VPB0312 - Thrombotic thrombocytopenic purpura in adult-onset Still's disease

Background: Coexistence of TTP and adult-onset Still’s disease is extremely rare.

Aims: There is increasing evidence that this association could be more
than just coincidental.

Methods: Hereby, we present a case of TTP occurring in patient with a known ASD and the successful outcome after plasma exchanges.

Results: A 24 year-old Tunisian woman was referred to our department for the first time in February 2006. The diagnosis of AOSD was established and the patient was treated first with high doses of corticosteroids and all her symptoms resolved.
At the age of 35 year-old, she was admitted again after one month of intense arthralgias involving her shoulders, elbows and wrists. She had also recurrent nosebleed episodes.
The physical examination was remarkable for multiple ecchymosis on the lowers extremities.
Blood smear showed frequent schistocytes (10%) and increased reticulocytes rate 286 000in addition to hemogram disorders: low circulating platelet count 6000,haemolytic anemia 7,4g/dland increased white cell count at 13000 Elts/mm3. Electrolytes, kidney function test, liver enzymes were within normal range. There was no evidence of an evolutive infection or neoplasia.
The diagnosis of TTP based on microangiopathic hemolytic anemia and thrombocytopenia that cannot be explained otherwise. Of note, blood tests for ADAMTS13 activity and inhibitor were not available in the hospital.
To reduce bleeding frequency, the patient received platelets transfusion and high-dose corticosteroid therapy. Then, intravenous immunoglobulin treatment was added but with no significant improvement. Finally, within the application of plasmapheresis regimen, her symptoms began to resolve: there have been no further arthralgias or hemorrhage signs.
The patient was discharged on prednisone therapy with plans for extended taper.

Conclusion(s): The purpose of this case is to insist on the importance of searching for a TTP once clinical evocative symptoms are reported by a patientwith AOSD.