Article: Multifocal lymphangioendotheliomatosis with thrombocytopenia: a newly recognized clinicopathological entity.
2004 Volume 140, Issue 5, Page(s) 599–606
Abstract: ... multifocal lymphangioendotheliomatosis with thrombocytopenia to distinguish this newly recognized ... clinicopathological entity. These congenital lesions, like tufted angioma and kaposiform hemangioendothelioma, show ... with extensive venous and lymphatic malformations. We have also observed patients with rare multifocal ...
| Abstract | Background: Severe thrombocytopenic coagulopathy may complicate platelet-trapping vascular tumors such as kaposiform hemangioendothelioma and tufted angioma. Low-grade, chronic consumptive coagulopathy may occur with extensive venous and lymphatic malformations. We have also observed patients with rare multifocal, congenital skin and gastrointestinal (GI) tract vascular anomalies of distinctive and remarkably similar appearance, all associated with coagulopathy. We studied the clinical and histopathologic features of 3 patients demonstrating this previously uninvestigated phenomenon. Observations: All 3 patients presented with hundreds of congenital red-brown skin plaques as large as a few centimeters, with similar lesions throughout the GI tract and severe GI tract bleeding. One patient had synovial involvement. All had significant thrombocytopenia, with prothrombin and partial thromboplastin times and fibrinogen levels near the reference range. Corticosteroids and/or interferon alfa treatment resulted in equivocal or no improvement. Skin lesions from all 3 patients were histologically distinctive and similar, including dilated, thin-walled vessels in the dermis and subcutis lined by hobnailed, proliferative endothelial cells (10%-15% immunoreactive for Ki-67), most displaying intraluminal papillary projections. Immunoreaction for the lymphatic marker LYVE-1 was uniformly present. Conclusions: We propose the term multifocal lymphangioendotheliomatosis with thrombocytopenia to distinguish this newly recognized clinicopathological entity. These congenital lesions, like tufted angioma and kaposiform hemangioendothelioma, show lymphatic differentiation, strengthening the association between abnormal lymphatic endothelium and coagulopathy. |
|||||
|---|---|---|---|---|---|---|
| MeSH term(s) | Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Glycoproteins ; Hemangioendothelioma/complications ; Hemangioendothelioma/diagnosis ; Hemangioendothelioma/pathology ; Humans ; Immunohistochemistry ; Infant ; Male ; Skin Neoplasms/complications ; Skin Neoplasms/diagnosis ; Skin Neoplasms/pathology ; Thrombocytopenia/complications ; Thrombocytopenia/diagnosis ; Thrombocytopenia/pathology ; Vesicular Transport Proteins | |||||
| Chemical Substances | Glycoproteins ; LYVE1 protein, human ; Vesicular Transport Proteins | |||||
| Language | English | |||||
| Publishing date | 2004-05 | |||||
| Publishing country | United States | |||||
| Document type | Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't ; Review | |||||
| ZDB-ID | 212139-6 | |||||
| ISSN | 1538-3652 ; 0003-987X | |||||
| ISSN (online) | 1538-3652 | |||||
| ISSN | 0003-987X | |||||
| DOI | 10.1001/archderm.140.5.599 | |||||
| Shelf mark |
|
|||||
| Database | MEDical Literature Analysis and Retrieval System OnLINE |
More links
Kategorien
In stock of ZB MED Cologne/Königswinter
| Ui VI Zs.32: Show issues | Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular ab Jg. 2022: Lesesaal (EG) |
Order via subito
This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.