Case Report
Intravascular Lymphoma with Progressive CNS Hemorrhage
and Multiple Dissections
Ricky Chen ,
1
Gurjeet Singh,
2
J. Scott McNally ,
3
Cheryl A. Palmer ,
4
and Adam de Havenon
5
1
Providence Brain & Spine Institute, Providence St. Vincent Medical Center, 9135 SW Barnes Road, Suite 461, Portland,
OR 97225, USA
2
Legacy Medical Group Neurology, Salmon Creek Medical Center, 2121 NE 139th St., Suite 205 Medical Office Building A,
Vancouver, WA 98686, USA
3
Department of Radiology and Imaging Sciences, University of Utah, 30 N 1900 E, Salt Lake City, UT 84132, USA
4
Department of Pathology, University of Utah, Huntsman Cancer Institute, 2000 Circle of Hope Dr., Salt Lake City,
UT 84112, USA
5
Department of Neurology, University of Utah, 175 N Medical Dr., Salt Lake City, UT 84132, USA
Correspondence should be addressed to Ricky Chen; ricky.chen2@providence.org
Received 25 June 2019; Revised 22 August 2019; Accepted 10 September 2019; Published 10 January 2020
Academic Editor: Isabella Laura Simone
Copyright © 2020 Ricky Chen et al. is 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.
Introduction. Intravascular lymphoma (IVL) is an uncommon and oſten fatal disease characterized by intraluminal proliferation of
lymphomatous cells within blood vessels. Because of a heterogeneous clinical presentation and lack of sensitive diagnostic protocols,
diagnosis of IVL is most oſten made at autopsy. However, with early diagnosis and appropriate chemotherapy, the prognosis is
greatly improved and complete remission is possible. In order to broaden the possible presentations of IVL, we present a patient
with an atypical manifestation of biopsy-proven intravascular large B-cell lymphoma who suffered dissections of both intracranial
and extracranial arteries in addition to progressive intracranial hemorrhages. Case Report. A 47-year-old woman presented with
unilateral paresthesias. She developed progressive multifocal infarcts and hemorrhage with dissections of both intracranial and
extracranial arteries, resulting in coma. Brain biopsy revealed IVL. She received aggressive chemotherapy and remains in complete
remission with good neurologic recovery. Conclusion. IVL is known to exert its pathology on small arteries and capillaries, but is
not known to cause dissections of large vessels. e diagnosis should be considered in cases with unexplained arterial dissections
and progressive strokes. Early diagnosis with appropriate laboratory screening and tissue confirmation by biopsy can lead to greatly
improved outcomes.
1. Introduction
Intravascular lymphoma (IVL) is recognized by the World
Health Organization (WHO) as an extremely rare subtype of
diffuse extranodal large B-cell non-Hodgkin’s lymphoma with
an estimated annual incidence of 0.5 cases per 1,000,000 [1].
IVL leads to intraluminal proliferation of neoplastic lymphoid
cells in small and medium vessels [2]. ese cells have lost the
ability for transvascular migration due to defects in adhesion
molecules [3]. IVL can involve any organ system, particularly
the central nervous system (CNS), and its protean neurological
manifestations oſten hinder diagnosis. Patients can present
with subacute encephalopathy or dementia, paralysis, seizures,
or multifocal cerebrovascular events, by order of frequency
[1, 4, 5]. Systemic symptoms vary, but fever and skin lesions
are the most common, whereas lymphadenopathy is usually
absent [2].
Serologic abnormalities include anemia (63%), high lac-
tate dehydrogenase (LDH) (82%) and beta 2-microglobulin
levels (86%) [6]. Bone marrow involvement occurs in 32% of
patients, and cerebrospinal fluid (CSF) rarely contains malig-
nant cells [6]. Magnetic resonance imaging (MRI) is nonspe-
cific and can be normal, though there are numerous reports
of rapid progression of cerebral infarcts [5]. Organ biopsy is
the gold standard for diagnosis of IVL but is frequently per-
formed too late, and cases with CNS involvement are most
Hindawi
Case Reports in Neurological Medicine
Volume 2020, Article ID 6134830, 7 pages
https://doi.org/10.1155/2020/6134830