Overlap of the symptoms of thrombotic thrombocytopenic purpura and antiphospholipid syndrome in a patient with juvenile systemic lupus erythematosus – case report

Czech version

Authors: M. Bělobrádková ¹; M. Cermanová ²; L. Minxová ³; Z. Bělobrádek ⁴; Z. Vavera ⁵; M. Tošovský ¹; T. Soukup ¹
Authors‘ workplace: Lékařská fakulta Univerzity Karlovy a Fakultní nemocnice v Hradci Králové, Česká republika ; II. interní gastroenterologická klinika ¹; IV. interní hematologická klinika ²; Dětská klinika ³; Radiologická klinika ⁴; I. kardioangiologická klinika ⁵
Published in: Čes. Revmatol., 25, 2017, No. 4, p. 190-198.
Category: Case Report

Overview

Thrombocytopenia accompanied by thrombosis is often observed in patients with systemic lupus erythematosus. The underlying process may be associated with more frequent secondary antiphospholipid syndrome characterized by the presence of antiphospholipid antibodies and thromboses, or with associated thrombotic thrombocytopenic purpura linked with insufficient ADAMTS13 protease activity (A Disintegrin-like And Metalloproteinase with Thrombospondin Type 1 motif 13). In our article we demonstrate a case of a fourteen-year-old girl without a serious pre-existing medical history, who was hospitalized for a stroke accompanied by severe thrombocytopenia and microangiopathic haemolytic anemia. The condition was evaluated as a thrombotic thrombocytopenic purpura, and the patient was treated with plasma exchange therapy (plasmapheresis), glucocorticoids, and then anticoagulation and antiaggregation therapy. Within 6 weeks of onset of the disease, thrombocytopenia has relapsed after tapering of the glucocorticoid dose. Additional plasmaphereses were initiated and rituximab was successfully administered at the same time. Based on further developments in the disease, imaging and laboratory tests, it is possible to admit that it was most likely a coincidence of thrombotic thrombocytopenic purpura and antiphospholipid syndrome. The clinical picture was later completed by a manifestation of systemic lupus erythematosus. The pathogenesis and the current prevalence of these three diseases are discussed.

Key words:
Systemic lupus erythematosus, thrombotic microangiopathy, thrombotic thrombocytopenic purpura, antiphospholipid syndrome

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Labels

Dermatology & STDs Paediatric rheumatology Physiotherapist, university degree Internal medicine Cardiology Orthopaedics Rheumatology

Atherosclerosis and cardiovascular risk in patients with systemic sclerosis

Joint inflammation and microbes in scale from microbiome to septic arthritis

Risk of liver damage during the treatment with tumor necrosis factor antagonists

Article was published in

Czech Rheumatology

2017 Issue 4