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1: Leuk Lymphoma. 2004 Aug;45(8):1691-3.
Fulminant intravascular lymphoma presenting as fever of unknown origin.
Zeidman A, Horowitz A, Fradin Z, Cohen A, Wolfson L, Elimelech O.
Department of Internal Medicine B, Rabin Medical Center, Petah Tikva, Sackler School of Medicine, Tel-Aviv University, Israel. alizaz@clalit.org.il
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.
2: Dermatology. 2004;209(2):135-7.
Senile angioma-like eruption: a skin manifestation of intravascular large B cell lymphoma.
Motegi S, Tamura A, Takeuchi Y, Ishikawa O.
Department of Dermatology, Gunma University Graduate School of Medicine, Gunma, Japan. smotegi@showa.gunma-u.ac.jp
Intravascular large B cell lymphoma (IVL) is a rare disease characterized by intravascular proliferation of neoplastic mononuclear cells within the vascular lumina. The diagnosis requires skin, liver, renal, meningeal, or brain biopsy, but is rarely made antemortem. We report a 67-year-old man in whom the histological examination of senile angioma-like eruption enabled us to establish the diagnosis of IVL. In a suspected case of IVL, a skin biopsy of the senile angioma-like eruption may be beneficial for an early diagnosis.
3: Am J Hematol. 2004 Jul;76(3):236-9.
Usefulness of FDG-PET to diagnose intravascular lymphomatosis presenting as fever of unknown origin.
Hoshino A, Kawada E, Ukita T, Itoh K, Sakamoto H, Fujita K, Mantani N, Kogure T, Tamura J.
Department of General Medicine, Gunma University Hospital, 3-39-15 Showa-machi, Maebashi, Gunma 371-8511, Japan. linmei131@yahoo.co.jp
Intravascular lymphomatosis (IVL) is a rare systemic disease characterized by proliferation of lymphoid cells within the lumina of small arteries, veins, and capillaries. Diagnosis requires skin, liver, lung, bone marrow, renal, meningeal, or brain vessel biopsy but is often made only when the illness has progressed or post mortem because early involvement of organs was not evident. We report a case of IVL presenting as fever of unknown origin (FUO). In this case, gallium scintigraphy and computed tomography (CT) showed no evidence of malignancy, whereas (18)F-fluorodeoxyglucose positron emission tomography (FDG-PET) showed increased FDG uptake in the sternum, left and right vertebra, humerus, femur, and especially ilium. The diagnosis was made on iliac bone marrow biopsy examination. FDG-PET was useful for the detection of spread of disease in a patient with IVL suffering from FUO.
4: J Clin Pathol. 2003 Jun;56(6):468-70.
Intravascular lymphomatosis.
Lui PC, Wong GK, Poon WS, Tse GM.
Department of Pathology, United Christian Hospital, Hong Kong.
Intravascular lymphomatosis (IVL) is a rare angiotrophic large cell lymphoma producing vascular occlusion of arterioles, capillaries, and venules. Antigenic phenotyping shows that these lymphomas are mostly of B cell type, and less commonly T cell or Ki-1 lymphomas. The central nervous system and skin are the two most commonly affected organs; patients usually present with progressive encephalopathy with mental status changes and focal neurological deficits and skin petechia, purpura, plaques, and discolouration. Other involved organs include adrenal glands, lungs, heart, spleen, liver, pancreas, genital tract, and kidneys. Bone marrow, blood, cerebrospinal fluid, and lymph nodes are typically spared. Fever of unknown origin is another common presentation. Only one case of IVL presenting with disseminated intravascular coagulation and anasarca (generalised oedema) has been reported in the literature. This report describes a postmortem case of a patient with IVL who initially presented with disseminated intravascular coagulation complicated by intracerebral haemorrhage. |
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