Clinical-Pathological Case Study
Section Editors: Daniel R. Gold, DO
Marc Levin, MD

Episodic Visual Distortions and Stroke-Like Symptoms
in a 56-Year-Old Man With Intravascular Lymphoma
Bart K. Chwalisz, MD, Vivian P. Douglas, MD, DVM, MBA,
Konstantinos A. A. Douglas, MD, DVM, MBA, Maria Martinez-Lage, MD,
Hilary R. Kelly, MD, Dean M. Cestari, MD

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Abstract: A healthy 56-year-old man presented with
vision changes and left upper extremity motor and
sensory changes. MRI of the brain without contrast was
significant for multifocal areas of restricted diffusion in
multiple vascular territories. Neuro-Ophthalmic evaluation revealed an inferonasal visual field defect in the left
eye, thickened choroid on optical coherence tomography, and bilateral delayed arteriovenous and choroidal
filling on fluorescein angiogram. Repeat MRI demonstrated interval enlargement of many of the same foci
of abnormal diffusion-weighted imaging signal. Computed tomography of the abdomen and pelvis revealed
3 distinct lobulated retroperitoneal masses that were
biopsied and found to be consistent with diffuse large
B-cell lymphoma. Brain biopsy specimens showed
intravascular lymphocytes, confirming a diagnosis of
intravascular lymphoma (IVL). In this diagnostically
challenging case, a link was established between the
presence of multiple strokes (some of which showed
slow evolution over time) and retinochoroidal hypoperfusion, which provided a critical clue to the ultimate
diagnosis of IVL.
Journal of Neuro-Ophthalmology 2020;40:265–270
doi: 10.1097/WNO.0000000000000900
© 2020 by North American Neuro-Ophthalmology Society

Division of Neuro-Ophthalmology (BKC, VPD, KAAD, DMC), Massachusetts Eye and Ear, Harvard Medical School, Boston, Massachusetts; and Departments of Neurology (BKC), Pathology (MML),
and Radiology (HRK), Harvard Medical School, Massachusetts
General Hospital, Boston, Massachusetts.
The authors report no conflicts of interest.
Address correspondence to Bart K. Chwalisz, MD, Department of
Neurology, Massachusetts General Hospital, 15 Parkman Street,
Boston, MA 02114; E-mail: bchwalisz@mgh.harvard.edu
Chwalisz et al: J Neuro-Ophthalmol 2020; 40: 265-270

Drs. Chwalisz, Douglas VP, Douglas
KAA, Cestari:

A

56-year-old man with no significant medical history
apart from red-green color blindness presented to the
emergency department of an outside hospital with a complaint of vision changes, left facial droop, left-hand incoordination, and sensory changes.
Four months before presentation, the patient had a first
episode of vision change characterized by not being able to
see oncoming traffic for several minutes. He subsequently
began experiencing episodes of left-sided visual distortions
lasting minutes. He reported looking at people and seeing
the right side of their face melting “like the faces of the
characters in the Indiana Jones movie.” He could not specify whether the changes were occurring in the left eye or the
left visual field.
Over the subsequent weeks, he continued to experience
episodes of visual change. In addition, he had episodes of
numbness in the left hand, face, and inside of his left cheek.
He also developed a tendency to veer to the left when
walking.
While on a hiking vacation in another state, the patient
had a sudden episode of left-hand incoordination making
him unable to turn the faucet. The left side of his face
drooped. Upon arrival to the emergency department, most
of his symptoms had resolved except the left facial drooping.
He denied fever, recent infection, skin lesions, weight loss,
or confusion. He did recall a similar episode of left-sided
weakness a week earlier, for which he did not seek medical
attention.
The patient was admitted with suspicion of cerebrovascular accident. Transthoracic echocardiogram (TTE)
and inpatient telemetry were unrevealing. The patient
was diagnosed with multifocal embolic strokes of
unknown etiology. He was discharged on aspirin 81 mg
and atorvastatin 10 mg daily.
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Clinical-Pathological Case Study

FIG. 1. MRI of the brain without intravenous contrast at initial presentation. Multiple foci of abnormal hyperintense signal
were identified on the diffusion weighted images involving the white matter of both frontal lobes, the left parietal lobe, the
splenium of the corpus callosum, the left temporal lobe and the left cerebellar hemisphere. Corresponding hypointense
signal consistent with restricted diffusion was seen within a few of the right frontal white matter lesions on the corresponding
apparent diffusion coefficient maps (not shown).

Dr. Kelly:
MRI of the brain without contrast demonstrated multifocal
areas of diffusion-weighted imaging (DWI) hyperintense signal
involving the white matter of both frontal lobes, the left parietal
lobe, the splenium of the corpus callosum, the left temporal
lobe, and the left cerebellar hemisphere (Fig. 1). There was
restricted diffusion within a few of the right frontal white
matter lesions on the corresponding apparent diffusion coefficient (ADC) maps, and all lesions demonstrated corresponding T2 FLAIR hyperintense signal (not shown). No
flow-limiting stenoses involving the cervical carotid or vertebral arteries were demonstrated on MRA (not shown).

Drs. Chwalisz, Douglas VP, Douglas
KAA, Cestari:
Given the persistence of symptoms of left upper extremity
weakness and paresthesias, the patient presented for reevaluation to the emergency department of our hospital. The
patient’s examination was notable for deficits in visuospatial
function, memory, and attention tasks, with a score of 23/30
on the Montreal Cognitive Assessment. He had mild left lower
facial weakness, left pronator drift, and left-sided Hoffmann’s
sign. His strength was full in all 4 extremities. Sensation was
intact, with no extinction to double simultaneous stimulation.
The neuro-ophthalmic examination revealed visual acuity
of 20/20 in the right eye and 20/25 in the left eye. There was
no afferent pupillary defect. On Ishihara color vision testing,
the patient identified 2/8 (test plate and 1 additional plate) in

the right eye and 1/8 (test plate only) in the left eye, consistent
with his known history of red-green color blindness. Extraocular motility and ocular alignment were normal. Humphrey
visual fields were reliable in both eyes and showed an
inferonasal visual field defect in the left eye (Fig. 2A, B).
Anterior segment and dilated fundus examination were normal
(Fig. 2C, D). Optical coherence tomography scans showed
thickening of the choroid (Fig. 3A, B). Fluorescein angiography showed bilateral delayed arteriovenous and choroidal filling with distal pruned capillary bed, leakage, and window
defects more prominent in the left eye (Fig. 4A–F).
TTE revealed evidence of a patent foramen ovale. Lower
extremity duplex ultrasound was negative for deep vein
thrombosis. Lumbar puncture showed pink cerebrospinal fluid
(CSF) with slight turbidity, 1 white blood cell, 35% neutrophils,
2% eosinophils, 2,195 (tube 1) to 255 (tube 4) red blood cells,
total protein 71 mg/dL (range 5–55), glucose 62, 0 oligoclonal
bands, immunoglobulin G 7.8 (upper limit of normal: 4.3), and
beta-2-microglobulin 1.98 (range 0.7–1.8). A serum and CSF
paraneoplastic panel and CSF IgH rearrangement were negative.

Dr. Kelly:
A repeat MRI of the brain with and without intravenous
contrast demonstrated enlargement of many of the foci of
abnormal DWI signal (Fig. 5A, B) compared with those seen
on the previous outside MRI. Evolving signal characteristics
were also noted, with the largest lesions in the right frontal and
left parietal white matter demonstrating geographic regions of

FIG. 2. Visual fields and fundus photos. A. Left eye. B. Right eye. C. Right eye. D. Left eye.

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Clinical-Pathological Case Study

FIG. 3. Optical coherence tomography demonstrating choroidal thickening. A. Right eye. B. Left eye.

restricted diffusion peripherally, whereas other lesions demonstrated intrinsic T1 hyperintense signal and enhancement. The
pattern was overall suggestive of multiple infarcts of varying
age, although the persistent restricted diffusion on ADC maps
within the right frontal white matter lesions, the pattern of
enhancement, and the white matter predominance were all
unusual features for multifocal ischemic infarcts.
Computed tomography of the abdomen and pelvis with
intravenous contrast revealed 3 distinct lobulated retroperitoneal masses that demonstrated soft tissue attenuation
with mass effect on the adjacent right kidney (Fig. 5C).
A biopsy of the perinephric mass was performed.

staining. In situ hybridization for Epstein–Barr virusencoded RNA (EBER) was negative.
A diagnostic brain biopsy was also performed.
The sections of right frontal lesion specimens showed
cerebral cortex with scattered parenchymal small vessels and
capillaries plugged with large atypical lymphoid cells, with
occasional mitoses (Fig. 6A–C).
Immunohistochemical stains were performed and showed
that the tumor cells are positive for CD20 and Pax-5, and show
extremely high positivity with Ki-67. CD3 shows scattered
perivascular and parenchymal mature T-cells (Fig. 7A–D).

Dr. Martinez-Lage:

Intravascular lymphoma (IVL) associated with systemic
large B-cell lymphoma.

The core biopsies of the right perinephric mass consist of
a dense proliferation of large neoplastic lymphoid cells with
moderate cytoplasm, marked nuclear pleomorphism and
irregularity, prominent nucleoli, and vesicular chromatin.
There is abundant tumor cell necrosis as well as frequent
mitotic figures and abundant apoptotic debris associated
with the proliferation.
Immunohistochemistry stains showed that the neoplastic cells were CD20+ PAX5+ B cells that coexpress
BCL6, MUM1, and BCL2. These cells were negative
for CD10, CD21, and CD30. Approximately 60% of
the cells stained positive for CMYC. The proliferation
fraction was approximately 90% on the basis of Ki-67

Final Diagnosis

Drs. Chwalisz, Douglas VP, Douglas
KAA, Cestari:
IVL is a rare subtype of diffuse extranodal large B-cell
lymphoma that involves neoplastic proliferation of mononuclear cells within the lumina of medium- to small-sized
vessels. It was first reported by Pfleger L and Tappeiner J. in
1959 as “angio-endotheliomatosis proliferans systematisata” (1).
Other historical terms for this condition include “proliferating
endotheliosis,” “malignant angioendotheliomatosis,” and “neoplastic endotheliosis” due to the confinement of neoplastic cells

FIG. 4. Fluorescein angiography showing bilateral delayed arteriovenous and choroidal filling with distal pruned capillary bed,
leakage, and window defects more prominent in the left eye. A. Left eye 18.654 sec. B. Left eye 22.053 sec. C. Left eye
31.853 sec. D. Left eye 59.063 sec. E. Right eye 3:18.433 min. F. Close-up of distal vessels of right eye at 3:18.433 min.
Chwalisz et al: J Neuro-Ophthalmol 2020; 40: 265-270

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Clinical-Pathological Case Study

FIG. 5. A and B. Repeat MRI of the brain without and with intravenous contrast demonstrated increase in size of some of the foci of
diffusion-weighted imaging hyperintense signal compared to the initial MRI (Fig. 1), as well as evolution of corresponding signal
characteristics on the apparent diffusion coefficient maps and T1-weighted noncontrast and postcontrast images (not shown). C. CT
of the abdomen and pelvis with intravenous contrast revealed 3 distinct lobulated soft tissue attenuation retroperitoneal masses.

within the intraluminal space without involvement of the adjacent tissues, which led to the notion that the malignant cells are
derived from the endothelium (2,3). In 1986, it was classified
as “intravascular lymphomatosis,” and the term angiotropic
(intravascular) large-cell lymphoma was proposed based on
the immunohistochemical findings (4,5). The World Health
Organization has classified IVL as a “Mature B cell” neoplasm
due to morphological and phenotypical similarities to the diffuse large B-cell lymphoma (DLBCL) (6). IVL can be primary,
when no other systems are involved, or secondary in the setting
of systemic lymphoma.
IVL is a rare disease with an estimated incidence of less
than one person per million worldwide (7). It has been reported only in adult population with median age of 67 years
(34–90 years range) without sex predilection. The disease is
characterized by its polymorphic clinical presentation as any
organ throughout the body can be affected, making diagnosis
challenging. Two variants with partially overlapping clinical
features have been described; the classic or “Western” variant
with high frequency of central nervous system (CNS) and
skin involvement (8) and the “Asian variant” that is characterized by a hemophagocytic syndrome (9). In the “Western”-type IVL, bone marrow and reticuloendothelial system
involvement are seen less often, suggesting that genetic predisposition or environmental factors have an impact on the
development of the clinical manifestations.
B symptoms (fever night sweats and weight loss),
cutaneous manifestations, (10) and neurologic disturbances

have been also reported in the medical literature with neurologic signs and symptoms correlating with the affected
area of the nervous system, secondary to compromised tissue perfusion by the tumor cells. Cognitive impairment in
the form of subacute encephalopathy or rapidly progressive
dementia are found in more than 60% of these patients
while paralysis, seizures, stroke-like symptoms, peripheral
neuropathy, and myopathy are among other less common
neurologic manifestations of IVL (11–13).
Ophthalmic involvement has been reported in only a few
case reports and includes cranial nerve disturbances, blurry
vision, nystagmus, papilledema, cortical blindness, retinal artery
occlusion, retinal pigment epithelial change, retinal and
choroidal detachment, iridocyclitis with keratic precipitates,
and vitreous cell (14). Most visual symptoms are caused by
cortical involvement, with only rare reports of involvement of
the anterior visual pathway and uveal tract (15). In rare cases,
the presence of intravascular tumor cells in ciliary body, retinal,
and choroidal blood vessels has been demonstrated pathologically (14). Of particular, neuro-ophthalmic interest, IVL-related
optic neuropathy, although rare, has been previously reported
(16,17). Dinkin et al recently published the first welldocumented case of bilateral optic neuropathy as a presentation
of underlying IVL (18); their case was also remarkable for
spontaneous visual improvement before treatment and ultimately prolonged survival after treatment.
Because of the rarity of the disease and lack of sensitive
diagnostic methods, early diagnosis of IVL is difficult, with

FIG. 6. Right frontal lesion specimens. A. Minimally hypercellular brain parenchyma with abnormal atypical cells within vessels.
B. The atypical cells have large nuclei with occasional nucleoli and relatively scant cytoplasm and (C) showing mitotic figure.

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Chwalisz et al: J Neuro-Ophthalmol 2020; 40: 265-270

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Clinical-Pathological Case Study

FIG. 7. The immunophenotype shows that the neoplastic intravascular cells are CD20+ (B-cells), and many of them are
actively proliferating (Ki-67). Perivascular reactive T cells are also present. A. CD20–intravascular neoplastic B-cells. B.
CD20–intravascular neoplastic B-cells (higher power). C. Ki-67–proliferating cells. D. CD3–reactive T-cells.

more than half of cases diagnosed at autopsy. The
laboratory findings in patients with IVL are nonspecific
but can suggest the diagnosis. Anemia, thrombocytopenia, leucopenia, increased serum lactate dehydrogenase,
increased b2-microglobulin, and erythrocyte sedimentation
rate are among the most common laboratory abnormalities
(8,19). In addition, altered renal, hepatic, and thyroid function tests have been observed in up to 20% of patients (20).
It has been suggested that random skin biopsies of macroscopically uninvolved skin are highly specific for IVL but
with unclear sensitivity (21); yield could potentially be
increased by biopsying angiomas (22). Blind muscle or
meningeal biopsy can also be performed. A combination
of various tests, such as biopsies of the skin and/or other
organs, bone marrow examination, blood test, and imaging
studies can be of value in the diagnostic process (23,24).

Dr. Kelly:
In general, neuroimaging studies are not considered
diagnostic and demonstrate nonspecific findings including
white matter lesions that can mimic embolic disease or
vasculitis. However, MRI with gadolinium and MRA of the
head and neck should always be performed on any patient
with suspicion of IVL not only to rule out other neurologic
diseases but also as part of the staging workup (20).

Dr. Martinez-Lage:
Small- and medium-sized vessels are more likely to be
infiltrated by neoplastic cells (25). Immunophenotypically,
the IVL tumor cells express mature B-cell-associated antigens with only rare cases of CD202expression and T and
Chwalisz et al: J Neuro-Ophthalmol 2020; 40: 265-270

natural killer cell immunophenotypes (9,26). There is however great variability in the markers that have been described
with CD19+, CD20+, CD22+, CD79a+, and CD5+ being
the most common (27).

Drs. Chwalisz, Douglas VP, Douglas
KAA, Cestari:
Therapeutic approaches have varied due to the lack of clinical
trials and are largely based on the evidence from reported case
series and case reports (28). IVL is always considered disseminated disease, warranting systemic therapy. Rituximab has
been associated with improved remission rate, event-free survival, and overall survival in a retrospective analysis of the
efficacy in 30 IVL patients with the Western variant (29).
Systemic treatment with cyclophosphamide, doxorubicin,
vincristine, prednisone, and rituximab (R-CHOP) (23) and
more aggressive therapies including high-dose methotrexate,
intrathecal methotrexate, and radiation have been tried in
patients with CNS involvement (30). Autologous stem cell
transplantation has demonstrated long-term survival rates
especially in younger patients in first complete remission,
and it should also be considered as part of the treatment plan
in patients with IVL (28,31).
IVL has a high mortality rate. Prognosis seems to be
related to the extent of the disease with lymphoma limited
to the skin carrying a favorable prognosis while CNS
involvement is associated with a dismal prognosis, although
it has improved since addition of rituximab (7,32).
Our patient received intercalated high-dose methotrexate
and R-CHOP (rituximab, cyclophosphamide, doxorubicin,
and prednisone) chemotherapy that was complicated by
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Clinical-Pathological Case Study
peripheral neuropathy, fatigue, and mild cytopenias. He
also received thiotepa, busulfan, and cyclophosphamide
conditioning followed by autologous hematopoietic stem
cell transplantation, which was complicated by mucositis,
febrile neutropenia, graft-versus-host disease, Clostridium
difficile colitis, cryptogenic cirrhosis, and severe malnutrition. The patient succumbed to the disease 13 months after
the initial diagnosis to complications of cirrhosis and portal
hypertension.

REFERENCES
1. Pfleger L, Tappeiner J. On the recognition of systematized
endotheliomatosis of the cutaneous blood vessels
(reticuloendotheliosis? [in German]. Hautarzt. 1959;10:359–363.
2. Fulling KH, Gersell DJ. Neoplastic angioendotheliomatosis.
Histologic, immunohistochemical, and ultrastructural findings
in two cases. Cancer. 1983;51:1107–1118.
3. Strouth JC, Donahue S, Ross A, Aldred A. Neoplastic
angioendotheliosis. Neurology. 1965;15:644.
4. Wick MR, Mills SE, Scheithauer BW, Cooper PH, Davitz MA,
Parkinson K. Reassessment of malignant
“angioendotheliomatosis.” Evidence in favor of its
reclassification as “intravascular lymphomatosis.” Am J Surg
Pathol. 1986;10:112–123
5. Sheibani K, Battifora H, Winberg CD, Burke JS, Ben-Ezra J,
Ellinger GM, Quigley NJ, Fernandez BB, Morrow D, Rappaport H.
Further evidence that malignant angioendotheliomatosis is an
angiotropic large-cell lymphoma. N Engl J Med.
1986;314:943–948.
6. Campo E, Swerdlow SH, Harris NL, Pileri S, Stein H, Jaffe ES.
The 2008 WHO classification of lymphoid neoplasms and
beyond: evolving concepts and practical applications. Blood.
2011;117:5019–5032.
7. Fonkem E, Lok E, Robison D, Gautam S, Wong ET. The natural
history of intravascular lymphomatosis. Cancer Med.
2014;3:1010–1024.
8. Ferreri AJM, Campo E, Seymour JF, Willemze R, Ilariucci F,
Ambrosetti A, Zucca E, Rossi G, López-Guillermo A, Pavlovsky
MA, Geerts ML, Candoni A, Lestani M, Asioli S, Milani M, Piris
MA, Pileri S, Facchetti F, Cavalli F, Ponzoni M; International
Extranodal Lymphoma Study Group (IELSG). Intravascular
lymphoma: clinical presentation, natural history, management
and prognostic factors in a series of 38 cases, with special
emphasis on the “cutaneous variant.” Br J Haematol.
2004;124:173–183.
9. Murase T, Nakamura S, Kawauchi K, Matsuzaki H, Sakai C,
Inaba T, Nasu K, Tashiro K, Suchi T, Saito H. An Asian variant
of intravascular large B-cell lymphoma: clinical, pathological
and cytogenetic approaches to diffuse large B-cell lymphoma
associated with haemophagocytic syndrome. Br J Haematol.
2000;111:826–834.
10. Röglin J, Böer A. Skin manifestations of intravascular
lymphoma mimic inflammatory diseases of the skin. Br J
Dermatol. 2007;157:16–25.
11. Chapin JE, Davis LE, Kornfeld M, Mandler RN. Neurologic
manifestations of intravascular lymphomatosis. Acta Neurol
Scand. 1995;91:494–499.
12. Beristain X, Azzarelli B. The neurological masquerade of
intravascular lymphomatosis. Arch Neurol.
2002;59:439–443.
13. Tahsili-Fahadan P, Rashidi A, Cimino PJ, Bucelli RC, Keyrouz
SG. Neurologic manifestations of intravascular large B-cell
lymphoma. Neurol Clin Pract. 2016;6:55–60.
14. Papalas JA, Proia AD, Cummings TJ. Hematolymphoid
malignancies with intraocular intravascular involvement: report
of 2 cases. Ann Diagn Pathol. 2011;15:286–290.
15. Mudhar HS, Sethuraman C, Khan MD, Jan SU. Intraocular,
pan-uveal intravascular large B-cell lymphoma associated

270

with choroidal infarction and choroidal tri-lineage
extramedullary haematopoiesis. Histopathology.
2007;51:275–279.
16. Baehring JM, Henchcliffe C, Ledezma CJ, Fulbright R,
Hochberg FH. Intravascular lymphoma: magnetic resonance
imaging correlates of disease dynamics within the central
nervous system. J Neurol Neurosurg Psychiatry. 2005;76:540–
544.
17. Pless ML, Chen YBin, Copen WA, Frosch MP. Case 9-2010:
a 37-year-old woman with paresthesias and ataxia. N Engl J
Med. 2010;362:1129–1138.
18. Dinkin M, Lavi E, Winterkorn J, Oliveira C, Merkler AE. Ischemic
optic neuropathy secondary to intravascular lymphoma. J
Neuroophthalmol. 2019;39:313–318.
19. Nizamutdinov D, Patel NP, Huang JH, Fonkem E. Intravascular
lymphoma in the CNS: options for treatment. Curr Treat
Options Neurol. 2017;19:35.
20. Ponzoni M, Campo E, Nakamura S. Intravascular large B-cell
lymphoma: a chameleon with multiple faces and many masks.
Blood. 2018;132:1561–1567.
21. Asada N, Odawara J, Kimura S, Aoki T, Yamakura M, Takeuchi
M, Seki R, Tanaka A, Matsue K. Use of random skin biopsy for
diagnosis of intravascular large B-cell lymphoma. Mayo Clin
Proc. 2007;82:1525–1527.
22. Satoh S, Yamazaki M, Yahikozawa H, Ichikawa N, Saito H,
Hanyuu N, Hata S, Hachyou M. Intravascular large B cell
lymphoma diagnosed by senile angioma biopsy. Intern Med.
2003;42:117–120.
23. Ponzoni M, Ferreri AJM, Campo E, Facchetti F, Mazzucchelli L,
Yoshino T, Murase T, Pileri SA, Doglioni C, Zucca E, Cavalli F,
Nakamura S. Definition, diagnosis, and management of
intravascular large B-cell lymphoma: proposals and
perspectives from an international consensus meeting. J Clin
Oncol. 2007;25:3168–3173.
24. Yamada S, Nishii R, Oka S, Higashi T, Yagi M, Satow T, Suzuki
T, Saiki M. FDG-PET a pivotal imaging modality for diagnosis of
stroke-onset intravascular lymphoma. Arch Neurol.
2010;67:366–367.
25. Liu Y, Wang L, Liao WJ, Wang G. Intravascular large B cell
lymphoma. J Clin Dermatol. 2016;45:259–261.
26. Wu H, Said JW, Ames ED, Chen C, McWhorter V, Chen P, Ghali
V, Pinkus GS. First reported cases of intravascular large cell
lymphoma of the NK cell type: clinical, histologic,
immunophenotypic, and molecular features. Am J Clin Pathol.
2005;123:603–611.
27. Khalidi HS, Brynes RK, Browne P, Koo CH, Battifora H,
Medeiros LJ. Intravascular large B-cell lymphoma: the CD5
antigen is expressed by a subset of cases. Mod Pathol.
1998;11:983–988.
28. Zuckerman D. Intravascular lymphoma: the oncologist’s “great
imitator.” Oncologist. 2006;11:496–502.
29. Ferreri AJM, Dognini GP, Bairey O, Szomor A, Montalbán C,
Horvath B, Demeter J, Uziel L, Soffietti R, Seymour JF,
Ambrosetti A, Willemze R, Martelli M, Rossi G, Candoni A, De
Renzo A, Doglioni C, Zucca E, Cavalli F, Ponzoni M;
International Extranodal Lymphoma Study Group. The addition
of rituximab to anthracycline-based chemotherapy significantly
improves outcome in “Western” patients with intravascular
large B-cell lymphoma. Br J Haematol. 2008;143:253–257.
30. Baehring JM, Longtine J, Hochberg FH. A new approach to the
diagnosis and treatment of intravascular lymphoma. J
Neurooncol. 2003;61:237–248.
31. Meissner J, Finel H, Dietrich S, Boumendil A, Kanfer E, Laboure
G, Abecasis M, Cornelissen J, Delage J, Finke J, Hess U,
Ludwig H, Mohty M, Pabst T, Pioltelli P, Robinson S, Samaras
P, Montoto S, Dreger P. Autologous hematopoietic stem cell
transplantation for intravascular large B-cell lymphoma: the
European Society for Blood and Marrow Transplantation
experience. Bone Marrow Transpl. 2017;52:650–652.
32. Shimada K, Kinoshita T, Naoe T, Nakamura S. Presentation
and management of intravascular large B-cell lymphoma.
Lancet Oncol. 2009;10:895–902.

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