intravascular large B-cell lymphoma

(redirected from angioendotheliomatosis proliferans)

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 ?
Initially described by Pflegrand and Tappeiner in 1959 as "angioendotheliomatosis proliferans systemisata" and in 2008 defined by the World Health Organization (WHO) as intravascular large B cell lymphomas (IVBCL), IVL is a type of extra-nodal large B cell lymphoma where growth is restricted to the lumina of the vessels, particularly the capillaries [7].
Believing that the intravascular growth of malignant cells represented a neoplasm of the vascular endothelium, they described the disease as "angioendotheliomatosis proliferans systemisata." In 1986 Sheibani et al (2) proved, via immunohistochemical investigation, that the cells of intravascular lymphoma were of lymphoid origin and described the disease as angiotropic (intravascular) large-cell lymphoma.
Angioendotheliomatosis proliferans systemisata [Angioendotheliomatosis proliferans systematisata: a clinically and pathohistologically new disease picture].