Discussion and Conclusion
We report a 64-year-old Iranian man with intravascular large B cell
lymphoma who was referred to our clinic with paresthesia of distal lower
extremities and motor symptoms that developed subsequently. These
findings and later electromyography and nerve conduction studies were
clinically compatible with a multiple mononeuritis pattern of
involvement. The patient underwent a thorough work-up, the results of
which were inconclusive. A nerve biopsy was done, and the findings led
to a diagnosis of intravascular lymphoma.
Notably, our patient did not have any specific symptoms that
specifically pointed to a diagnosis of lymphoma. A few cases of
intravascular B-cell lymphoma have been reported that were associated
with multiple mononeuropathy during the course of the illness. However,
in most of these cases, MM was a late finding in the course of the
disease, following weeks to months after the diagnosis that is usually
based on other symptoms such as skin lesions, fever, or rigors [7, 15,
16]. Another case received an inaccurate diagnosis of vasculitis,
which was revealed only after autopsy [7].
Patients with intravascular lymphoma most commonly present with symptoms
related to the involvement of the central nervous system (39 percent)
and skin (39 percent). Fever and skin lesions are common [5, 8, 20].
Bone marrow (32 percent), spleen (26 percent), and liver (26 percent)
are less frequently involved [17]. Our patient did not have any
evidence of CNS involvement at presentation, nor did he have fever or
skin lesions. Also, bone marrow biopsy and aspiration did not show any
significant pathological changes. However, splenomegaly was seen in his
abdominal CT scan. Previous reports of similar cases are compatible with
associated infiltration of the spleen and liver [7].
More importantly, our patient presented with mononeuropathy multiplex
and weight loss without other symptoms. This is a rare presentation in
IVLBCL but has been previously reported [13, 16]. In a case series
of 26 patients with lymphoma-associated neuropathy, six patients had an
MM pattern [7, 21, 22]. Most of these patients had a favorable
hematological prognosis except for one patient who did not respond to
chemotherapy and died as a result of infectious complications of bone
marrow transplantation. Half of the patients experienced neurological
improvement after chemotherapy [16]. In general, the prognosis is
very poor for patients with intravascular lymphoma, with most of them
dying within one year of their diagnosis [7, 9, 15, 16]. Although
our patient’s neurological disability did not completely respond to
chemotherapy and he experienced a relapse of IVLBCL in the skin, he had
a favorable survival of more than two years after the initial
presentation, partly due to timely diagnosis and early treatment.
Our case report, in line with previous reports, highlights the
importance of considering neurolymphomatosis and intravascular lymphoma
as possible causes of MM. Specifically, a nerve biopsy with an
assessment of clonal perivascular infiltrates may aid clinicians in
differentiating between intravascular neoplastic infiltration from
vasculitis.
Declarations
Ethics approval and consent to
participate
The patient provided written informed consent for publication of this
case report.
Consent to publish
The patient provided written informed consent for publication of this
case report.
Data Access statement:
Data sharing does not apply to this article as no datasets were
generated or analyzed during the current study.
Authors’ contributions
BHA and MAD were the attending physicians who participated in patient
assessment and made the final diagnoses. PM and FB gathered and
organized the patient data, searched the literature, and composed the
manuscript. AZM was the attending pathologist who assessed the tissue
specimens and provided the diagnosis. All authors contributed to editing
the manuscript. All authors have read and approved the submitted version
of the manuscripts.
Acknowledgments
The authors did not receive any funding for this article. The authors
declare that they have no competing interests.