AIDS-related primary pulmonary lymphoma (PPL) is defined as exclusive involvement of the lung by lymphoma with no evidence of extrapulmonary disease at the time of diagnosis and three months thereafter. It is an aggressive disease with a median survival time of four months. A 46 year old male patient with a seven year history of HIV/AIDS and CD4 count of 200/ul was hospitalized because of flank pain, hematuria and hemoptysis. Abdominal CT scan incidentally revealed multiple nodules in the lower lobes of both lungs. Serial chest CT scans demonstrated multiple nodular opacities in all lobes of the lungs that enlarged rapidly and coalesced together. Clinicians favored opportunistic infection due to rapid disease progression but included Wegener's granulomatosis in the differential diagnosis because of hemoptysis. Open lung biopsy showed diffuse large B cell lymphoma (DLBCL), NOS, with an activated B cell phenotype (CD10−/BCL6−/MUM1+) and high proliferative index (Ki67 90%). CD79A expression confirmed B cell lineage. The diagnostic criteria for PPL were fulfilled because staging failed to detect extrapulmonary disease. Infarction due to the angiodestructive nature of the PPL and short doubling time accounted for hemoptysis and rapid disease progression, respectively. The patient died 45 days after admission because of shock, presumably due to superimposed infection. Unlike prior reports of AIDS-related PPL, our case was negative for CD19, CD20 and Epstein-Barr virus (EBV). Although PPL is a rare disease, it deserves consideration in the differential diagnosis of rapidly progressive lung nodules in AIDS patients. To the best of our knowledge, this is the first reported case of EBV-negative PPL.
No relevant conflicts of interest to declare.
Asterisk with author names denotes non-ASH members.