Angioimmunoblastic Lymphadenopathy

Epidemiology

  • Almost all patients are adults
  • Associated with: Sjogren’s, HIV

Physiology

  • T-cell-mediated immune defect and dysregulation (with B-cell overactivity) due to unknown etiology (EBV has been found in lymphatic tissue of AILD patients)
  • Disease presents similarly in HIV patients

Pathology

  • Pathology may resemble peripheral T-cell, Lennert’s, lymphoma or giant lymph node hyperplasia (Castleman’s disease)
    • Proliferation or arborization of blood vessels within enlarged lymph nodes or lymphatic tissue
    • Effacement of normal nodal archi-tecture by polymorphous inflamma-tory cell infiltrate
    • Depletion of normal lymphocytes and germinal centers
    • Amorphous eosinophilic material in intracellular spaces
    • Lack of Reed-Sternberg cells (excludes Hodgkin’s disease)

Diagnosis

  • CXR/Chest CT Patterns
    • Diffuse interstitial infiltrates
    • Pleural effusion/pleural thickening
    • Massive hilar/mediastinal adenopathy
  • CBC
    • Aplastic anemia: may occur
    • Autoimmune Hemolytic Anemia (40% of cases)
    • Leukocytosis (with lymphocytopenia/eosinophilia)
    • Thrombocytopenia (may be autoimmune)
  • Pleural Fluid: exudate
  • Other Autoantibodies: may occur
  • Paraproteinemia
    • Polyclonal hyperglobulinemia (70% of cases)
    • Hypoglobulinemia: may occur
    • Monoclonal hyperglobulinemia (occurs late in course)
  • Skin/Liver/Bone Marrow/Lung Biopsy: usually necessary for diagnosis

Clinical Presentation

  • Pleural Effusion (see [[Pleural Effusion-Exudate]])
  • Constitutional: fever, weight loss, fatigue
  • Skin: rash, pruritus
  • Lymphadenopathy (always present)
  • Hepatosplenomegaly:
  • Lymphoma (usually intermediate or high-grade types): develops in 5-20% of cases

Treatment

  • Benign course (33% of cases): mild disease or spontaneous remission
  • Progressive course (67% of cases): poor prognosis despite treatment
    • Danazol: reported efficacy for hemolysis/ thrombocytopenia in some cases
    • Steroids/Cytotoxics: have been used
    • CSA/Interferon-alpha: have been used

Prognosis

  • Median survival: 1.5 years
  • Most deaths occur due to infection

References

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