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Cryosupernatant and Immunosuppression as Effective Alternative Therapies for TTP in Three Pediatric SLE Patients

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Abstract

Thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE) very rarely present simultaneously and pose a diagnostic and therapeutic dilemma to the physician. Prompt diagnosis and management with plasma exchange and immunosuppression is life-saving. To describe the effectiveness of cryosupernatant and steroids in pediatric SLE with TTP. We describe three children aged 12–14 years with SLE who were diagnosed with TTP based on fever, CNS manifestations, ANA, anti-dsDNA, anti-sm positivity, hypocomplementemia, and microangiopathic anemia with thrombocytopenia. All three children were managed with cryosupernatant and steroids without plasmapheresis. All children improved with cryosupernatant and steroids. All attained remission within 10 days. They were doing well at last follow up without relapse or flare. Cryosupernatant and steroids may be an effective therapy for Thrombotic thrombocytopenic purpura with systemic lupus erythematosus

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References

  1. Kwok SK, Ju JH, Cho CS, Kim HY, Park SH (2009) Thrombotic thrombocytopenic purpura in systemic lupus erythematosus: risk factors and clinical outcome: a single centre study. Lupus 18:16–21

    Article  PubMed  CAS  Google Scholar 

  2. Hamasaki K, Mimura T, Kanda H, Kubo K, Setoguchi K, Satoh Y et al (2003) Systemic lupus erythematosus and thrombotic thrombocytopenic purpura: a case report and literature review. Clin Rheumatol 22:355–358

    Article  PubMed  CAS  Google Scholar 

  3. Kiss JE (2010) Thrombotic thrombocytopenic purpura: recognition and management. Int J Hematol 91:36–45

    Article  PubMed  Google Scholar 

  4. Brunner HI, Freedman M, Silverman ED (1999) Close relationship between systemic lupus erythematosus and thrombotic thrombocytopenic purpura in childhood. Arthritis Rheum 42:2346–2355

    Article  PubMed  CAS  Google Scholar 

  5. Musio F, Bohen EM, Yuan CM, Welch PG (1998) Review of thrombotic thrombocytopenic purpura in the setting of systemic lupus erythematosus. Semin Arthritis Rheum 28:1–19

    Article  PubMed  CAS  Google Scholar 

  6. Rock GA, Shumak KH, Buskard NA, Blanchette VS, Kelton JG, Nair RC et al (1991) Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. N Engl J Med 325:393–397

    Article  PubMed  CAS  Google Scholar 

  7. Altuntas F, Aydogdu I, Kabukcu S et al (2007) Therapeutic plasma exchange for the treatment of thrombotic thrombocytopenic purpura: a retrospective multicenter study. Transfus Apher Sci 36:57–67

    Article  PubMed  Google Scholar 

  8. Muscal E, Edwards RM, Kearney DL, Hicks JM, Myones BL, Teruya J (2011) Thrombotic microangiopathic hemolytic anemia with reduction of ADAMTS13 activity: initial manifestation of childhood-onset systemic lupus erythematosus. Am J Clin Pathol 135:406–416

    Article  PubMed  Google Scholar 

  9. Rock G, Anderson D, Clark W, Leblond P, Palmer D, Sternbach M et al (2005) Does cryosupernatant plasma improve outcome in thrombotic thrombocytopenic purpura? No answer yet. Br J Haematol. 129:79–86

    Article  CAS  Google Scholar 

  10. Allford SL, Hunt BJ, Rose P (2003) Machin SJ; Haemostasis and Thrombosis Task Force, British Committee for Standards in Haematology. Guidelines on the diagnosis and management of the thrombotic microangiopathic hemolytic anaemias. Br J Haematol 120:556–573

    Article  PubMed  Google Scholar 

  11. Michael M, Elliott EJ, Ridley GF, Hodson EM, Craig JC (2009) Interventions for hemolytic uraemic syndrome and thrombotic thrombocytopenic purpura. Cochrane Database Syst Rev 1:CD003595

    PubMed  Google Scholar 

  12. Scully M, Longair I, Flynn M, Berryman J, Machin SJ (2007) Cryosupernatant and solvent detergent fresh-frozen plasma (Octaplas) usage at a single centre in acute thrombotic thrombocytopenic purpura. Vox Sang 93:154–158

    Article  PubMed  CAS  Google Scholar 

  13. Cacoub P, Limal N, Sène D, Guichard I, Piette JC (2008) Rituximab for the treatment of thrombotic thrombocytopenic purpura in systemic lupus erythematosus. Lupus 17:69–71

    Article  PubMed  Google Scholar 

  14. Shah AA, Higgins JP, Chakravarty EF (2007) Thrombotic microangiopathic hemolytic anemia in a patient with SLE: diagnostic difficulties. Nat Clin Pract Rheumatol 3:357–362

    Article  PubMed  Google Scholar 

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Correspondence to Sathish Kumar.

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Palaniappan, P., Lionel, A.P., Agarwal, I. et al. Cryosupernatant and Immunosuppression as Effective Alternative Therapies for TTP in Three Pediatric SLE Patients. Indian J Hematol Blood Transfus 30 (Suppl 1), 232–236 (2014). https://doi.org/10.1007/s12288-014-0338-8

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  • DOI: https://doi.org/10.1007/s12288-014-0338-8

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