Successful diagnosis of intravascular large B-cell lymphoma from a neuromyelitis optica presentation in a patient with primary Sjögren’s syndrome: a case report

Abstract

Abstract Background Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune neurological disorder characterized by the presence of serum anti-aquaporin-4 autoantibodies (AQP4 IgG). There have been previous reports of NMOSD associated with primary Sjögren’s syndrome (pSS), with syringomyelia, in a small number of NMOSD cases. We report here the case of a patient with NMOSD and pSS who presented with various clinical manifestations, including long segmental cavitated changes in the spinal cord, who was finally diagnosed with intravascular large B-cell lymphoma (IVLBCL) through adrenal biopsy. Case presentation An 80-year-old Chinese woman was admitted to the hospital with weakness in her extremities for half a month and progressive deterioration with urinary and bowel functions for a week. Cervical and thoracic magnetic resonance imaging (MRI) of the patient indicated abnormal C2-T2 signals. Considering these findings in combination with clinical data, NMOSD was initially considered, and steroids and proglobulin were administered. However, the patient’s symptoms did not improve, and her level of consciousness gradually decreased. Subsequently, craniospinal MRI and abdominal computed tomography (CT) revealed multiple abnormal lesions. Adrenal biopsy revealed atypical lymphoid cell invasion of the small vessels consistent with IVLBCL, which was treated with cyclophosphamide, vincristine, doxorubicin, and prednisone (CHOP). Conclusions Currently, cases of IVLBCL combined with NMOSD and pSS are rare, and the myelopathy of this patient presented with long segmental cavity-like changes. Early diagnosis of such patients is difficult, misdiagnosis rate is high, conventional treatment is not effective, and deterioration rate is high. When the central nervous system is involved, the effect of conventional CHOP chemotherapy is poor; therefore, early diagnosis and early application of chemotherapy drugs are recommended.