Rapid deterioration of intravascular large B-cell lymphoma with mass formation in the trigeminal nerve and multiple organ infiltration: An autopsy case report

Abstract

Intravascular large B-cell lymphoma (IVLBCL) is characterized by lymphoma cell invasion in the lumen of vessels, particularly in capillaries. 1 IVLBCL widely disseminates to extranodal sites, including the skin and bone marrow (BM). 2,3 Among the many clinical presentations of IVLBCL, B symptoms are a common manifestation, occurring in 54-76% of patients. [4][5][6] Although the prognosis of IVLBCL is poor due to the frequent delay in early diagnosis, random skin biopsy (RSB) and BM biopsy (BMB) have improved the accuracy of diagnosis, resulting in the prompt initiation of chemotherapy and improved outcomes. 1,3,5,6 However, the results, including flow cytometry and pathological findings of these samples, are often unhelpful in making a diagnosis.We present the case of a patient with IVLBCL resulting in mass formation in the trigeminal nerve (TN). This case had concurrent abnormal clinical manifestations, such as failure to detect IVLBCL by RSB and BMB, resulting in death due to rapid deterioration. Autopsy revealed unique IVLBCL invasion of the TN, left lumbar plexus, and lumen of vessels of multiple organs, excluding the skin and BM. CASE REPORTA 69-year-old man was transferred to our hospital because of progressive headache and numbness in his left face for a month. Although an anti-inflammatory analgesic was administered, pain and B symptoms, such as fever and unintentional weight loss of 16% over 6 months, were not relieved. Neither the patient nor his family had a remarkable medical history Physical examination, including neurological examination, revealed no abnormalities, though he had severe allodynia in the left side of his face.