山口医学

Back to Top

山口医学 Volume 26 Issue 2
published_at 1977-06

A Case of Angio-Immunoblastic Lymphadenopathy

Angio-Immunoblastic Lymphadenopathy (A.I.L)の一症例
Hujimoto Shigeki
Santokii Yosiro
Santoki Osamu
Murakami Michiharu
Mizuta Eishi
Oku Tomozo
Descriptions
A new disease entity of the lymphocyte-plasma cell system characterized by generalised lymphadenop athy, skin rashes, fever, and hypergammaglobulinemia has recently been reported by Frizzera and his colleagues as angio-immunoblastic lymphadenopathy with dysproteinemia (A . I . L .) and by Lukes and Tindle as immunoblastic lymphadenopathy. The lymph nodes are dfffusely involved and show proliferation of immunoblasts, plasma cells, and lymphocytes, and many arborizing small vessels, and amorphous, acidphilc materils are also observed. The onset is frequently sudden and the course stormy and complicated by infections. The commonest immunological abnormality in A . I .L .is a diffuse heterogeneous elevation of one, two, or three immunoglobulins. It is not yet clear whether A . I . L . is an immunological or a neoplastic process, but is possible that it is caused by hyperactivity of the immune system. We have lately seen a 72-year-old man who had generalized lymphadenopathy which on biopsy showed finding similar to A . I. L . Serum protein electrophoresis showed a hypergammaglobulinemia and hypoalubuminemia. Radial immunodiffusion showed elevation of IgG and IgM. He was given combination chemotherapy, including Vincristin, Endoxan, 6-MP, and Predonine and accuired complete remission. In view of the suggested possibility of a malignant evolution of this disease, as in order abnormal immunological precesses, a careful long-term follow up is indicated.