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Int J Clin Exp Pathol 2012;5(5):448-454

Case Report
Intravascular large B-cell lymphoma with hemophagocytic syndrome (Asian variant) in a
Caucasian Patient

Kar-Ming Fung MD, Jennifer H Chakrabarty, William F Kern, Hany Magharyous, Bradley C Gehrs, Shibo Li

Department of Pathology, Medicine-Hematology/Oncology/Bone Marrow Transplantation, Pediatrics, University of Oklahoma Health Sciences
Center, and Department of Pathology, Oklahoma City Veterans Affair Medical Center, Oklahoma City, OK, 73104, USA.

Received May 7, 2012; accepted May 18, 2012; Epub May 23, 2012; Published June 30, 2012

Abstract: Intravascular lymphoma is an aggressive and extremely rare extranodal lymphoma with neoplastic lymphoid cells confined
exclusively within intravascular spaces. The histopathologic findings are subtle due to the rarity of the neoplastic cells in blood vessels. Clinical
presentations are non-specific and focal space-occupying lesions or lymphoadenopathy are always lacking. It is a diagnostic challenge.
Secondary hemophagocytic syndrome is uncommon and is typically associated with infection, malignancy, and suppressed immune states.
Intravascular lymphoma has a strong association with hemophagocytic syndrome in Asian patients, the so-called "Asian variant", but not in
Western patients. We report a case of intravascular B-cell lymphoma in a Caucasian patient associated with secondary hemophagocytic
syndrome. The patient was diagnosed by core liver biopsy and successfully treated. This case demonstrates the importance of high index of
suspicion and astute histopathologic examination in recognition of this unusual clinical and pathologic combination. (IJCEP1205001)

Keywords: Intravascular lymphoma, hemophagocytic syndrome, liver, Asian variant, hepatosplenomegaly

Address all correspondence to:
Dr. Kar-Ming Fung
Department of Pathology
University of Oklahoma Health Sciences Center
BMSB 451, 940 Stanton Young Blvd
Oklahoma City, OK 73104, USA.
Tel: (405) 271-5653; Fax: (405) 271-2524
E-mail: karming-fung@ouhsc.edu