Multifocal lymphangioendotheliomatosis with thrombocytopenia: a newly recognized clinicopathological entity.

Multifocal lymphangioendotheliomatosis with thrombocytopenia: a newly recognized clinicopathological entity. Research Abstract Details 

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Multifocal lymphangioendotheliomatosis with thrombocytopenia: a newly recognized clinicopathological entity. Abstract Text:

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.

Multifocal lymphangioendotheliomatosis with thrombocytopenia: a newly recognized clinicopathological entity. Publishing Authors By Initials

For similar proteins: membrane proteins: vesicular transport proteins research abstracts see: proteins: membrane proteins: vesicular transport proteins research

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Multifocal lymphangioendotheliomatosis with thrombocytopenia: a newly recognized clinicopathological entity. Journal Published:

PUBLICATION TYPE: Review

Journal: Archives of dermatology

VOLUME: 140

Page Numbers: 599-606

Journal Abbreviation: Arch Dermatol

ISSN: 0003-987X

DAY: 17

MONTH: May

YEAR: 2004

Multifocal lymphangioendotheliomatosis with thrombocytopenia: a newly recognized clinicopathological entity. Information

Number of References: 44

LANGUAGE: eng

NlmUniqueID: 372433

Multifocal lymphangioendotheliomatosis with thrombocytopenia: a newly recognized clinicopathological entity. Keywords Mesh Terms:

KEYWORDS: Vesicular Transport Proteins

MESH TERMS: pathology

Chemical & Substance for Abstract: Multifocal lymphangioendotheliomatosis with thrombocytopenia: a newly recognized clinicopathological entity. Information

Substance Name: Vesicular Transport Proteins

Registry Number: 0

Grant and Affiliation Information for Multifocal lymphangioendotheliomatosis with thrombocytopenia: a newly recognized clinicopathological entity.

AFFILIATION: Departments of Pathology and Otolaryngology, the University of Arkansas for Medical Sciences and Arkansas Children's Hospital, Little Rock, AR 72202, USA.

Country: United States

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MEDLINETA: Arch Dermatol

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