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Cyclosporine for Thrombotic Thrombocytopenic Purpura
- Horst Kierdorf, MD;
- Norbert Maurin, MD; and
- Bernhard Heintz, MD
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TO THE EDITOR:
Thrombotic thrombocytopenic purpura consists of thrombocytopenia, microangiopathic hemolytic anemia, fluctuating neurologic signs, fever, and renal impairment [1]. The primary treatment modality is plasmapheresis, which is sometimes combined with antiplatelet therapy [2]. Splenectomy and immunosuppressive therapy have been used where the response is poor [2, 3].
We describe a 64-year-old man in whom the disease was controlled by cyclosporine after plasmapheresis failed. When admitted, he was in a confused state and had an unexplained fever (temperature, 39.1 C), a slightly enlarged liver, a hemoglobin concentration of 97 g/L, a platelet count of 31 000/mm3, red cell fragmentation (25% of all erythrocytes), proteinuria, and hematuria. Creatinine (250 mol/L), urea (14 mmol/L), bilirubin (69 mol/L), and lactic dehydrogenase (1500 IU/L; normal range, 80 to 240 IU/L) levels were increased. No autoantibodies were detected. Coombs tests were negative; disseminated intravascular coagulation was excluded. Thrombotic thrombocytopenic purpura was diagnosed, and treatment was begun with a 3-liter plasma exchange every second day. In addition, aspirin, dipyridamole, and prostacyclin were administered. No stable remission could be achieved, and after day 15 treatment was ineffective.
On day 20, conventional therapy was discontinued and cyclosporine, 300 mg/d, was instituted. Therapeutic blood levels were attained within 3 days, and complete remission occurred within 8 days. Cyclosporine therapy was continued for 3 months and stopped by the patient.
Three months after discontinuing cyclosporine treatment, the patient had a relapse that was characterized by thrombocytopenia (40 000/mm3), anemia (hemoglobin, 90 g/L), neurologic symptoms, and an increased creatinine level. Cyclosporine (300 mg/d) was administered as the sole therapy, and complete remission was achieved within 10 days. The patient has been in full clinical and hematologic remission for 9 months on cyclosporine (Figure 1). Although plasmapheresis has reduced the mortality associated with thrombotic thrombocytopenic purpura, it is not universally effective, either alone or in combination with antiplatelet agents [2, 3]. Because some patients with thrombotic thrombocytopenic purpura have cytotoxic antibodies, immunosuppressive agents such as prednisolone, azathioprine, vincristine, cyclophosphamide, and immunoglobulins have been used empirically for the proposed immunologic damage [3-5].
Cyclosporine alone appears to have induced a remission in one patient with thrombotic thrombocytopenic purpura resistant to conventional therapy and was also effective when the patient experienced a subsequent relapse. In contrast to other immunosuppressive therapy, cyclosporine has fewer side effects and may prove to be a useful alternative therapy for patients with chronic thrombotic thrombocytopenic purpura.
The Editors welcome submissions for possible publication in the Letters section. Authors of letters should:
Include no more than 300 words of text, three authors, and five references
Type with double-spacing
Send three copies of the letter, an authors' form signed by all authors, and a cover letter describing any conflicts of interest related to the contents of the letter.
Letters commenting on an Annals article will be considered if they are received within 6 weeks of the time the article was published. Only some of the letters received can be published. Published letters are edited and may be shortened; tables and figures are included only selectively. Authors will be notified that the letter has been received. If the letter is selected for publication, the author will be notified about 3 weeks before the publication date. Unpublished letters cannot be returned.
Annals welcomes electronically submitted letters.
- Copyright 2004 by the American College of Physicians
