T. Ninomiya et al. / Open Journal of Internal Medicine 2 (2012) 15-18
18
diagnosed with AIVL on the basis of Murase’s criteria
for AIVL [2-4]. Although we could not obtain histopa-
thological confirmation of malignant lymphoma or he-
mophagocytosis on the first admission, his symptoms
were suspected to be caused by AIVL.
The clinical course of AIVL is typically very aggres-
sive. However, a few reports have demonstrated an indo-
lent type of IVLBCL. Ishiko et al. reported that a 59-
year-old woman with ataxia and gait disturbance after a
3-year history of serum LDH elevation was finally diag-
nosed with IVLBCL by brain biopsy [5]. They suggested
that there are certain IVLBCL patients with brain in-
volvement who present with relatively slow progression,
similar to a low-grade lymphoma [5]. Nakao et al. re-
ported that a 62-year-old woman with slowly developing
paraplegia and a 7-month history of recto-urinary dys-
function was diagnosed with IVLBCL by autopsy [6]. In
that case, lymphoma cells were only identified in the
bone marrow and blood vessels of the cauda equina. Ad-
ditionally, Sekine et al. reported that a 71-year-old man
with respiratory failure after developing intermittent fe-
ver and a high serum LDH for 7 months was diagnosed
with IVLBCL by lung biopsy [7]. In that case, lym-
phoma cells were identified in the cauda equina and lung.
From these different lines of evidence, we suggest that
IVLBCL may have a slow progressive clinical course
when lymphoma cells are present in limited organs. Al-
though we could not show the limited lymphoma cell
involvement in a specific organ, our case might be one of
indolent AIVL similar to these previous reported cases.
In two of three previous cases, a diagnosis of IVLBCL
was established in living patients who were treated using
CHOP (cyclophosphamide, doxorubicin, vincristine, pred-
nisone) with rituximab and showed complete remission.
In the other case and in our case, the diagnosis was es-
tablished at autopsy. IVLBCL is difficult to diagnose
because patients generally show little or no tumor mass
formation and more than half of patients are diagnosed
postmortem. Recently, some reports have suggested that
a random skin biopsy may be efficacious for the early
diagnosis of IVLBCL. Asada et al. reported that early
diagnosis of IVLBCL was possible by random skin bi-
opsy and the early institution of rituximab-based chemo-
therapy induced a favorable response [8]. In our case, a
liver biopsy or random skin biopsy should have been
performed even after the high fever and thrombocyto-
penia disappeared.
In conclusion, we present a case of AIVL with an un-
usual clinical course and spontaneous improvement. We
suggest that an indolent clinical course or spontaneous
improvement does not exclude AIVL, although most
AIVL cases are aggressive and progressive. Importantly,
AIVL should be treated immediately with intensive ex-
amination for diagnosis, even though the clinical course
is apparently indolent.
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