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Catastrophic antiphospholipid syndrome in a patient with Down syndrome

Clinical Research Unit, Hospital de Especialidades Centro Médico La Raza, IMSS, Mexico City, México

Consuelo Calleja

Department of Pathology, Hospital de Especialidades Centro Médico La Raza, IMSS, México City, México

Massiel Morán

Medical Student, Universidad Autónoma de Nayarit, Tepic, México

Olga Vera-Lastra

Department of Internal Medicine, Hospital de Especialidades Centro Médico La Raza, IMSS, México City, México; Universidad Nacional Autónoma de México, México City, México

Luis J Jara

Universidad Nacional Autónoma de México, México City, México; Direction of Education and Research, Hospital de Especialidades Centro Médico La Raza, IMSS, México City, Méxicoluis_jara_quezada{at}hotmail.com

We report a patient with Down syndrome, under treatment with carbamazepine, levopromazine and clonazepam. After urinary infection he developed glans necrosis requiring excision of prepuce. Six hours post surgery he presented right-hand ischemia followed by arterial and venous thrombosis of the right thoracic extremity. Later, he progressed to a compartment syndrome and presented ischemia of toes. All the clinical manifestations developed over a week. Anticardiolipin (aCL) antibodies, lupus anticoagulant and perinuclear antineutrophil antibodies were positive. Anticoagulant and immunosuppressive treatment were initiated. Owing to the failure of both treatments, the patient underwent amputation of right hand and a toe. Histopathology revealed recent and old thrombosis of medium- and small-sized vessels without vasculitis. Diagnosis of catastrophic antiphospholipid syndrome (CAPS) was made. At present, the patient continues on oral anticoagulants, IgG aCL remains positive, and no further episodes of thromboses have been observed after 4 years of follow-up. To our knowledge, this is the first case of CAPS in a patient with Down syndrome.

Key Words: anticardiolipin antibodies • catastrophic antiphospholipid syndrome • Down syndrome

Lupus, Vol. 18, No. 12, 1104-1107 (2009)
DOI: 10.1177/0961203309105878


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