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Intravenous gamma globulin for thrombotic microangiopathy of unknown etiology

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Abstract

We encountered the case of a 4-year-old boy with thrombotic microangiopathy (TMA) of unknown etiology. Verotoxin-induced hemolytic uremic syndrome (HUS), Streptococcus-pneumoniae-related HUS, factor H deficiency, drug-induced thrombotic thrombocytopenic purpura (TTP), and ADAMTS13 (von Willebrand factor-cleaving protease; a disintegrin-like and metalloprotease with thrombospondin type 1 repeats)-related TTP were excluded. His condition was refractory to anticoagulants and plasma exchange, and his clinical course was catastrophic, with central nervous system symptoms and progressive renal failure. However, factual treatment of intravenous gamma globulin (IVIG) ended the hemolysis and resulted in a rise in platelet count. He fully recovered except for end-stage renal failure, but he underwent a successful renal transplant after peritoneal dialysis. He has not suffered a relapse of TMA or an allograft rejection for 4 years. IVIG might be an option for some patients with TMA of unknown etiology refractory to conventional treatment.

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References

  1. Amorosi EL (1966) Thrombotic thrombocytopenic purpura: report of 16 cases and review of the literature. Medicine (Baltimore) 45:139

    Article  Google Scholar 

  2. Remuzzi G (1987) HUS and TTP: variable expression of a single entity. Kidney Int 32:292–308

    Article  CAS  Google Scholar 

  3. Rock GA, Shumak KH, Buskard NA, Blanchette VS, Kelton JG, Nair RC, Spasoff RA (1991) Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med 325:393–397

    Article  CAS  Google Scholar 

  4. Bell WR, Braine HG, Ness PM, Kickler TS (1991) Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients. N Engl J Med 325:398–403

    Article  CAS  Google Scholar 

  5. von Baeyer H (2002) Plasmapheresis in thrombotic microangiopathy-associated syndromes: review of outcome data derived from clinical trials and open studies. Ther Apher 6:320–328

    Article  Google Scholar 

  6. Mori Y, Wada H, Gabazza EC, Minami N, Nobori T, Shiku H, Yagi H, Ishizashi H, Matsumoto M, Fujimura Y (2002) Predicting response to plasma exchange in patients with thrombotic thrombocytopenic purpura with measurement of vWF-cleaving protease activity. Transfusion 42:572–580

    Article  CAS  Google Scholar 

  7. Fujikawa K, Suzuki H, McMullen B, Chung D (2001) Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family. Blood 98:1662–1666

    Article  CAS  Google Scholar 

  8. Plaimauer B, Zimmermann K, Volkel D, Antoine G, Kerschbaumer R, Jenab P, Furlan M, Gerritsen H, Lammle B, Schwarz HP, Scheiflinger F (2002) Cloning, expression, and functional characterization of the von Willebrand factor-cleaving protease (ADAMTS13). Blood 100:3626–3632

    Article  CAS  Google Scholar 

  9. Levy GG, Motto DG, Ginsburg D (2005) ADAMTS13 turns 3. Blood 106:11–17

    Article  CAS  Google Scholar 

  10. Chow TW, Turner NA, Chintagumpala M, McPherson PD, Nolasco LH, Rice L, Hellums JD, Moake JL (1998) Increased von Willebrand factor binding to platelets in single episode and recurrent types of thrombotic thrombocytopenic purpura. Am J Hematol 57:293–302

    Article  CAS  Google Scholar 

  11. Coppo P, Bengoufa D, Veyradier A, Wolf M, Bussel A, Millot GA, Malot S, Heshmati F, Mira JP, Boulanger E, Galicier L, Durey-Dragon MA, Fremeaux-Bacchi V, Ramakers M, Pruna A, Bordessoule D, Gouilleux V, Scrobohaci ML, Vernant JP, Moreau D, Azoulay E, Schlemmer B, Guillevin L, Lassoued K (2004) Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count, and mild renal involvement. Medicine (Baltimore) 83:233–244

    Article  CAS  Google Scholar 

  12. Rieger M, Mannucci PM, Kremer Hovinga JA, Herzog A, Gerstenbauer G, Konetschny C, Zimmermann K, Scharrer I, Peyvandi F, Galbusera M, Remuzzi G, Bohm M, Plaimauer B, Lammle B, Scheiflinger F (2005) ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immuno-mediated diseases. Blood 106:1262–1267

    Article  CAS  Google Scholar 

  13. Zheng XL, Kaufman RM, Goodnough LT, Sadler JE (2004) Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura. Blood 103:4043–4049

    Article  CAS  Google Scholar 

  14. Ruggenenti P, Noris M, Remuzzi G (2001) Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. Kidney Int 60:831–846

    Article  CAS  Google Scholar 

  15. Horton TM, Stone JD, Yee D, Dreyer Z, Moake JL, Mahoney DH (2003) Case series of thrombotic thrombocytopenic purpura in children and adolescents. J Pediatr Hematol Oncol 25:336–339

    Article  Google Scholar 

  16. Wong P, Itoh K, Yoshida S (1986) Treatment of thrombotic thrombocytopenic purpura with intravenous gamma globulin. N Engl J Med 314:385–386

    CAS  PubMed  Google Scholar 

  17. Staszewski H, Colbourn D, Donovan V, Ludman H (1989) Thrombotic thrombocytopenic purpura: report of a case with a possible response to high-dose intravenous gamma globulin. Acta Haematol 82:201–204

    Article  CAS  Google Scholar 

  18. Heyman MR, Sweet T (1990) Thrombotic thrombocytopenic purpura treated with high-dose intravenous gamma globulin. South Med J 83:1471–1474

    Article  CAS  Google Scholar 

  19. Nosari A, Muti G, Busnach G, Cantoni S, Strinchini A, Morra E (1996) Intravenous gamma globulin in refractory thrombotic thrombocytopenic purpura. Acta Haematol 96:255–257

    Article  CAS  Google Scholar 

  20. Kondo H (1993) Effect of intravenous gamma globulin infusion on recurrent episodes of thrombotic thrombocytopenic purpura (TTP). Eur J Haematol 50:55–56

    CAS  PubMed  Google Scholar 

  21. Thompson CE, Damon LE, Ries CA, Linker CA (1992) Thrombotic microangiopathies in the 1980s: clinical features, response to treatment, and the impact of the human immunodeficiency virus epidemic. Blood 80:1890–1895

    Article  CAS  Google Scholar 

  22. Ucar A, Fernandez HF, Byrnes JJ, Lian EC, Harrington WJ Jr (1994) Thrombotic microangiopathy and retroviral infections: a 13-year experience. Am J Hematol 45:304–309

    Article  CAS  Google Scholar 

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Authors and Affiliations

  1. Children’s Medical Center, Yokohama City University Medical Center, 4-57, Urafune-cho, Minami-ku, Yokohama, 232-0024, Japan

    Shuichi Ito, Kenichi Okuyama, Tomoko Nakamura, Yukoh Aihara & Shumpei Yokota

  2. Department of Urology, Yokohama City University Medical Center, Yokohama, Japan

    Jun-ichi Tetanishi & Kazuo Saito

  3. Department of Blood Transfusion Medicine, Nara Medical University, Nara, Japan

    Masanori Matsumoto & Yoshihiro Fujimura

Authors
  1. Shuichi Ito
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  2. Kenichi Okuyama
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  3. Tomoko Nakamura
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  4. Jun-ichi Tetanishi
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  5. Kazuo Saito
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  6. Masanori Matsumoto
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  7. Yoshihiro Fujimura
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  8. Yukoh Aihara
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  9. Shumpei Yokota
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Correspondence to Shuichi Ito.

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Ito, S., Okuyama, K., Nakamura, T. et al. Intravenous gamma globulin for thrombotic microangiopathy of unknown etiology. Pediatr Nephrol 22, 301–305 (2007). https://doi.org/10.1007/s00467-006-0326-9

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  • DOI: https://doi.org/10.1007/s00467-006-0326-9

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