Intravascular Lymphomatosis Presenting as Fever of Unknown Origin with Peripheral Polyneuropathy / 감염과화학요법

ABSTRACT

Fever of unknown origin (FUO) is caused by infections (30-40%), neoplasms (20-30%), collagen vascular diseases (10-20%), and numerous miscellaneous diseases (15-20%). The literatures also reveal that 5-15% of FUO cases defy diagnosis, despite extensive studies. Diagnostic advances continuously modify the spectrum of FUO-causing diseases. Modern imaging techniques (e.g., ultrasound, CT scan, MRI) enable early detection of abscesses and solid tumors that used to be difficult to diagnose. But the correct diagnosis can be delayed because the tumor, such as rare hematologic malignancy, is difficult to be detected by various imaging studies. Intravascular lymphomatosis (IVL) is a rare form of non-Hodgkin's lymphoma and generally fatal disease characterized by extensive proliferation of neoplastic mononuclear cells within the lumina of blood vessels. The prognosis is usually extremely poor, with rapid death despite chemotherapy. The diagnosis is most frequently made after biopsy of skin or brain but is often established post mortem. Most patients with IVL present with FUO and nonspecific cutaneous and neurologic manifestations. Peripheral nerve system or pulmonary involvements are not common as initial menifestations. We report a case of intravascular lymphomatosis, presenting as FUO with peripheral polyneuropathy. We made early diagnosis of IVL by video-assisted thoracoscopic lung biopsy. She is improving with good performance after the 4th cycle of chemotherapy till now.