NON-HODGKIN'S LYMPHOMA
 
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NON-HODGKIN'S LYMPHOMA

 

DEFINITION:

A heterogeneous group of solid malignant lymphoid tumors characterized by painless lymphadenopathy, respiratory, cardiovascular, and/or gastrointestinal manifestations.

EPIDEMIOLOGY:

  • incidence: ?
    • lymphomas 3rd most common form of childhood cancer (NHL: 7-10%)
    • (behind leukemia and CNS tumors)
    • NHL:HL (3:2)
  • age of onset:
    • usually >5 years of age
  • risk factors:
    • M > F (3:1)
    • viral infections
      • EBV (African Burkitt's Lymphoma)
      • HIV (primary CNS B-cell NHL)
    • immunodeficiencies (EBV-associated NHL)
      • Bruton's Agammaglobulinemia
      • common variable immunodeficiency
      • immunosuppression following renal transplant
      • severe combined immunodeficiency
      • Wiskott-Aldrich syndrome
    • chromosomal breakage syndromes
      • ataxia telangiectasia
      • Bloom syndrome
    • chromosomal abnormalities
      • translocation of 8q
      • translocation of alpha & beta T-cell receptor genes

PATHOGENESIS:

  • proposed mechanisms:

1. Viral Infections

  • viral infection -> chronic immunostimulation -> uncontrolled proliferation of lymphoid cells -> lymphomatous transformation -> polyclonal expansion of B-cells

2. Chromosomal Abnormalities

  • translocation of 8q -> translocation of c-myc gene -> gene does not respond to usual cellular controlls -> abnormal expression of gene product -> cellular proliferation +/-neoplastic transformation (Burkitt's Lymphoma)

PATHOLOGY:

Classification (%) - Cell Types

  • Lymphoblastic (38%) - T-cell; non-T non-B; undifferentiated (small noncleaved)
  • Burkitt's (14%) - B-cell
  • Non-Burkitt's (20%) - B-cell
  • Large Cell (20%) - B-cell
  • Other (8%)

    • CLINICAL FEATURES:

    1. Generalized Disease

    • because lymphomas in children tend to be aggressive and grow rapidly:
      • short period between onset of symptoms and diagnosis (days to weeks)
      • children often present with generalized disease
    • has a propensity for extranodal involvement with early non-contiguous dissemination
      • may present with generalized disease +/- symptoms related to involvement of specific lymphoid organs

    2. Abdominal Lymphadenopathy (35%)

    • usually B-cell or nonlymphoblastic type
    • lymphoma of Peyer's Patch with involvement of ileocecal region: distal ileum, appendix and caecum

    1. Gastrointestinal Manifestations

    1. Pain

    • may mimic appendicitis with RLQ tenderness, fever, vomiting, and intestinal obstruction

    2. Ileocecal Intussusception

    • especially in those >5 years of age
    • secondary to an intraluminal lymphoma

    3. Abdominal Distension

    • due to ascites or rapidly-growing ovarian, pelvic, or retroperitoneal masses

    3. Anterior Mediastinal Lymphadenopathy (26%)

    • usually T-cell or lymphoblastic type

    1. Respiratory Manifestations

    • asymptomatic
    • 1. Tracheal Compression
      • acute airway obstruction - pain, tachypnea, chronic cough, wheeze, dyspnea, pleural effusion

    2. Cardiovascular Manifestations

    1. Superior Vena Cava Compression

    • plethora of the face and neck
    • edema of upper extremities and face
    • conjunctivitis
    • altered mental status

    4. Peripheral Lymphadenopathy (13%)

    • inguinal and axillary nodes
    • often painless

    5. Head and Neck Lymphadenopathy (13%)

    • Waldeyer's ring, nasal sinuses, cervical (most common) and supraclavicular nodes
    • often painless, nontender, firm, discrete early but confluent later, rapid increase in size (within 1-2 weeks)

    6. Extralymphoid Sites (12%)

    • usually large-cell type
      • bone
      • bone marrow (>5% but <25% blasts)
      • face
      • skin
      • testes
      • CNS - intracerebral, meninges, epidura, cranial nerves, spinal cord (extradural)

    7. Complications

    1. Tumor Lysis Syndrome

    • particularly with disseminated lymphomas (Burkitt's), abdominal presentation, and lymphoblastic
    • may present before or after initiation of therapy
    • due to disruption of tumor burden -> release of electrolytes and metabolites:
      • uric acid - nephropathy
      • phosphate - " (with calcium precipitates)
    • nephropathy -> hyperkalemia -> arrhythmias hypocalcemia -> tetany, seizures

    INVESTIGATIONS (Diagnosis & Staging):

    1. Pathology (Diagnosis)

    • biopsy of nodes and bone marrow
      • 20% of patients have bone marrow involvement at presentation
    • aspiration of ascites, bone marrow, pleural effusions
    • histopathology via specific cell-marker studies for immuno-phenotyping

    2. Staging (define extent of disease)

    1. Serum

    • CBC, AST, bilirubin
    • uric acid, LDH (for tumor burden)
    • BUN, creatinine, phosphate, calcium, electrolytes (for tumor-induced renal dysfunction)

    2. Imaging Studies

    • chest x-ray
    • abdominal ultrasound
    • CT (chest and abdomen)

    3. Staging (St. Jude Children's Research Hospital)

  • Stage I: single node or anatomic area (except mediastinal or abdominal)
  • Stage II: single extranodal tumor with regional node involvement
    • two or more nodal areas on the same side of diaphragm
    • primary GI tumor with or without associated mesenteric nodes
  • Stage III: 2 or more extranodal tumors on both sides of diaphragm
    • 2 or more nodal areas on both sides of diaphragm
    • primary thoracic tumor
    • extensive intra-abdominal disease
    • all paraspinal or epidural tumors
  • Stage IV: CNS or bone marrow involvement

    • MANAGEMENT:

    1. Localized Disease (Stages I and II):

    • specifics of treatment differ with lymphomas of T- or B-cell lineages

    1. Chemotherapy

    • therapy less than 1 year (induction + continuation)
      • induction - 7 weeks of vincristine, prednisone, doxorubicin, cyclophosphamide
      • continuation - 6 mo. of mercaptopurine & methotrexate

    2. Others

    • radiotherapy - no role
    • CNS prophylaxis - no role unless head and neck involved

    3. Survival

    • 80-90% long-term, disease-free survival

    2. Advanced and Disseminated Lymphoblastic

    1. Chemotherapy

    • multi-agent
    • therapy for 2-3 years

    2. Radiotherapy

    • occasionally need local radiotherapy

    3. CNS prophylaxis

    • intrathecal chemotherapy and/or cranial radiotherapy

    4. Survival

    • 76% long-term, disease-free survival

    3. Advanced Non-Lymphoblastic

    1. Chemotherapy

    • multi-agent
    • therapy for 6-12 months

    2. Radiotherapy

    • no role

    3. CNS prophylaxis

    • intrathecal chemotherapy and/or cranial radiotherapy

    4. Survival

    • 57-76% long-term, disease-free survival

    4. Relapse While on Therapy

    • intensive chemotherapy -> bone marrow transplant
    • lymphoblastic lymphoma tend to show relapse to extramedullary sites (testes, CNS, skin, kidneys)

    5. Tumor Lysis Syndrome

    • allopurinol
    • intense hydration
    • alkalinization of urine
    • phosphate binding gels
    • diuretics
    • dialysis

    6. Surgery

    • resection of abdominal or mediastinal masses

     

     

     

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