Abstract

Introduction Intravascular lymphoma (IVL) is an uncommon and often fatal disease characterized by intraluminal proliferation of lymphomatous cells within blood vessels. Because of a heterogeneous clinical presentation and lack of sensitive diagnostic protocols, diagnosis of IVL is most often made at autopsy. However, with early diagnosis and appropriate chemotherapy, the prognosis is greatly improved and complete remission is possible. In order to broaden the possible presentations of IVL, we present a patient with an atypical manifestation of biopsy-proven intravascular large B-cell lymphoma who suffered dissections of both intracranial and extracranial arteries in addition to progressive intracranial hemorrhages. Case Report. A 47-year-old woman presented with unilateral paresthesias. She developed progressive multifocal infarcts and hemorrhage with dissections of both intracranial and extracranial arteries, resulting in coma. Brain biopsy revealed IVL. She received aggressive chemotherapy and remains in complete remission with good neurologic recovery. Conclusion IVL is known to exert its pathology on small arteries and capillaries, but is not known to cause dissections of large vessels. The diagnosis should be considered in cases with unexplained arterial dissections and progressive strokes. Early diagnosis with appropriate laboratory screening and tissue confirmation by biopsy can lead to greatly improved outcomes.

Highlights

  • Intravascular lymphoma (IVL) is an uncommon and o en fatal disease characterized by intraluminal proliferation of lymphomatous cells within blood vessels

  • We describe an unusual presentation of IVL with multiple dissections of both intracranial and extracranial blood vessels and progressive intracranial hemorrhages (ICH)

  • IVL involving the central nervous system (CNS) is an aggressive condition with a poor prognosis because its protean clinical manifestations frequently preclude diagnosis

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Summary

Introduction

Intravascular lymphoma (IVL) is recognized by the World Health Organization (WHO) as an extremely rare subtype of di use extranodal large B-cell non-Hodgkin’s lymphoma with an estimated annual incidence of 0.5 cases per 1,000,000 [1]. IVL can involve any organ system, the central nervous system (CNS), and its protean neurological manifestations o en hinder diagnosis. Bone marrow involvement occurs in 32% of patients, and cerebrospinal uid (CSF) rarely contains malignant cells [6]. Organ biopsy is the gold standard for diagnosis of IVL but is frequently performed too late, and cases with CNS involvement are most. (b) Le to right: Carotid and cerebral angiography showing irregular corrugated and beaded appearance of the cervical carotid arteries and subtle intracranial vessel narrowing (arrows), and two new foci of restricted di usivity on di usion weighted imaging (DWI) in the bilateral centrum semiovale consistent with interval infarcts (arrows). We describe an unusual presentation of IVL with multiple dissections of both intracranial and extracranial blood vessels and progressive intracranial hemorrhages (ICH)

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