Thrombotic thrombocytopenic purpura (TTP) and warm-antibody autoimmune hemolytic anemia (AIHA) are uncommon diseases. Although TTP has been increasingly described in association with autoimmune antibodies, there are very few reports of the association with autoimmune hematological conditions, including idiopathic thrombocytopenic purpura and AIHA. Here we describe a patient with classic manifestations of TTP, who was successfully treated with plasma exchange. A few weeks later, she developed warm-antibody AIHA, which responded promptly to prednisone.

Keywords:
Autoimmune hemolytic anemia, Evans’ syndrome, Thrombotic thrombocytopenic purpura
1.
van Oss CJ, Beckers D, Engelfriet CP, Absolom DR, Newmann AW: Elution of blood group antibodies from red blood cells. Vox Sang 1981;40:367–371.
2.
Tsai HM, Lian EC: Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 1998;339:1585–1594.
3.
Lian EC: Thrombotic thrombocytopenic purpura – A syndrome caused by multiple pathogenetic mechanisms. Invest Clin 2001;42(suppl 1):75–86.
4.
Karakantza M, Mouzaki A, Theodoropoulou M, Bussel JB, Maniatis A: Th1 and Th2 cytokines in a patient with Evans’ syndrome and profound lymphopenia. Br J Haematol 2000;110:968–970.
5.
Torok TJ, Holman RC, Chorba TL: Increasing mortality from thrombotic thrombocytopenic purpura in the United States – Analysis of national mortality data, 1968–1991. Am J Hematol 1995;50/2:84–90.
6.
Gehrs BC, Friedberg RC: Autoimmune hemolytic anemia. Am J Hematol 2002;69:258–271.
7.
Allford SL, Harrison P, Lawrie AS, Liesner R, MacKie IJ, Machin SJ: Von Willebrand factor-cleaving protease activity in congenital thrombotic thrombocytopenic purpura. Br J Haematol 2000;11:1215–1222.
8.
Furlan M, Robles R, Galbusera M, Remuzzi G, Kyrle PA, Brenner B, Krause M, Scharrer I, Aumann V, Mittler U, Solenthaler M, Lammle B: Von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med 1998;339:1578–1584.
9.
Stricker RB, Davis JA, Gershow J, Yamamoto KS, Kiprov DD: Thrombotic thrombocytopenic purpura complicating systemic lupus erythematosus: Case report and literature review from the plasmapheresis era. J Rheumatol 1992;19:1469–1473.
10.
Steinberg AD, Green WT, Talal N: Thrombotic thrombocytopenic purpura complicating Sjogren syndrome. JAMA 1971;215:757–761.
11.
Ellingson TL, Wilske K, Aboulafia DM: Case report: Thrombotic thrombocytopenic purpura in a patient with polymyositis: Therapeutic importance of early recognition and discussion of pathogenic mechanisms. Am J Med Sci 1992;303:407–410.
12.
Cookson S, Krueger ML, Bennett RM: Fulminant thrombotic thrombocytopenic purpura in a patient with the limited form of scleroderma: Successful outcome using plasma exchange. J Rheumatol 1991;18:900–901.
13.
Fessler BJ: Thrombotic syndromes and autoimmune diseases. Rheum Dis Clin North Am 1997;23:461–479.
14.
Majado MJ, Gonzalez C, Candel R: Thrombocytopenic purpura after immune thrombocytopenic purpura. Sangre 1993;38:504–505.
15.
Yospur LS, Sun NC, Figueroa P, Niihara Y: Concurrent thrombotic thrombocytopenic purpura and immune thrombocytopenic purpura in a HIV-positive patient: Case report and review of the literature. Am J Hematol 1996;51:73–78.
16.
Olenich M, Schattnet EL: Postpartum thrombotic thrombocytopenic purpura (TTP) complicating pregnancy-associated immune thrombocytopenic purura (ITP). Ann Intern Med 1994;120:845–847.
17.
Zacharski LR, Lusted D, Glick JL: Thrombotic thrombocytopenic purpura in a previously splenectomized patient. Am J Med 1976;60:1061–1063.
18.
Fagiolo E: Thrombotic thrombocytopenic purpura with autoimmune pancytopenia. Acta Haematol 1974;53:356–361.
19.
Krupsky M, Sarel R, Hurwitz N, Resnitzky P: Late appearance of thrombotic thrombocytopenic purpura after autoimmune hemolytic anemia and in the course of chronic autoimmune thrombocytopenic purpura: Two case reports. Acta Haematol 1991;85:139–142.
20.
Ridolfi RL, Bell WR: Thrombotic thrombocytopenic purpura: Report of 25 cases and review of the literature. Medicine 1981;60:413–428.
21.
Amorosi EL, Ultmann JE: Thrombotic thrombocytopenic purpura: Report of 16 cases and review of the literature. Medicine (Baltimore) 1966;45:139–159.
22.
Elliot MA, Nichols WL: Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Mayo Clin Proc 2001;76:1154–1162.
© 2002 S. Karger AG, Basel
2002
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.