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Primary antiphospholipid syndrome with thrombotic thrombocytopenic purpura: a very unusual association

Rheumatology Division, Faculdade de Medicina da Universidade de São Paulo e Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulojotafc{at}gmail.com

CAEW Freitas

Rheumatology Division, Faculdade de Medicina da Universidade de São Paulo e Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo

IVS Lima

Rheumatology Division, Faculdade de Medicina da Universidade de São Paulo e Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo

CC Leite

Radiology Department, Faculdade de Medicina da Universidade de São Paulo e Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo

LV Lage

Rheumatology Division, Faculdade de Medicina da Universidade de São Paulo e Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo

This report considers the rare situation in which primary antiphospholipid syndrome (PAPS) is linked with thrombotic thrombocytopenic purpura (TTP). It describes the case of a young lady with PAPS, characterized by recurring cerebro-vascular abnormalities and marked livedo reticularis, combined with circulating anticardiolipin and lupus anticoagulant antibodies. On follow-up, while on oral anticoagulation, she developed severe thrombocytopenia associated with hematuria, microangiophatic anaemia and neurological manifestations consistent with a diagnosis of TTP. The patient was treated with pulses of methylprednisolone and plasmapheresis with plasma exchange. The result was a favourable outcome. To our knowledge, this is the seventh report on this rare association in the English-language literature of this field.

Key Words: anticardiolipin antibodies • Hughes’ syndrome • microangiopathic anaemia • primary antiphospholipid syndrome • thrombotic thrombocytopenic purpura

Lupus, Vol. 18, No. 9, 841-844 (2009)
DOI: 10.1177/0961203308101958


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