Svajdler Marián, Lazúrová Ivica, Bohus Peter, Pal'ko Miroslav
1st Department of Internal Medicine, Medical Faculty, University Kosice, Trieda SNP 1, 040-01 Kosice, Slovakia.
Wien Klin Wochenschr. 2006 Jul;118(13-14):422-5. doi: 10.1007/s00508-006-0617-y.
The intravascular variant of diffuse large B-cell lymphoma (IVBL) is a rare form of non-Hodgkin lymphoma that is frequently diagnosed at autopsy because the symptoms are nonspecific or confusing. We report a case of IVBL in a woman with pre-existing myelodysplastic syndrome manifested as a fever of unknown origin, bilateral adrenal enlargement and subsequent development of panhypopituitarism. Lymphomatous infiltration or osteomyelitis of the sella was supposed. Despite antibiotic and corticosteroid therapy the patient died within three months. An intravascular variant of B-cell lymphoma with intravascular collections of lymphomatous cells predominantly localized in the adrenal and pituitary glands was found at autopsy. The association of panhypopituitarism with bilateral adrenal enlargement is uncommon in endocrinological praxis and the occurrence of combined endocrine involvement in a patient with IVBL has not been described in previous literature.
弥漫性大B细胞淋巴瘤血管内变异型（IVBL）是一种罕见的非霍奇金淋巴瘤，常因症状不具特异性或令人困惑而在尸检时被诊断出来。我们报告一例患有骨髓增生异常综合征的女性IVBL病例，其表现为不明原因发热、双侧肾上腺肿大以及随后发生的全垂体功能减退。推测存在鞍区的淋巴瘤浸润或骨髓炎。尽管进行了抗生素和皮质类固醇治疗，患者仍在三个月内死亡。尸检发现一种血管内变异型B细胞淋巴瘤，其血管内有聚集的淋巴瘤细胞，主要位于肾上腺和垂体。全垂体功能减退与双侧肾上腺肿大的关联在内分泌实践中并不常见，且先前文献中未描述过IVBL患者出现合并内分泌受累的情况。
