Intravascular cutaneous t-cell lymphoma C49.M

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 31.08.2024

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Synonym(s)

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

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

Localization
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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).

Histology
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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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Therapy
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A causal therapy is not known. Cytostatic polychemotherapy can be tried.

Progression/forecast
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Prognosis unfavorable.

Literature
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  2. Crane GM et al (2014). Primary effusion lymphoma presenting as a cutaneous intravascular lymphoma. J Cutan Pathol 41: 928-935.
  3. Han K et al. (2003) Regression of cutaneous intravascular lymphoma with rituximab. Cutis 72: 137-140
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Last updated on: 31.08.2024