Baylor University Medical Center Proceedings April 2017 - 186

Asian-variant intravascular large B-cell lymphoma
Derrick W. Su, MD, Whitney Pasch, DO, Cristina Costales, MD, Imran Siddiqi, MD, and Ann Mohrbacher, MD

Intravascular large B-cell lymphoma (IVLBCL) is a rare and deadly
malignancy involving the growth of lymphoma cells within vessel lumina
of all organ types. IVLBCL is further divided into the hemophagocytic
Asian variant and a classical Western variant. Both variants are difficult
to diagnose by imaging, and although diagnostic criteria have been developed to guide workup, histopathological examination remains imperative. Treatment of IVLBCL remains difficult given the high mortality of the
disease, but rituximab has emerged as a promising therapeutic option
when combined with various cytotoxic regimens. The two main variants
of IVLBCL generally manifest in their respective Asian or Western populations, and crossover between ethnicities is rare. We present the second
described case of Asian-variant IVLBCL in an African American individual.

ntravascular large B-cell lymphoma (IVLBCL) is a rare large
B-cell lymphoma that often has a poor clinical outcome (1).
Despite its propensity to involve all organ types in a diﬀuse
vascular growth pattern, IVLBCL is diﬃcult to diagnose
due to the lack of overt lymphadenopathy and peripheral blood
involvement (2-5). Asian and Western variants of IVLBCL
share similar features, but generally diﬀer in presentation (5).
We present a rare case of Asian-variant IVLBCL in a black man.
CASE PRESENTATION
A 69-year-old black man with coronary artery disease and
chronic kidney disease presented to an outside facility with
2 weeks of B symptoms. He had no smoking, tobacco, or illicit
drug history and was active and functional prior to these symptoms. His pertinent travel history included military service in
Vietnam during the Vietnam War. He had no known family
history of malignancies and no known Asian family ancestry.
After initially leaving against medical advice, he presented to
another facility where he developed refractory hypotension and
fevers, acute-on-chronic kidney disease progressing to end-stage
renal disease requiring hemodialysis, and progressive pancytopenia. An extensive workup ruled out many bacterial, fungal,
parasitic, viral, and atypical infections, and a bone marrow
biopsy revealed no infection, tuberculosis, or evidence of malignancy. Thrombotic thrombocytopenic purpura was ruled
out by peripheral blood smear examination and ADAMTS13
testing. Given the patient's progressive deterioration and lack

186

of etiology for his fevers, he was transferred to our facility for
a higher level of care.
On transfer, his temperature was 39.2°C (102.6°F);
pulse rate, 122 beats per minute; and systolic blood pressure,
70 mm Hg. He had mild bilateral upper lobe wheezing, epigastrium tenderness, and marked pretibial pitting edema in
the lower extremities bilaterally. There was no skin rash or
hepatosplenomegaly. Laboratory studies revealed leukocytosis of 11.2 × 103/μL (range 3.8-10.8) with a neutrophil predominance, a hemoglobin of 9.2 g/dL (range 13.8-17), and
platelets of 21 × 103/μL (range 135-400). Blood urea nitrogen
and creatinine were 87 mg/dL (range 8-23) and 5.0 mg/dL
(range 0.67-1.17), respectively. The aspartate transaminase was
264 U/L (range 0-40); alanine transaminase, 42 U/L (range
0-41); alkaline phosphatase, 371 U/L (range 40-130); total
bilirubin, 7.4 mg/dL (normal high ≤1.0); and direct bilirubin, 5.6 mg/dL (range 0.0-0.3). Lactate dehydrogenase was
2240 U/L (range 135-225), D-dimer was 4375 ng/mL (normal
high ≤250.0), and ferritin was 15,219 ng/mL (range 30-400).
Fibrinogen was 293 mg/dL (range 201-485). Peripheral blood
ﬁlm revealed one to two schistocytes per high-powered ﬁeld,
anisocytosis, numerous shift cells, large granular lymphocytes,
large platelets, and nucleated red blood cells.
The patient was treated with broad-spectrum antibiotics and
antifungals, continuous renal replacement therapy for refractory
hypotension, and intubation for progressive altered mentation
and inability to protect the airway. Computed tomography disclosed hepatomegaly (24 cm), splenomegaly (14 cm), bilateral
nephromegaly (right kidney 13 cm, left kidney 15 cm), and
bilateral adrenal enlargement without evidence of a focal mass.
A serum protein electrophoresis was negative for paraproteins,
and a peripheral blood leukemia and lymphoma ﬂow cytometry
panel identiﬁed predominantly mature T cells with fewer NK
cells and polyclonal B cells, but no clonal population. Bone marrow biopsy showed a mildly hypocellular marrow with trilineage
From the Jane Anne Nohl Division of Hematology (Su, Mohrbacher) and the
Hematopathology Section, Department of Pathology (Pasch, Costales), Norris
Comprehensive Cancer Center and Hospital, University of Southern California,
Los Angeles, California.
Corresponding author: Derrick W. Su, MD, 1441 Eastlake Avenue, Los Angeles,
CA 90033 (e-mail: derrick.su@med.usc.edu).
Proc (Bayl Univ Med Cent) 2017;30(2):186-189

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