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Chronic Relapsing Thrombotic Thrombocytopenic Purpura in Adult Onset Still's Disease

Martin G.V. Perez, MD, Francis R. Rodwig Jr., MD
Volume: 96 Issue: 1 January, 2003

Abstract:

We report the first known case of chronic relapsing thrombotic thrombocytopenic purpura associated with adult-onset Still's disease. The patient presented with diffuse arthralgias, sore throat, and a maculopapular rash involving the trunk and extremities; she was hospitalized with fever and confusion. Thrombocytopenia, renal failure, and microangiopathic hemolytic anemia developed within several days. After a diagnosis of thrombotic thrombocytopenic purpura was made, she responded well to a series of plasma exchanges. Evaluation for infection, autoimmune disorders, and malignancy was negative. She was discharged to home in good condition, with normal renal function and normal platelet count. Two more episodes of TTP developed 7 and 9 months after the first hospitalization. The diagnosis of adult-onset Still's disease was then determined on the basis of clinical and laboratory criteria. She was successfully treated with plasma exchange, prednisone, and azathioprine. She later had splenectomy and has subsequently been wihout recurrence of thrombotic thrombocytopenic purpura for 2 years.

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References:

1. Cush JJ, Medsger TA Jr, Christy WC, Herbert DC, Cooperstein LA. Adult-onset Still's disease: Clinical course and outcome. Arthritis Rheum 1987; 30: 186–194.
 
2. Ohta A, Yamaguchi M, Kaneoka H, Nagayoshi T, Hiida M. Adult Still's disease: Review of 228 cases from the literature. J Rheumatol 1987; 14: 1139–1146.
 
3. Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol 1992; 19: 424–430.
 
4. Nguyen KH, Weisman MH. Severe sore throat as a presenting symptom of adult onset Still's disease: A case series and review of the literature. J Rheumatol 1997; 24: 592–597.
 
5. Mok CC, Lau CS, Wong RW. Clinical characteristics, treatment, and outcome of adult onset Still's disease in southern Chinese. J Rheumatol 1998; 25: 2345–2351.
 
6. Bywaters EG. Still's disease in the adult. Ann Rheum Dis 1971; 30: 121–133.
 
7. Masson C, Le Loet X, Liote F, et al. Comparative study of 6 types of criteria in adult Still's disease. J Rheumatol 1996; 23: 495–497.
 
8. Portoles J, de Tomas E, Espinosa A, et al. Thrombotic thrombocytopenic purpura and acute renal failure in adult Still's disease. Nephrol Dial Transplant 1997; 12: 1471–1473.
 
9. Pouchot J, Sampalis JS, Beaudet F, et al. Adult Still's disease: Manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore) 1991; 70: 118–136.
 
10. Ohta A, Yamaguchi M, Tsunematsu T, et al. Adult Still's disease: A multicenter survey of Japanese patients. J Rheumatol 1990; 17: 1058–1063.
 
11. Sobieska M, Fassbender K, Aeschlimann A, et al. Still's disease in children and adults: A distinct pattern of acute-phase proteins. Clin Rheumatol 1998; 17: 258–260.
 
12. Van Reeth C, Le Moel G, Lasne Y, et al. Serum ferritin and isoferritins are tools for diagnosis of active adult Still's disease. J Rheumatol 1994; 21: 890–895.
 
13. Magadur-Joly G, Billaud E, Barrier JH, et al. Epidemiology of adult Still's disease: Estimate of the incidence by a retrospective study in west France. Ann Rheum Dis 1995; 54: 587–590.
 
14. Moschowitz E: An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries. Arch Intern Med 1925; 36: 89–93.
 
15. Pasquale D, Vidhya R, DaSilva K, et al. Chronic relapsing thrombotic thrombocytopenic purpura: Role of therapy with cyclosporine. Am J Hematol 1998; 57: 57–61.
 
16. Diamond JR. Hemolytic uremic syndrome/thrombotic thrombocytopenic purpura (HUS/TTP) complicating adult Still's disease: Remission induced with intravenous immunoglobulin G. J Nephrol 1997; 10: 253–257.
 
17. Masson C, Myhal D, Menard H, Lussier A. Fatal thrombopenic thrombotic purpura in a female patient with adult Still's disease [in French]. Rev Rhum Mal Osteoartic 1986; 53: 389–391.
 
18. Boki KA, Tsirantonaki MJ, Markakis K, Moutsopoulos HM. Thrombotic thrombocytopenic purpura in adult Still's disease. J Rheumatol 1996; 23: 385–387.
 
19. Bell WR. Thrombotic thrombocytopenic purpura/hemolytic uremic syndrome relapse: Frequency, pathogenesis, and meaning. Semin Hematol 1997; 34: 134–139.
 
20. Lian EC. Pathogenesis of thrombotic thrombocytopenic purpura. Semin Hematol 1987; 24: 82–100.
 
21. Sarode R, Gottschall JL, Aster RH, McFarland JG. Thrombotic thrombocytopenic purpura: early and late responders. Am J Hematol 1997; 54: 102–107.
 
22. Onundarson PT, Rowe JM, Heal JM, Francis CW. Response to plasma exchange and splenectomy in thrombotic thrombocytopenic purpura: A 10-year experience at a single institution. Arch Intern Med 1992; 152: 791–796.
 
23. Rock GA, Shumak KH, Buskard NA, et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura: Canadian Apheresis Study Group. N Engl J Med 1991; 325: 393–397.
 
24. Shumak KH, Rock GA, Nair RC. Late relapses in patients successfully treated for thrombotic thrombocytopenic purpura: Canadian Apheresis Group. Ann Intern Med 1995; 122: 569–572.
 
25. Furlan M, Robles R, Galbusera M, et al. Von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med 1998; 339: 1578–1584.
 
26. Moake JL, Rudy CK, Troll JH, et al. Therapy of chronic relapsing thrombotic thrombocytopenic purpura with prednisone and azathioprine. Am J Hematol 1985; 20: 73–79.
 
27. Phillips WG, Weller R, Handfield-Jones SE, Kobza-Black A. Adult Still's disease. Br J Dermatol 1994; 130: 511–513.
 
28. Yamaguchi M, Matsukawa Y, Takahashi N, et al. Successful methotrexate therapy for adult Still's disease with marked thrombocytopenia. Clin Rheumatol 1998; 17: 256–257.

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