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Clin J Gastroenterol. 2013 Dec;6(6):420-3. doi: 10.1007/s12328-013-0419-5. Epub 2013 Sep 07.

Laparoscopic splenectomy for the treatment of refractory thrombotic thrombocytopenic purpura.

Clinical journal of gastroenterology

Akira Umemura, Akira Sasaki, Hiroyuki Nitta, Toru Obuchi, Shigeaki Baba, Go Wakabayashi

Affiliations

  1. Department of Surgery, Iwate Medical University, 19-1 Uchimaru, Morioka, 020-8505, Japan. [email protected].
  2. Department of Surgery, Iwate Medical University, 19-1 Uchimaru, Morioka, 020-8505, Japan.

PMID: 26182130 DOI: 10.1007/s12328-013-0419-5

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a serious hematologic disorder with a high rate of morbidity and mortality. We report here on the surgical and homological outcomes of laparoscopic splenectomy (LS) in a patient with refractory TTP. A 69-year-old Japanese woman was referred to our hospital because of purpura in the lower extremities. In addition to the marked thrombocytopenia, hemolytic anemia and progressive mental disorder were noted. The ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motif 13) activity was undetectable and ADAMTS13 inhibitor was extremely increased. The diagnosis of TTP was made based on the clinical features and laboratory abnormalities. She received steroid-pulse therapy for 3 days, low-dose methylprednisolone continuous infusion and plasma exchange (PE) daily for 14 days. However, the patient was found to be refractory TTP to PE. The LS was performed at 15 days after diagnosis. The ADAMTS13 inhibitor was not detected after LS, and in addition, the platelet count had increased to over 100,000/mm(3) on postoperative day 17. The patient remains in remission 24 months after surgery. The results of our case demonstrate that LS is a safe and reasonable treatment option for patients with TTP refractory to PE.

Keywords: A disintegrin and metalloprotease with thrombospondin type 1 motif 13; Laparoscopic splenectomy; Plasma exchange; Thrombotic thrombocytopenic purpura

References

  1. Am J Hematol. 2006 Dec;81(12):895-900 - PubMed
  2. Br J Haematol. 2005 Sep;130(5):768-76 - PubMed
  3. Surg Endosc. 2008 Apr;22(4):821-48 - PubMed
  4. N Engl J Med. 1991 Aug 8;325(6):393-7 - PubMed
  5. J Clin Apher. 2003;18(2):51-4 - PubMed
  6. Can J Surg. 2010 Oct;53(5):349-55 - PubMed
  7. Arch Surg. 2001 Nov;136(11):1236-8; discussion 1239 - PubMed
  8. N Engl J Med. 2002 Aug 22;347(8):589-600 - PubMed
  9. Blood. 1998 Apr 15;91(8):2839-46 - PubMed
  10. Am J Hematol. 1999 Sep;62(1):56-7 - PubMed
  11. N Engl J Med. 1998 Nov 26;339(22):1585-94 - PubMed
  12. Blood. 2004 Jun 1;103(11):4043-9 - PubMed

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