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