Objective The Asian variant of intravascular lymphoma (AIVL) is a rare non-Hodgkin's lymphoma, characterized by hemophagocytic syndrome and the absence of neurological abnormality or skin lesions, which are typical features of classical IVL. The purpose of this study was to describe the characteristics and outcomes of AIVL patients.
Patients and Methods We conducted a retrospective chart review of AIVL patients who were admitted to our institution between January 1999 and December 2006.
Results Eight patients met the criteria. All patients presented with typical clinical features, including fever, hepatosplenomegaly, and cytopenia. The median time from onset to diagnosis was 4 weeks (range 2-12). Pathological diagnoses were made by bone marrow biopsy in three patients, transjugular liver biopsy in two patients, splenectomy in two patients, and percutaneous liver biopsy in one patient. All patients were treated with CHOP-like chemotherapy and rituximab was administered in five patients. Six patients achieved complete response, one achieved partial response, and two patients had central nervous system (CNS) relapse. Among the five patients treated with rituximab, four are still alive, with a median follow-up of 28 months.
Conclusion A CHOP-like regimen with rituximab may be effective for AIVL cases, but, as in classical IVL, CNS prophylaxis should be considered.