intravascular large B-cell lymphoma


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Related to intravascular large B-cell lymphoma: Primary effusion lymphoma

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.
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References in periodicals archive ?
Kimura et al., "Use of random skin biopsy for diagnosis of intravascular large B-cell lymphoma," Mayo Clinic Proceedings, vol.
Govi et al., "Can rituximab change the usually dismal prognosis of patients with intravascular large B-cell lymphoma?," Journal of Clinical Oncology, vol.
Routy et al., "Retrospective study of intravascular large B-cell lymphoma cases diagnosed in Quebec," Medicine, vol.
Gaur, "A Case of De Novo CD5+ Disseminated Intravascular Large B-Cell Lymphoma Presenting as Multiorgan Failure," Case Reports in Hematology, vol.
Arbini, "Intravascular large B-cell lymphoma with multi-organ failure presenting as a pancreatic mass: a case with atypical presentation and definite diagnosis postmortem," Autopsy and Case Reports, vol.
In Table 2 we compare LBCL-BLS as defined in this manuscript (LBCL involving BM, liver, and/or spleen but lacking intravascular or sinusoidal involvement and lacking nodal or other extranodal disease) with intravascular large B-cell lymphomas (Western and Asian types) and THRLBCL [1].
Intravascular large B-cell lymphoma cells have been demonstrated to lack cell surface proteins critical to lymphocyte transvascular migration, including CD29 ([beta]1 integrin) and CD54 (ICAM-1 or CD11a ligand).
Intravascular large B-cell lymphoma cells irregularly express several immature and mature B-cell antigens, and may be of a germinal center (20%) or nongerminal center (80%) B-cell immunophenotype.
Intravascular large B-cell lymphoma is a rare type of extranodal large B-cell lymphoma.
Intravascular large B-cell lymphoma: the CD5 antigen is expressed by a subset of cases.

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