Display options
Cite
Musa F, Baidas S. Idiopathic Relapsing Thrombotic Thrombocytopenic Purpura with Persistent ADAMTS13 Inhibitor Activity Treated Sequentially with Plasmapheresis, Rituximab, Cyclophosphamide and Splenectomy. Case Rep Oncol. 2015;8(1):196-9doi: 10.1159/000381868.
Musa, F., & Baidas, S. (2015). Idiopathic Relapsing Thrombotic Thrombocytopenic Purpura with Persistent ADAMTS13 Inhibitor Activity Treated Sequentially with Plasmapheresis, Rituximab, Cyclophosphamide and Splenectomy. Case reports in oncology, 8(1), 196-9. https://doi.org/10.1159/000381868
Musa, Faisal, and Baidas, Said. "Idiopathic Relapsing Thrombotic Thrombocytopenic Purpura with Persistent ADAMTS13 Inhibitor Activity Treated Sequentially with Plasmapheresis, Rituximab, Cyclophosphamide and Splenectomy." Case reports in oncology vol. 8,1 (2015): 196-9. doi: https://doi.org/10.1159/000381868
Musa F, Baidas S. Idiopathic Relapsing Thrombotic Thrombocytopenic Purpura with Persistent ADAMTS13 Inhibitor Activity Treated Sequentially with Plasmapheresis, Rituximab, Cyclophosphamide and Splenectomy. Case Rep Oncol. 2015 Apr 22;8(1):196-9. doi: 10.1159/000381868. eCollection 2015. PMID: 26034479; PMCID: PMC4448046.
Copy Download .nbib Format: NLM
Share it on

Case Rep Oncol. 2015 Apr 22;8(1):196-9. doi: 10.1159/000381868. eCollection 2015.

Idiopathic Relapsing Thrombotic Thrombocytopenic Purpura with Persistent ADAMTS13 Inhibitor Activity Treated Sequentially with Plasmapheresis, Rituximab, Cyclophosphamide and Splenectomy.

Case reports in oncology

Faisal Musa, Said Baidas

Affiliations

  1. University of Florida Health Cancer Center at Orlando Health, Orlando, Fla., USA.

PMID: 26034479 PMCID: PMC4448046 DOI: 10.1159/000381868

Abstract

We here describe a patient with an idiopathic thrombotic thrombocytopenic purpura (TTP) secondary to an ADAMTS13 inhibitor that continued to be dependent on plasmapheresis until the patient was treated with rituximab. TTP manifestations subsided with rituximab treatment in spite of a persistently low ADAMTS13 activity and continued a detectable inhibitor activity until the patient developed an intolerance to rituximab due to an allergic reaction when cyclophosphamide was added; this resulted in a normalization of ADAMTS13 activity and the disappearance of the inhibitor. Later, the patient developed an intolerance to rituximab due to a severe allergic reaction. Soon after stopping rituximab, the ADAMTS13 activity level dipped below 5% in addition to the appearance of the ADAMTS13 inhibitor. The patient had a splenectomy after rituximab and cyclophosphamide treatment; the medication was stopped based on several case reports of a complete remission of TTP after splenectomy. We believe that the reason TTP went into remission in our patient was because of rituximab treatment, in spite of both persistently low ADAMTS13 activity and a detectable inhibitor activity due to reducing the release of von Willebrand factor large multimers from the endothelial cells. We found that ADAMTS13 activity normalized and the inhibitor activity became undetectable when cyclophosphamide was added to rituximab. We suggest adding cyclophosphamide to rituximab for the treatment of patients with persistent ADAMTS13 inhibitors in order to prolong the remission period and lower the rate of relapse.

Keywords: ADAMTS13; Idiopathic relapsing thrombotic thrombocytopenic purpura; Plasmapheresis; Rituximab

References

  1. Br J Haematol. 2004 Mar;124(6):787-95 - PubMed
  2. Semin Hematol. 2004 Jan;41(1):75-82 - PubMed
  3. N Engl J Med. 1991 Aug 8;325(6):393-7 - PubMed
  4. Blood Cells Mol Dis. 2002 May-Jun;28(3):385-91 - PubMed
  5. Mayo Clin Proc. 2003 Apr;78(4):421-30 - PubMed
  6. Ann Intern Med. 2004 Feb 17;140(4):314-5 - PubMed
  7. Curr Opin Hematol. 2001 Sep;8(5):286-93 - PubMed
  8. Blood. 2004 Jun 1;103(11):4043-9 - PubMed
  9. Am J Obstet Gynecol. 1976 Oct 15;126(4):452-8 - PubMed
  10. Haematologica. 1999 Mar;84(3):260-9 - PubMed
  11. Br J Haematol. 2001 Jun;113(3):649-51 - PubMed
  12. Ann Intern Med. 1988 Aug 1;109(3):194-7 - PubMed
  13. Blood. 2005 Aug 1;106(3):925-8 - PubMed

Publication Types