Intravascular lymphoma (IVL) is a rare type of extranodal lymphoma with an aggressive
clinical course characterized by proliferation of large lymphoma cells within the
lumina of the small vessels. Because of its varied clinical symptoms and the absence
of lymphadenopathy, diagnosis of IVL is extremely difficult and requires histological
confirmation. We report here 6 consecutive patients with IVL, admitted to Kameda General
Hospital, Kamogawa-shi, Japan, from June 7, 2006, to February 28, 2007, whose IVL
was diagnosed by random skin biopsy of healthy-appearing skin. Three patients presented
with progressive neurological deterioration and 2 others with hypoxemia with interstitial
infiltration on chest radiography. One patient presented with confusion and severe
hypoxia without apparent interstitial infiltration. Two patients showed localized
skin involvement. Irrespective of the presence of skin lesions, almost all skin biopsy
specimens showed obliteration of small vessels of subcutaneous fat tissues by lymphoma
cells, allowing a prompt diagnosis of IVL. Early institution of rituximab-based chemotherapy
induced favorable responses in all patients treated. Because diagnosis based on tissue
other than skin is usually difficult in patients with suspected IVL, random skin biopsy
should be considered even in patients with no evident skin lesions.
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© 2007 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.
