intravascular large B-cell lymphoma

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intravascular large B-cell lymphoma

A high-grade non-Hodgkin lymphoma arising in and generally confined to vascular lumina, which usually begins in the skin and CNS and rapidly spreads elsewhere.

Clinical findings
Often begins with fever of unknown origin and nonspecific cutaneous (e.g., plaques) and neurologic complaints.
 
DiffDx
Skin lesions (erythematous/purple plaques on the trunk and lower legs) may be confused with mycosis fungoides, sarcoidosis, vascular neoplasms (e.g., Kaposi sarcoma) or involvement by lymphoma or leukaemia.

Prognosis
Extremely poor; short survival is the norm despite aggressive chemotherapy. The diagnosis is often established postmortem.
References in periodicals archive ?
Intravascular lymphoma masquerading as Vogt-Koyanagi-Harada syndrome Ocul Immunol Inflamm.
42) However, the "other" category consists of rarer lesions that can be subcategorized into well-known morphologic entities: T-cell/histiocyte-rich large B-cell lymphoma (TC/HR LBCL), plasmablastic lymphoma, and intravascular lymphoma.
Intravascular lymphoma presenting as an orbital mass lesion: a case report.
Test results revealed intravascular lymphoma, a type of cancer.
There was a prominent component of large atypical cells within the dilated vascular channels (figure, A), which initially raised the possibility of an intravascular lymphoma or sarcoma.
Intravascular lymphoma was first described in 1959 by Pfleger and Tappeiner (1) in Germany.
5) Intravascular lymphoma is most frequently a disease of B lymphocytes, although rare cases of thymus-cell (T-cell) and natural killer-cell disease have been reported; (6) the World Health Organization considers these cases to be a separate entity.
Rare cases of intravascular lymphoma have the phenotype of T cells, but most are [CD20.

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