Asian Variant of Intra Vascular Large B cell Lymphoma of the Ad-Renal Glands with CNS Relapse-A Case Report

A 57-year-old gentleman presented with recurrent episodes of lowgrade fever, fatigue, and headache. Initial blood investigations showed pancytopenia with a serial decrease in counts. A laboratory workup ruled out infective pathology. Further, bone marrow aspiration was inconclusive. Provisionally, he was diagnosed with probable macrophage activation syndrome and was therefore pulsed with steroids. Subsequently, he showed improvement and was later discharged. However, after two weeks, he was readmitted with altered sensorium and lethargy. As his condition was found to be gradually deteriorating, a positron emission tomography-computed tomography was done; it showed significantly enlarged bilateral adrenal glands. Adrenal biopsy confirmed that it was non-Hodgkin’s lymphoma, specifically Intravascular Large B-Cell Lymphoma (IVLBCL). Afterwards, he was treated with R-CHOP, followed by intermediate doses of methotrexate for Central Nervous System (CNS) prophylaxis. His condition had improved on follow-up. However, after 18 months since diagnosis, he developed an isolated CNS relapse, confirmed by a brain biopsy. He was treated with modified DeAngelis protocol followed by whole-brain radiation therapy. To the best of our knowledge, this is the first reported case in India of adrenal gland IVLBCL with CNS relapse that responded to second-line chemotherapy with an overall survival of 60 months.