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Intravascular Large B Cell Lymphoma Presenting as Fever of Unknown Origin and Diagnosed by Random Skin Biopsies: A Case Report and Literature Review

Challenging differential diagnosis, Rare disease, Educational Purpose (only if useful for a systematic review or synthesis)

Horacio di Fonzo, Damian Contardo, Diego Carrozza, Paola Finocchietto, Adriana Rojano Crisson, Cecilia Cabral, Maria de los Angeles Juarez

Argentina Department of Internal Medicine, Hospital de Clinicas “Jose de San Martin”, University of Buenos Aires, Buenos Aires, Argentina

Am J Case Rep 2017; 18:482-486

DOI: 10.12659/AJCR.903816

Available online:

Published: 2017-05-02

BACKGROUND: Intravascular lymphoma (IVL) is a rare lymphoproliferative disorder characterized by the proliferation of large B lymphoma cells within the lumen of small-caliber blood vessels. Clinical features are nonspecific, presenting as a systemic disease with fever and may be life-threatening. Antemortem diagnosis is difficult but may be made with biopsies of affected tissues or with random skin biopsies.
CASE REPORT: We report the case of a 66-year-old white woman presenting with fever of unknown origin (FUO) who developed neurologic, pulmonary, and hematologic manifestations. The diagnosis of intravascular large B cell lymphoma (IVLBCL) was made by random skin biopsies. She received treatment with steroids, rituximab, cyclophosphamide, vincristine, and doxorubicin (R-CHOP). Her disease evolution was unfavorable and she died after her first cycle of chemotherapy.
CONCLUSIONS: Our case illustrates that IVL can present as FUO and should be considered in the differential diagnosis of this syndrome, especially in patients with neurologic compromise and persistently elevated serum lactate dehydrogenase. In this case, the diagnosis was made with cutaneous biopsies of visibly unaffected skin. As in our patient, the course of IVL is usually fatal within a few months. 

Keywords: Fever of Unknown Origin, Lymphoma, B-Cell, Lymphoproliferative Disorders