Intravascular large B-cell lymphoma as a cause of hypopituitarism: gradual and late reversal of hypopituitarism after long-term remission of lymphoma with immunochemotherapy.

Intravascular large B-cell lymphoma (IVL) is a rare generally fatal disease characterized by massive proliferation of lymphoid cells within the small and medium blood vessels. Hypopituitarism has been reported only in a few fatal cases. We describe the clinical course of hypopituitarism as a complication of IVL, successfully treated with immunochemotherapy (cyclophosphamide/doxorubicin/vincristine/prednisone-CHOP) plus Rituximab anti-CD20 humanized antibody). Before immunochemotherapy, basal hormonal analysis and dynamic test for pituitary function were performed in a 67-year-old female with IVL. Endocrinological evaluation of the pituitary function was repeated after complete hematological remission and during the 2 years of follow-up. Multiple pituitary hormone deficiencies were diagnosed before therapy for IVL. Magnetic resonance imaging (MRI) of the pituitary gland showed partially empty sella. The patient was replaced with thyroxine 50 microg/day and prednisone 5 mg/day between the cycles of chemotherapy. After complete hematological remission (6 months after initial diagnosis) reversal of cortisol and gonadotropin deficiency occurred. After 18 months of hematological remission there was further improvement in growth hormone (GH) response to provocative testings (partial GH deficiency), with normalization of somatotropic and thyreotropic axis after 2 years of follow-up. This is the first case of IVL complicated with hypopituitarism, treated with immunochemotherapy which resulted in complete hematological remission and gradual and late reversal of hypopituitarism.