54. Lymphomatoid Granulomatosis
Definition
Lymphomatoid granulomatosis (LYG) is a rare angiodestructive, lymphoproliferative disease with significant pulmonary involvement in the form of pulmonary angiitis and granulomatosis. LYG mimics Wegner’s granulomatosis (see p. 345), both in clinical and radiographic presentation.
Incidence
The frequency of LYG occurrence is not known. Males are affected more than females at a 2:1 rate.
Etiology
The true cause of LYG is not known. Recent evidence strongly suggests it is a distinct type of malignant lymphoma associated with immunosuppression. LYG is distinguished from Wegner’s granulomatosis by the involvement of B-cells.
Signs and Symptoms
Definitive diagnosis of LYG is based on the histologic triad of (1) granulomatosis, (2) polymorphic lymphocytic infiltrate, and (3) angiitis.
Symptoms include:
• Ataxia
• Cough
• Distal sensory neuropathy
• Dyspnea
• Erythema
• Fever
• Hemiparesis
• Hemoptysis
• Hepatomegaly
• Lymphocytic infiltration of meninges, cerebral vessels, and peripheral nerves
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