Intravascular cutaneous t-cell lymphoma C49.M

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 11.11.2021

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Angioendotheliomatosis proliferans systematisata; Endothelioma intravascular; Endotheliomatosis systematized; IVL; malignant angioendotheliomatosis

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Multisystemic disease with proliferation of multicentre intravascular tumour cell infiltrates, predominant involvement of the central nervous system and a poor prognosis despite therapy. It is a very rare manifestation of a malignant cutaneous T-cell lymphoma with multicenter intravascular proliferation of tumor cells (previously mistakenly considered atypical endothelial cells).

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Occurs mainly on the trunk, lower extremities and face.

Clinical features
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Multiple vessel occlusion and thrombosis. Almost always skin involvement: blue to brown-red, blurred, fingernail to palm sized plaques or nodules. Variable clinical picture. In addition, mostly infestation of the nervous system with various neurological deficits. Ultimately any organ can be affected. Mostly general symptoms (fever, fatigue).

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Intravascularly located pleomorphic T-cell lymphocytes with round or oval chromatin-tight nuclei. Blood vessels in the corium, partly also in the subcutis, are dilated and increasingly filled with highly pleomorphic, hyperchromatic cells. The cells may be embedded in fibrinous thrombi. Immunohistologically the cells are derived from T-lymphocytes (CD2, CD3, CD43 pos.; detection of a clonal lymphocyte population). They do not express antigens characteristic of endothelial cells.

Differential diagnosis
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A causal therapy is not known. Cytostatic polychemotherapy can be tried.

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Inaustere prognosis. Death occurs after a few months to max. 2 years.

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.


Last updated on: 11.11.2021