Fulminant Intravascular Lymphoma Presenting as Fever of Unknown Origin

Abstract

Intravascular large cell lymphoma (IVLL) is a rare neoplasm characterized by the proliferation of malignant lymphoid cells within the lumens of small to medium-sized blood vessels. The central nervous system, skin, and endocrine systems are most commonly involved. IVLL may disseminate to the heart, pancreas, liver, spleen, kidneys, and adrenal glands. We report a 52-year-old patient who was admitted for fever of unknown origin for 3 weeks, jaundice and abnormal liver function tests. Fever, high levels of bilirubin, severe anemia, thrombocytopenia, and a very fulminant course characterized the clinical picture. Although bone marrow involvement is quite rare, the diagnosis of IVLL in this patient was done by bone marrow biopsy. The patient was treated with CHOP protocol and received the first course but the aggressive disease led to the patient's death.

A 63‐year‐old Man With Dementia, Ataxia and Vi Nerve Palsy

“…The determinant histologic feature of IVLL is the intraluminal presence of tumor cells and thus the extensive extravascular involvement of CNS with diffuse infiltration of lymphoma cells seen in our cases is remarkable as this finding is described in only 13 cases in the literature (4). Beside the mass in the CNS, the large tumor in the adrenal glands and surrounding tissue is a known feature of IVLL (2, 8) and probably contributed to the fever (8) that is often encountered in IVLL (9). This is the first published case of an intravascular B‐cell lymphoma to become predominantly manifest as a large sellar tumor mass.…”

FDG-PET imaging to detect and characterize underlying causes of fever of unknown origin: an unavoidable path for the foreseeable future

“…Therefore, FDG-PET imaging may prove to be superior in detecting disease sites compared to pelvic CT and MRI, which often appear negative [36,37]. In addition to primary splenic lymphoma, rare sites of involvement such as colonic lymphoma and pituitary lymphoma have been found to cause FUO and have been effectively detected by FDG-PET [38,39]. In rare cases, FDG-PET will yield false-negative results in carcinoid tumor Fig.…”

Molekulare Pathogenese primär systemischer Vaskulitiden

“…Andere Formen der Kleingefäßbeteiligung sekundärer Vaskulitiden sind die nicht maligne, lymphozytische Vaskulitis und die leukämische Vaskulitis, die im Rahmen von lympho-und myeloproliferativen Erkrankungen auftreten können [45,35]. Hiervon zu trennen sind die seltenen, intravaskulären (angiotropen), großzelligen B-und T-Zell Lymphome (früher: maligne Angioendotheliomatose), die die Gefäßwände nicht infiltrieren, jedoch Vaskulitismanifestationen imitieren können oder in seltenen Fällen mit einer lymphozytären oder leukozytoklastischen Vaskulitis einhergehen können [80]. Riesenzellarteriitis und Polymyalgia rheumatica sind mit malignen Erkrankungen assoziiert.…”