reactive angioendotheliomatosis

Reactive angioendotheliomatosis (RAE) is a very rare disorder characterized by marked proliferation of endothelial cells. It is often associated with infections, such as subacute bacterial endocarditis, but has also been described as an early sign of a developing hematological malignancy.

Synopsis

 intravascular proliferation of endothelial cells with histologic resemblance to glomeruloid hemangioma
 foci of many small vessels with intravascular proliferation of endothelial cells
 solid proliferation of endothelial cells

Associations

 infections

• subacute bacterial endocarditis

 ulceration

• Crohn disease

 monoclonal gammopathy (14657635)
 myelodysplastic syndrome (16040421)
 chronic lymphocytic leukemia (8926174)
 antiphospholipid syndrome (12752193, 10767702)
 rheumatoid arthritis (10949459)
 cold agglutinins (9990375, 1479103)
 sarcoidosis (12530356)
 Castleman disease and POEMS syndrome (8403477)
 peripheral vascular atherosclerosis (11939805, 10025757)
 iatrogenic arteriovenous fistulas (10235383)
 hepatopathy and hypertensive portal gastropathy (9620488)
 renal disease (12023572)
 post-renal transplantation (12023572)
 valvular cardiac disease (12023572)
 alcoholic cirrhosis (12023572)
 glioblastoma multiforme (12023572)
 polymyalgia rheumatica (12023572)

Differential diagnosis

 hemangiomas
 hemangioma-like lesions

References

 Ogawa K, Tada T, Takeuchi Y, Suenaga M, Suzuki S, Shirai T. Reactive angioendotheliomatosis of the intestine. Am J Surg Pathol. 2004 Feb;28(2):257-61. PMID: 15043317

 McMenamin ME, Fletcher CD. Reactive angioendotheliomatosis: a study of 15 cases demonstrating a wide clinicopathologic spectrum. Am J Surg Pathol. 2002 Jun;26(6):685-97. PMID: 12023572