Lymphomatoid Granulomatosis Clinical Presentation

Updated: Dec 31, 2015
  • Author: Nader Kamangar, MD, FACP, FCCP, FCCM; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Presentation

History

The clinical features of lymphomatoid granulomatosis reflect systemic multiorgan disease. Pulmonary involvement usually is present, while the skin (50%), nervous system (25%), kidneys, and liver are affected less commonly. The lymph nodes, spleen, and bone marrow usually are spared until late in the course of illness. Araki et al reported primary orbital involvement. [3]

  • Pulmonary involvement

    • Cough and dyspnea are present in most patients.

    • Sputum production may reflect associated pneumonia.

    • Hemoptysis usually indicates disease cavitation.

  • Systemic presentation of lymphoma-related B symptoms: Patients may have fever, weight loss, and malaise.

  • Skin

    • Patchy, occasionally painful, erythematous macules, papules, and plaques typically involve the gluteal regions and extremities. [4]

    • Erythema may involve nodosumlike subcutaneous nodules that may ulcerate but are often truncal.

    • Isolated cutaneous lymphomatoid granulomatosis has been reported.

  • Nervous system

    • Extensive lymphocytic infiltration of the meninges, cerebral vessels, and peripheral nerves is found in as many as 25% of patients.

    • CNS may include mass lesions. Neurological manifestations, including mental status changes, ataxia, hemiparesis, and seizures, may occur.

    • Peripheral nerve involvement may include distal sensory neuropathy or mononeuritis multiplex.

    • Isolated neurological lymphomatoid granulomatosis has been reported. [5, 6]

  • Renal

    • Clinically significant renal disease is uncommon.

    • At autopsy, renal involvement is present in 45% of cases.

    • Unlike WG, glomerulonephritis is not a feature of lymphomatoid granulomatosis.

  • Liver

    • Hepatic involvement also is frequent at autopsy (29% of cases), but clinical disease is rare.

    • Hepatomegaly is present in 12% of cases and may carry a worse prognosis.

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Causes

Other than its association with opportunistic disease and EBV, the etiology of lymphomatoid granulomatosis is unknown.

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