Korean J Lab Med.  2006 Apr;26(2):119-122. 10.3343/kjlm.2006.26.2.119.

A Splenectomy in Plasma Exchange Refractory Thrombotic Thrombocytopenia Purpura

Affiliations
  • 1Department of Laboratory Medicine, School of Medicine, Wonkwang University, Iksan, Korea. jin20@wonkwang.ac.kr
  • 2Department of Internal Medicine, School of Medicine, Wonkwang University, Iksan, Korea.
  • 3Department of Surgery, School of Medicine, Wonkwang University, Iksan, Korea.
  • 4Institute of Wonkwang Medical Science, School of Medicine, Wonkwang University, Iksan, Korea.

Abstract

Thrombotic thrombocytopenic purpura (TTP) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, and variable abnormalities in renal function and mental status. The pathogenesis of TTP is related to an inhibitor or deficiency of the von Willebrand factor (vWF)-cleaving protease (a disintegrin and metalloprotease with thrombospondin type 1 repeats; ADAMTS-13) that cleaves the large vWF multimers. Uncleaved, large vWF molecules are present in TTP and induce thrombosis in small vessels. Even though plasma exchange was proven effective in TTP, 20-40% of the cases showed refractory to plasma exchange. We describe a 41 years old female with plasma exchange refractory TTP who was completely recovered from anemia, thrombocytopenia, and accompanying symptoms following splenectomy.

Keyword

TTP; vWF; ADAMTS-13; Plasma Exchange; Splenectomy

MeSH Terms

Adult
Anemia
Anemia, Hemolytic
Female
Fever
Humans
Plasma Exchange*
Plasma*
Purpura*
Purpura, Thrombotic Thrombocytopenic
Splenectomy*
Thrombocytopenia*
Thrombosis
Thrombospondins
von Willebrand Factor
Thrombospondins
von Willebrand Factor

Figure

  • Fig. 1. The sequential change of the value of lactate dehydrogenase (▴) and platelet number (Δ) before and after plasma exchanges, splenectomy (), vincristine (), and steroid therapy.


Reference

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