intravascular large B-cell lymphoma(redirected from haemangioendotheliosmatosis)
intravascular large B-cell lymphomaA 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.
Often begins with fever of unknown origin and nonspecific cutaneous (e.g., plaques) and neurologic complaints.
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.
Extremely poor; short survival is the norm despite aggressive chemotherapy. The diagnosis is often established postmortem.
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