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Annals of Oncology 13:1503-1506, 2002
© 2002 European Society for Medical Oncology

Clinical Investigations

Intravascular large B-cell lymphoma with a fulminant clinical course: a case report with definite diagnosis post mortem

M. Fiegl1,+, R. Greil1, C. Pechlaner2, J. Krugmann3 and S. Dirnhofer3

1 Department of Internal Medicine, Division of Hematology and Oncology; 2 Intensive Care Unit; 3 Institute of Pathology, University of Innsbruck, Innsbruck, Austria

Received 30 January 2002; accepted 19 February 2002

Abstract

A patient is described who presented with pancytopenia, splenomegaly and excessively elevated lactate dehydrogenase levels in concurrence with signs of extramedullary hematopoiesis. Although initially considered in the differential diagnostic spectrum, a highly aggressive lymphoma could not be identified before the patient died, 6 weeks after admission. Even an intensive diagnostic work-up including splenectomy and repeated bone marrow biopsies was inconclusive. Finally, the diagnosis of an intravascular large B-cell lymphoma, a highly aggressive clinical subtype of a diffuse large B-cell lymphoma, spreading within vascular structures of multiple organs was established by autopsy. Intravascular large B-cell lymphoma is often not diagnosed before death due to the exclusive intravascular growth pattern of the tumor cells and a fulminant clinical course. The heterogeneous clinical features of this lymphoma subtype are discussed.

Key words: angioendotheliomatosis, angiotropic lymphoma, intravascular lymphoma, splenomegaly


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