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Case Reports in Hematology
Volume 2016, Article ID 6239416, 5 pages
http://dx.doi.org/10.1155/2016/6239416
Case Report

A Case of De Novo CD5+ Disseminated Intravascular Large B-Cell Lymphoma Presenting as Multiorgan Failure

1Department of Internal Medicine, The University of Missouri Kansas City School of Medicine, 2301 Holmes Street, Kansas City, MO 64108, USA
2Department of Laboratory Medicine, Saint Luke’s Hospital of Kansas City, 4401 Wornall Road, Kansas City, MO 64111, USA
3Department of Hematology/Oncology, Saint Luke’s Hospital of Kansas City, Saint Luke’s Cancer Specialists, 4321 Washington Street, No. 4000, Kansas City, MO 64111, USA

Received 7 July 2016; Accepted 14 September 2016

Academic Editor: Gergely Feher

Copyright © 2016 Daulath Singh et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Intravascular large B-cell lymphoma is an extremely rare extranodal lymphoma that proliferates in the lumen of the blood vessels while sparing the organ parenchyma. It usually presents with CNS and skin involvement. A 65-year-old Caucasian female presented with fevers and chills of 3-4 months’ duration. Bone marrow biopsy done 3 months prior showed no significant myelodysplasia or lymphoid aggregates. The patient later died due to multiorgan failure. A bone marrow biopsy showed 20–30% CD5+ B cells consistent with infiltrative large B-cell lymphoma. An autopsy performed revealed diffuse intravascular invasion by lymphoma cells. Multiorgan involvement by intravascular B-cell lymphoma is very rare. Based on our literature review and to the best of our knowledge, there are only 5 case reports describing the presentation of this lymphoma with multiorgan failure. The immunophenotypic studies performed revealed that our patient had de novo CD5+ intravascular large B-cell lymphoma which is known to be aggressive with very poor prognosis. Although it is an extremely rare lymphoma, it should be considered as a potential cause of multiorgan failure when no other cause has been identified. A prompt tissue diagnosis and high-dose chemotherapy followed by ASCT can sometimes achieve remission.