Synonym(s)
Angioendotheliomatosis proliferans systematisata; Endothelioma intravascular; Endotheliomatosis systematized; IVL; malignant angioendotheliomatosis
DefinitionThis section has been translated automatically.
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).
LocalizationThis section has been translated automatically.
Occurs mainly on the trunk, lower extremities and face.
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Clinical featuresThis section has been translated automatically.
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).
HistologyThis section has been translated automatically.
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 diagnosisThis section has been translated automatically.
TherapyThis section has been translated automatically.
A causal therapy is not known. Cytostatic polychemotherapy can be tried.
Progression/forecastThis section has been translated automatically.
Prognosis unfavorable.
LiteratureThis section has been translated automatically.
- Asagoe K et al. (2003) Intravascular lymphomatosis of the skin as a manifestation of recurrent B-cell lymphoma. J Am Acad Dermatol 48: S1-4
- Crane GM et al (2014). Primary effusion lymphoma presenting as a cutaneous intravascular lymphoma. J Cutan Pathol 41: 928-935.
- Han K et al. (2003) Regression of cutaneous intravascular lymphoma with rituximab. Cutis 72: 137-140
- Kamath NV et al. (2001) Primary cutaneous large B-cell lymphoma of the leg relapsing as cutaneous intravascular large B-cell lymphoma. Arch Dermatol 137: 1657-1658
- Kolde G et al (1991) Angioendotheliomatosis proliferans systematisata. Dermatologist 42: 589-592
- Kutzner H et al. (1991) Angioendotheliomatosis proliferans systematisata: A cutaneous manifestation of malignant B-cell lymphomas. Dermatology 42: 384-390
- McDonough SP et al. (2002) Clinicopathological and immunophenotypical features of canine intravascular lymphoma (malignant angioendotheliomatosis). J Comp Pathol 126: 277-288
- Samols MA et al. (2014) Intralymphatic cutaneous anaplastic large cell lymphoma/lymphomatoid papulosis: expanding the spectrum of CD30-positive lymphoproliferative disorders. Am J Surg Pathol 38:1203-1211.
- Satzger I et al. (2009) Intravascular B-cell lymphomas. Dermatology 60: 131-136
- Schley G et al (2001) Angioendotheliomatosis proliferans systematisata. Dermatology 52: 1030-1034
- Schmidt K et al. (1996) Reactive angioendotheliomatosis in chronic lymphocytic leukemia. Dermatologist 47: 550-555
- Willemze R et al. (2005) WHO-EORTC classification for cutaneous lymphomas. Blood 105: 3768-3785
- Zanelli M et al. (2022) Intravascular NK/T-Cell Lymphoma: What We Know about This Diagnostically Challenging, Aggressive Disease. Cancers (Basel). 14:5458. doi: 10.3390/cancers14215458. PMID: 36358876; PMCID: PMC9658079.
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Incoming links (9)
Angioendotheliomatosis; Angioendotheliomatosis; Angioendotheliomatosis proliferans systematisata; Diffuse dermal angiomatosis; Diffuse dermal angiomatosis ; Endothelioma, intravascular; Endotheliomatosis, systemized; Intravascular large b-cell lymphoma; Malignant angioendotheliomatosis;Outgoing links (1)
Intravascular large b-cell lymphoma;Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
Prof. Dr. med. Peter Altmeyer