特異な臨床経過を示した進行性鼻壊疸の一剖検例

概要

論文の詳細を見る
We report herein a case of so-called rhinopathia gangrenosa progressiva in a man, aged 29, who demonstrated very unusual clinical manifestations of panmyelopathy. The patient initially developed a necrotic granulomatous lesion of the nose with no specific pathology on consecutive biopsies, and laboratory data were not remarkable except for an increased α2-globulin. Under the suspicion of malignant granuloma, the patient was given 60Co irradiation and systemic administrations of antibiotics and steroids and he had a complete remission for two years. After this remission, however, the patient developed high fever accompanied with severe anemia and leukopenia which increasingly progressed despite all intensive treatment. The patient died of panmyelopathy and acute renal failure. Though repeated biopsies and blood examinations including bone marrow puncture failed to reveal any neoplastic lesions, the autopsy demonstrated diffuse intense infiltration of malignant atypical cells in the different organs such as nasal and paranasal sinus mucosa, bone marrow, spleen and myocardial tissue.The diagnosis of rhinopathia gangrenosa progressiva is essentially a clinical one and includes two different entities of diseases in its spectrum, e. g. Wegener's granulomatosis and malignant reticulosis or reticulum cell sarcoma which should be treated in a different manner. It is most difficult to differentiate each of these diseases and to prolong the remission. Since this patient had a relatively long-term remission and lacked any pathological lesions in the upper respiratory tract, only a hematopoietic suppression in the recurrence of disease, the diagnosis and the treatment were confused and late. In the treatment of rhinopathia gangrenosa progressiva, we should remember that the disease is not localized and the remission is unstable. Strict follow-up observations should be faithfully carried out.
耳鼻咽喉科臨床学会の論文

特異な臨床経過を示した進行性鼻壊疸の一剖検例

スポンサーリンク

概要

著者

関連論文

スポンサーリンク