Intravascular variant of diffuse large B-cell lymphoma with combined endocrine involvement

Abstract

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.

Primary Bilateral Adrenal Diffuse Large B-cell Lymphoma Demonstrating Adrenal Failure

“…IVL is characterized by intravascular proliferation of malignant lymphomatous cells, which frequently invade the central nervous system (CNS), skin, lungs and kidneys (12,18,19). Both pituitary and adrenal involvement are frequent in IVL patients (12,20,21). Noteworthy is that CNS infiltration is described in 50% of Japanese patients with primary adrenal IVL (12).…”

Intravascular lymphomatosis of the brain in a patient with myelodysplastic syndrome

“…Despite a relative paucity of literature on this topic, several other cases have been reported in which Ivl followed a previous diagnosis of MDS. [16][17][18] In one of these cases, otherwise lymphomatous cells were shown to be positive for myeloperoxidase, an enzyme that is considered indicative of myeloid differen tiation and is used to distinguish myeloid from lymphoid cells; this observation led the researchers to consider the possible involvement of a multipotential neoplastic stem cell. 17 Furthermore, as noted, laboratory studies have described B-cell abnormalities in subsets of patients with MDS.…”

Reversible Hypopituitarism Associated with Intravascular Large B-Cell Lymphoma: Case Report of Successful Immunochemotherapy

“…Moreover, many of the patients died, and the hormonal evaluation after treatment was not always mentioned even in the survived cases (Wick et al 1986;Smadja et al 1991;Prayson et al 1991;Demirer et al 1994;Kraus et al 1999;Mathiasen et al 2000;Schleinitz et al 2002;Price et al 2002;Svajdler et al 2006;Lee et al 2011;Anila et al 2012;Akhtar et al 2013). Thus far, the endocrinological course has been described in only two cases in the literature (Table 4) (Pekic et al 2008;Yasuda et al 2010).…”