Lymphomatoid Granulomatosis : Clinical Features I

Comments:
Lymphomatoid granulomatosis (LG) presents in adults usually between 4th and 6th decades of life with a male preponderance (M:F=2:1). Children and young adults may be sometimes affected. Most patients with LG are previously healthy individuals.

Some cases occur in the setting of primary or secondary immunodeficiencies, including Wiskott-Aldrich syndrome, HIV infection and AIDS, human T-cell leukemia infection, X-linked lymphoproliferative syndrome and immunosuppressive states associated with chemotherapy and organ transplantation. Even patients with no known immunodeficiency state appear to have impaired humoral and cell-mediated immune response to EBV infection.

Sites of Involvement: LG is predominantly a pulmonary disease with bilateral lung involvement in >90% of cases at presentation. Concomitant extrapulmonary lesions are seen in about one-third of cases. The most common sites are skin (usually lower extremity), central nervous system, liver, and kidneys. Isolated cases have been reported in adrenal glands, heart, and gastrointestinal tract. Lymph nodes and spleen are rarely involved at diagnosis. In exceptional cases, the lesions occur solely in extrapulmonary sites with no involvement of the lungs.

This image shows dense infiltrate in the lung from a patient with LG (clinical features continue in the next slide).