Abstract

CASE PRESENTATION AND REVIEW OF LITRATURE FOR A RARE CASE OF HEREDITORY HEMORRHAGIC TELANGIECTASIA AS SECONDARY POLYCYTHEMIA

Aman Chaudhary, *Lalit P. Meena, Jaya Chakravarty, Madhukar Rai and Shyam Sundar

Abstract

Hereditary hemorrhagic telangiectasia (also known as Osler-Weber- Rendu disease) is an autosomal dominant disorder that results from multisystem vascular dysplasia. It is characterized by telangiectasias and arteriovenous malformations of skin, mucosa, and viscera. Epistaxis is the most common presenting problem, occurring in 90 percent of affected patients. Diagnosis is clinical and made using the Curacao criteria. Patients usually seek medical attention due to bleeding manifestations from telangiectasias or arteriovenous malformation. Herein we report a case who presented to us mainly due to features of hyperviscosity of blood due to secondary polycythemia which developed due to an underlying Pulmonary arteriovenous malformation.

Keywords: HHT, Osler-Weber-Rendu disease, Pulmonary arteriovenous malformation.