intravascular lymphomatosis

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.

intravascular lymphomatosis

Angiotropic large cell lymphoma, malignant angioendotheliomatosis, neoplastic angioendotheliomatosis Oncology A lymphoma in which the lymphocytes home to vascular endothelium, possibly related to expression of specific lymphocyte-endothelium adhesion molecules Prognosis Poor; most die, often before diagnosis
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References in periodicals archive ?
Intravascular lymphomatosis presenting as adult respiratory distress syndrome.
Epstein-Barr virus-associated intravascular lymphomatosis within Kaposi's sarcoma in an AIDS patient.
Lack of CD 29 (beta 1 integrin) and CD 54 (ICAM-1) adhesion molecules in intravascular lymphomatosis.
Intravascular lymphomatosis (malignant angioendotheliomatosis): a B-cell neoplasm expressing surface homing receptors.
In the past, the term angioendotheliomatosis was used to describe a condition that has now been renamed intravascular lymphomatosis (IVL).
Intravascular lymphomatosis (IVL) is a rare entity reported initially by Pfleger and Tappeiner in 1959.
Intravascular lymphomatosis typically presents as a primary intravascular neoplasm without a solitary component; however, it rarely can result from a disseminated lymphoma.
Intravascular lymphomatosis usually presents as a primary intravascular neoplasm without known primary lymphoma, although it may also occur as a manifestation of disseminated disease.
Intravascular large B-cell lymphoma (IVLBL) is an uncommon form of non-Hodgkin lymphoma that is also known as malignant angioendotheliosis, intravascular lymphomatosis, and angiotropic large-cell lymphoma.
Intravascular large B-cell lymphoma is an uncommon lymphoid neoplasm, which has been alternatively designated as angiotropic large cell lymphoma, intravascular lymphomatosis, or malignant angioendotheliosis.

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