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.