HODGKIN'S LYMPHOMA
 
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HODGKIN'S LYMPHOMA

 

DEFINITION:

A heterogeneous group of solid malignant lymphoid tumors characterized by painless lymphadenopathy and systemic manifestations.

EPIDEMIOLOGY:

  • incidence: 0.6/100,000 children <16 years of age
    • lymphomas 3rd most common form of childhood cancer (HL: 5%)
  • age of onset:
    • rarely before 5 years of age
    • bimodal: 15-34 years then 50-70 years
  • risk factors:
    • M > F (2:1) overall but (5:1) when <10 years of age
    • viral infections
      • Brucella
      • diphtheroids
      • EBV
      • mycobacteria
    • immunodeficiencies
      • ataxia telangiectasia
      • inheritied hypogammaglobulinemia
    • familial
      • increased risk in close relatives
      • increased risk in consanguineous marriages
      • increased risk with specific HLA antigens

PATHOGENESIS:

  • it appears that to develop Hodgkin's Disease requires some degree of genetic susceptibility (i.e., immunodeficiency, specific HLA antigens) and then exposure to specific environmental agents (i.e., viruses)
  • contiguity theory of spread:
    • neoplastic transformation in lymph node -> spread to other lymph nodes in group -> nodal spread to other distinct groups of lymph nodes via the lymphatics and/or early direct spread into adjacent organs (i.e., spleen) -> late blood-borne dissemination to extranodal structures (i.e., liver, bone marrow, lungs and bone)
    • extranodal primary sites occur in <1% of cases
  • 80% cure rate

CLINICAL FEATURES:

1. Painless Lymphadenopathy

  • initial presentation in over 90% of children
  • usually present with painless, enlarged lymph node(s) in the cervical or supraclavicular regions
  • occasionally axillary or inguinal nodes primary site
  • node(s) - firm, nontender, discrete with no evidence of local inflammation, may increase or decrease in size, single or matted, rubbery

2. Systemic Manifestations

  • in 30% of cases
    • anorexia
    • fatigue
    • fever
    • lethargy
    • night sweats
    • weight loss (>10% of body weight)

3. Extranodal Manifestations

1. Mediastinal Lymphadenopathy

  • may produce a nonproductive chronic cough, wheeze, dyspnea, and/or respiratory distress

2. Liver

  • hepatomegaly/hepatosplenomegaly
  • intrahepatic biliary obstruction - jaundice

3. Bone Marrow

  • anemia, neutropenia, thrombocytopenia, chronic ITP
  • recurrent infections - varicella-zoster, fungal

4. Extradural

  • spinal cord compression

INVESTIGATIONS (Diagnosis & Staging):

1. Pathology (Diagnosis)

1. Cell Types in Affected Nodes (2):

1. Malignant Hodgkin's Cells

  • include Reed-Sternberg cell and its mononuclear variants (lacunar, lymphoctyic, histiocytic, or pleomorphic cell types)
  • usually comprise <1% of involved tissue and occur singly or in small clusters
  • cell lineage unknown

2. Stromal Cells

  • include lymphocytes, histiocytes, eosinophils, monocytes, PMN leukocytes, fibroblasts
  • surround Hodgkin's Cells and by invasion and proliferation are responsible for the destruction of the normal lymph node architecture

2. Classical Histological Appearances (4):

  • classified according to degree of fibrosis, type of stroma cells, and progressive stage of the disease:

1. Lymphocyte Predominant (10-20%)

  • paucity of Hodgkin's cells surrounded by mature lymphocytes
  • over 50% may actually be benign Non-Hodgkin's Lymphoma

2. Nodular Sclerosing (50-70%)

  • most common form - 50% children; 70% adolescents
  • lacunar cells surrounded by broad bands of collagen dividing the affected lymph node into nodular cellular cellular areas

3. Mixed Cellularity (40-50%)

  • 2nd most common form
  • Reed-Sternberg cells plus variants plus mixed stromal cells

4. Lymphocytic Depletion (<10%)

  • least common and least favourable form of disease
  • Reed-Sternberg cells plus variants plus few lymphocytes
  • majority may actually be aggressive NHL

2. Staging (define extent of disease)

1. Serum

  • CBC - anemia, thrombocytopenia, leukopenia
    • also neutrophilic leukocytosis, lymphopenia, eosinophilia, monocytosis
  • ESR, copper, ferritin - markers of response
  • liver function tests, electrolytes, calcium

2. Imaging Studies

  • chest x-ray - may show diffuse fluffy exudates
  • CT/MRI of abdomen and chest
  • bipedal lymphangiography (contraindicated if massive mediastinal lymphadenopathy or involvement of pulmonary parenchyma)
  • bone scan

3. Bilateral Bone Marrow Biopsies

  • will confirm if disease is disseminated

4. Staging Laparotomy

  • to assess extent of intra-abdominal disease
    • sample all node groups and suspicious nodes
    • remove abnormal nodes
    • biopsy liver and/or bone marrow (wedge or multiple)
    • total splenectomy unless disseminated disease
    • oophopexy to move ovaries out of potential radiation field
  • will assign a pathological stage which, in 30% of cases, is different from the clinical stage
  • indicated for patients likely to receive radiation alone
  • contraindicated for patients with disseminated disease

STAGING (Ann Arbor Staging System)

Stage I: single lymph node region

  • single extralymphatic organ or site

 

Stage II: two or more lymphoid regions on same side of diaphragm

  • localized involvement of an extralymphatic organ or site and of one or more lymph node regions on the same side of diaphragm

 

Stage III: lymph node regions on both sides of the diaphragm

  • plus localized involvement of extralymphatic organ or site
  • plus localized involvement of the spleen
  • plus localized involvement of the upper abdomen (spleen, splenic, celiac and/or portal nodes)
  • plus all other intra-abdominal nodes with or without upper abdominal involvement (mesenteric, para-aortic, iliac nodes)

 

Stage IV: diffuse or disseminated involvement of one or more extralymphatic organs or tissues with or without associated lymph node enlargement

 

  • A: no systemic symptoms
  • B: with systemic symptoms

    • MANAGEMENT:

    1. Radiotherapy

    • can cure localized disease
    • involved field
      • radiation to localized area of affected nodes
    • extended field
      • mantle field - supradiaphragmatic nodes
      • spade field - upper abdominal nodes & splenic pedicle
      • final field - pelvic nodes
    • disadvantages
      • needs staging laparotomy with splenectomy
      • inhibits skeletal growth in young children
      • decreased lung capacity, cardiac involvement, hypothyroidism, sterility, premature menopause, short stature, inter-feres with breast bud development

    2. Chemotherapy

    • types
      • MOPP - mechlorethamine, oncovin, procarbazine, prednisone
      • ABVD - adriamycin, bleomycin, vinblastine, dacarbazine
    • disavantages
      • late and/or local relapses
      • long-term side effects

    3. Combined Chemo- and Radiotherapy

    • advantages
      • decreases likelihood of local recurrence
      • can use lower doses of radiation
      • can be used in the early and advanced stages of disease
      • no need for staging laparotomy

    4. Post Splenectomy

    • pneumococcal vaccines
    • long-term prophylactic penicillin

    5. Prognosis

    • 90% achieve initial remission
      • Stage I + II (>75% cure rate)
      • Stage III (75% cure rate)
      • Stage IV (50% cure rate)

    6. Relapse

    • over 2/3rds recur within 2 years of diagnosis
    • 2/3rds have lymph node involvement and 1/3rd extranodal
    • careful follow-up required every 3momths for 2 years

     

     

     

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