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  • Title: Malignant angioendotheliomatosis mimicking systemic necrotizing vasculitis.
    Author: Kao NL, Broy S, Tillawi I.
    Journal: J Rheumatol; 1992 Jul; 19(7):1133-5. PubMed ID: 1512773.
    Abstract:
    Malignant angioendotheliomatosis is a rare disease characterized by an intravascular proliferation of atypical mononuclear cells. Manifestations result from occlusion of small blood vessels. Multiple organ systems are involved and the clinical presentation resembles a systemic necrotizing vasculitis with skin and central nervous system most commonly involved. The clinical course is characterized by progressive organ failure with death usually within 2 years after presentation. Based on its assumed origin as an intravascular lymphoma, patients may respond to chemotherapy.
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