Title : ANGIOTROPHIC B CELL LYMPHOMA: A Great Chameleon
Angiotrophic B cell lymphoma, also called as Intravascular Lymphoma is a rare extra nodal type of lymphoma. The Clinical symptoms of the disease depends on the organs involved. It often presented atypically and that makes timely and accurate diagnosis of angiocentric lymphomas very difficult and often delayed. It can present with neurological, dermatological symptoms, Fever of unknown origin, Haemophagocytic syndrome (fever, amemia, thrombocytopenia). Majority of the cases are presented with Central Nervous System (CNS) involvement with diffuse encephalopathy and subacute multiple focal neurological deficits resembling vasculitis / neuroinflammation. Majority of the patients have poor prognosis2.The prognosis of the patient was primarily dependent on early diagnosis and treatment with chemotherapy, with or without radiotherapy.
A 60 year old gentleman had a past medical history of alcoholism & symptomatic anaemia for 6 months. He was under observation by haematologist and transient lymphadenopathy was found on CT neck/thorax/abdomen/pelvis. However, transient reactive cause was thought to be the most probable cause. Attempted ultrasound guided biopsy did not materialised due to resolution of lymphadenopathy. Three months later, he was admitted with severe delirium. He was initially treated as alcoholic related meningoencephalopathy. Blood tests showed borderline pancytopenia and raised ESR. CSF was acellular with raised protein. Matched oligoclonal band were found subsequently. MRI brain showed multiple foci of T2 lesion in the cerebral hemisphere which are nonspecific and could be in favour of neuroinflammation process, infective or infarction. He received an empiric acyclovir and corticosteroid therapy for which he showed partial transient response. Neuroradiological & Neurosurgical opinion were sought. He was scheduled for brain biopsy in a Tertiary Centre. Unfortunately, he suddenly developed seizure and steroid related gastrointestinal bleed. He was managed in Intensive care unit (ITU). Whilst having treatment in ITU, he had myocardial infarction (NSTEMI) and had cardiac arrest. Despite all active treatment, he deteriorated and passed away. His post mortem examination revealed angiotrophic B Cell Lymphoma in multiple organs.