGICAL FINDINGS
  • Reed-Sternberg (RS) cell
    • Abundant cytoplasm
    • Two or more nuclei or nuclear lobes, each with a prominent nucleoli
  • Background infiltrate of lymphocytes, histiocytes, granulocytes, plasma cells and fibroblasts
SPECIAL TESTS
N/A
IMAGING
  • Chest X-ray
  • Thoracic CT scan
  • Abdominal and pelvic CT scan (or possibly MR scan)
  • Lymphangiography
  • Bone scan, gallium scan, abdominal ultrasound (used infrequently)
DIAGNOSTIC PROCEDURES
  • Excisional lymph node biopsy (needle biopsy not sufficient)
  • Exploratory laparotomy with splenectomy - only if treatment will be altered by results
  • Bone marrow biopsy - especially with systemic symptoms
  • Liver biopsy (in selected cases)

TREATMENT

APPROPRIATE HEALTH CARE
  • Initial staging is critical to therapy
  • Cotswold classification
    • Stage I - single lymph node group
    • Stage II - two or more node groups on same side of diaphragm
    • Stage III - Node groups on both sides of diaphragm
    • Stage IV - dissemination involving extra-nodal organs (not the spleen which is considered lymphoid tissue)
    • Subclass designations: A = no symptoms; B = systemic symptoms (fever, night sweats, weight loss >10% body weight); X = bulky disease (widened mediastinum > 1/3 intrathoracic diameter or > 10 cm nodal mass); E = single extra-nodal site involvement in proximity with known nodal site
GENERAL MEASURES
  • Treatment aimed for cure with minimum toxicity, including treatment-induced late mortality
  • Treatment can be radiation therapy (RT), chemotherapy, or combined radiation and chemotherapy (CMT), based on stage and tumor burden
    • Stage IA, IIA, nonbulky - usually RT alone; chemotherapy for selected high risk patients
    • Stage IB, IIB, nonbulky - RT, if staging laparotomy is done: otherwise, CMT
    • Stage IIAX, IIBX (bulky disease) - CMT
    • Stage IIIA - possibly RT for carefully selected patients (minimal disease limited to upper abdomen); otherwise, CMT
    • Stage IIIB, IV - CMT
  • Autologous bone marrow transplant for selected patients who fail conventional therapy
SURGICAL MEASURES

N/A

ACTIVITY

As tolerated

DIET

No restrictions

PATIENT EDUCATION
  • Reproductive impact
    • Spermatogenesis often impaired prior to therapy
    • Gonadal side effects of therapy
    • Sperm banking option for males
    • Oophoropexy in premenopausal female if pelvic RT contemplated
  • Risks of secondary malignancy
  • Careful oral and dental care during therapy
  • Patient education material
    • Leukemia Society of America, 733 3rd Avenue, New York, NY 10017

FOLLOW UP

PREVENTION/AVOIDANCE
  • Pneumococcal vaccine, if splenectomy is planned for staging
  • Consider vaccines for Haemophilus and Neisseria species as well
POSSIBLE COMPLICATIONS
  • Secondary malignancies following therapy
  • Sterility, gonadal dysfunction
  • Hypothyroidism
  • Bone marrow suppression
  • Immunosuppressed infections, including herpes zoster
  • Anemia
  • ITP, TTP
  • Coronary artery disease, cardiomyopathy
  • Radiation pneumonitis, pulmonary fibrosis
  • Transient radiation myelopathy (Lhermitte's sign)
EXPECTED COURSE AND PROGNOSIS
  • Overall 5 year survival 82%
  • 75% long term survival
  • 10 year survival rates correlate with stage at diagnosis
    • Stage IA, IB, IIA nonbulky 85-95%
    • Stage IIA bulky, IIB 80-85%
    • Stage IIIA 75-90%
    • Stage IIIB 60-65%
    • Stage IV 55-60%
  • Unfavorable prognostic factors in advanced disease
    • Sed rate > 70
    • Age > 45
    • Male gender
    • Albumin < 4
    • Hemoglobin < 10.5
    • Lymphopenia < 600

MISCELLANEOUS

ASSOCIATED CONDITIONS
  • T lymphocyte defects, which persist after successful treatment
  • Patients with HIV tend to present with more advanced disease
AGE-RELATED FACTORS

Pediatric: Increased risk for males
Geriatric: Usually presents in more advance stage and shows unfavorable histology
Others: N/A

PREGNANCY
  • Pregnancy not known to affect course of disease
  • Hodgkin's disease not known to affect pregnancy or fetus IF therapy can be postponed until delivery
  • Normal pregnancy can occur after treatment, if fertility is maintained
  • Risk of disease progression during pregnancy is variable. Management must be individualized
OTHER NOTES

N/A

ABBREVIATIONS

N/A

Clinical Investigations

ROLE OF HOMOEOPATHY

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