Baylor University Medical Center Proceedings April 2017 - 188

a

b

Figure 2. (a) Bone marrow, hematoxylin and eosin stain, and (b) CD68 immunohistochemical stain highlighting
numerous hemophagocytic histiocytes.

Table 1. Comparison between sites of disease and laboratory findings in Asian and Western
variants of intravascular large B-cell lymphoma*
Asian variant
(n = 96)

Western variant
(n = 38)

P
value

76%

55%

0.018

Bone marrow

75%

32%

<0.001

Spleen

67%

26%

<0.001

Liver

55%

26%

0.003

Peripheral blood

24%

5%

0.012

Central nervous system

27%

39%

0.171

Skin

15%

39%

0.002

Lymph node

11%

11%

0.99

Variable
B symptoms
Sites of disease

Laboratory findings
Anemia (hemoglobin < 12 g/dL)

78%

63%

0.075

Thrombocytopenia (platelet < 150 × 109/L)

76%

29%

<0.001

Elevated serum lactate dehydrogenase

93%

86%

0.278

Hypoalbuminemia (< 36 g/L)

84%

18%

<0.001

*Reprinted from Ponzoni et al, 2007 (5) with permission from the American Society of Clinical Oncology. Data on the Asian
variant are from Murase et al, 2007 (7), and data on the Western variant are from Ferreri et al, 2004 (2).

Table 2. Criteria for the diagnosis of Asian-variant intravascular lymphoma*
Category

Criteria

1. Clinical and laboratory
* Cytopenia (not caused by hypoplastic or dysplastic marrow), affecting at
criteria (at least 2 of the 3
least one of the two lineages, i.e., erythrocytes and platelets. Leukocytes
are required)
are not included. It is prescribed by hemoglobin (<11 g/dL) or red blood
cells (<3.5 × 1012/L) and/or platelet count (<100 × 109/L).
* Hepatomegaly and/or splenomegaly, identified by computed tomography,
ultrasonography, or physical examination.
* Absence of overt lymphadenopathy and tumor formation.
2. Histopathological criteria
(all 3 terms are required)

* Erythrocyte-hemophagocytosis; usually seen mildly or moderately in hematopoietic
system.
* Immunophenotypical evidence of proliferating neoplastic B cells with large
cell morphology.
* Pathological findings of intravascular proliferation and/or sinusoidal involvement
of lymphoma cells.

*Reprinted from Murase et al, 2000 (4) with permission from John Wiley & Sons.

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marrow, central nervous system,
spleen, liver, gastrointestinal tract,
adrenal glands, skin, and others,
though lymphadenopathy is usually
absent (2-4). Despite its diffuse vasculature growth pattern, peripheral
blood involvement is seen in only 5%
to 9% of patients (5).
The two distinct variants of
IVLBCL-a hemophagocytic Asian
variant and a classical Westernvariant-were described through
examination of patient case series in
Japan (4, 7) and Italy (2), respectively
(Table 1) (5). Both variants appear to
affect men and women equally and
present with B symptoms. Central
nervous system involvement is manifested by heterogeneous symptoms of
sensory and motor deficits, seizures,
vertigo, and altered mentation and is
more common in the Western variant (2, 7). The Asian variant more
frequently involves the liver, spleen,
and bone marrow, while skin involvement occurs more often in the Western variant (2, 7). The Asian variant
IVLBCL is also strongly associated
with HLH (7), whereas HLH is not
typically seen in the Western variant
(2, 8). Skin lesions vary widely in
morphology and distribution and are
commonly located in the upper arms
and legs, low abdomen, and breast
(2). Interestingly, the Asian variant is
rarely seen outside of Asian patient
populations. To date, there have
only been occasional case reports
of IVLBCL with hemophagocytic
syndrome in Caucasian patients (9,
10) and only one other case report of
Asian-variant IVLBCL in a patient of
African descent (11).
Diagnostic criteria for Asianvariant IVLBCL have been proposed;
these are summarized in Table 2 (4,
12). Workup of IVLBCL should
include a search for suspicious cutaneous lesions for biopsy, peripheral
blood smear, and routine blood tests.
Given anecdotal reports of cerebrospinal fluid involvement, lumbar
puncture with cerebrospinal fluid examination is recommended, whereas
bone marrow biopsy may reveal
lymphomatous or hemophagocytic
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