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 ?
Evidence in favor of its reclassification as "intravascular lymphomatosis"," American Journal of Surgical Pathology, vol.
Intravascular lymphomatosis presenting as adult respiratory distress syndrome.
Mann, "Intravascular lymphomatosis: a clinicopathologic study of 10 cases and assessment of response to chemotherapy," Journal of Clinical Oncology, vol.
Cox et al., "Pulmonary intravascular lymphomatosis: presentation with dyspnea and air trapping," Chest, vol.
Reassessment of malignant "angioendotheliomatosis." Evidence in favor of its reclassification as "intravascular lymphomatosis." Am J Surg Pathol.
Angiotropic large cell lymphoma (intravascular lymphomatosis) occurring after follicular small cleaved cell lymphoma.
Epstein-Barr virus-associated intravascular lymphomatosis within Kaposi's sarcoma in an AIDS patient.
Intravascular lymphomatosis: clinicopathologic features and differential diagnosis.
Intravascular lymphomatosis (IVL) is a rare entity reported initially by Pfleger and Tappeiner in 1959.[1] It is a variant of aggressive non-Hodgkin lymphoma characterized by a peculiar intravascular proliferation of morphologically neoplastic lymphoid cells.
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.

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