Abstract
Intravascular large B-cell lymphoma (IVLBCL) is a rare form of extranodal large B-cell lymphoma characterized by the growth of lymphoma cells within lumina of blood vessels, especially capillaries, which aggregate to form clots, resulting in organ ischemia. In Caucasians, it predominantly involves the central nervous system (CNS) and the skin, with the cutaneous variant carrying a better prognosis. Whereas in Asians it preferentially involves the bone marrow, liver, and spleen and is associated with hemophagocytic syndrome. We report a case of a young Asian male with neurological, pulmonary, and hepatosplenic involvement. He presented with recurrent strokes, chronic cough, and unintentional weight loss. The chest radiograph (CXR) on admission was clear. Magnetic resonance imaging (MRI) of the brain showed acute multifocal infarcts, and a whole-body computed tomography (CT) scan revealed upper-lobe predominant pulmonary ground glass opacities (GGOs) with mediastinal lymphadenopathy. Interestingly, a CXR performed one week after the CT scan remained clear. The positron emission tomography-computed tomography (PET-CT) showed hepatosplenic and adrenal involvement. The diagnosis was confirmed via a bronchoscopic approach. The patient received chemotherapy consisting of MR-CHOP (methotrexate, rituximab, cyclophosphamide, adriamycin, vincristine, and prednisolone), high-dose methotrexate, and intrathecal cytarabine, which led to complete remission. Subsequently, he underwent an autologous peripheral blood stem cell transplant. At the time of writing this case report, the patient is still in complete remission for three years after the initial diagnosis. As IVLBCL has a non-specific clinicoradiological presentation, it is important to suspect IVLBCL in patients with an atypical neurological and pulmonary presentation in the presence of raised serum lactate dehydrogenase (LDH) and to consider a CT scan of the thorax if CXR is normal.