Baylor University Medical Center Proceedings April 2017 - 187

a

b

c

d

e

f

g

h

cardia and vasopressor-refractory shock, worsening
acidosis, and shock liver.
On hospital day 7, the
family opted for comfortoriented care, and shortly
after palliative extubation,
the patient died.
Autopsy disclosed the
spleen to weigh 500 g (normal 150-200 g) and liver
2750 g (normal ≤1700 g)
but found no discrete
masses, enlarged lymph
nodes, or skin lesions.
Microscopic examination revealed widespread,
multiorgan capillary infiltration by enlarged, atypical lymphocytes. Organs
involved included vessels
throughout the brain and
brainstem, lungs, vaso vasorum of the aorta, epicardiac and cardiac vessels,
kidneys, adrenal glands,
gastrointestinal tract,
psoas muscle, gallbladder,
pancreas, urinary bladder,
spleen, liver, and bone marrow (Figures 1 and 2). Immunohistochemical stains
showed the atypical intravascular lymphocytes to be
immunoreactive for CD5,
CD20, Bcl-2 (focally positive), and MUM1 (subset)
and negative for Bcl-1, Bcl6, CD10, and CD30. The
bone marrow also revealed
extensive CD68+ hemophagocytic histiocytes
(Figure 2). The final diagnosis and cause of death
was IVLBCL with hemophagocytic syndrome and
multiple organ failure.
DISCUSSION
IVLBCL is classified by
the World Health Organization as a rare type of extranodal large B-cell lymphoma characterized by the selective growth of lymphoma cells within vessel
lumina-particularly capillaries-with the exception of large
arteries and veins. Historically, 53% of patients are diagnosed on
autopsy (1). IVLBCL involves all organ types, including bone

Figure 1. Representative hematoxylin and eosin sections of atypical intravascular lymphocytes infiltrating capillaries of (a) lung,
(b) thalamus, (c) esophagus, (d) bladder, (e) pancreas, and (f) psoas muscle. CD20 stain of (g) spleen and (h) bladder.

hematopoiesis, mild megakaryocytic hyperplasia, and rare hemophagocytic cells. As the patient met criteria for hemophagocytic
lymphohistiocytosis (HLH), he was started on dexamethasone
10 mg/m2 intravenously (6). Unfortunately, prior to etoposide
administration, the patient developed supraventricular tachyApril 2017

Asian-variant intravascular large B-cell lymphoma

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