Abstract | BACKGROUND: STUDY DESIGN AND METHODS: The activities of vWF-CPase and its inhibitor were measured in 27 patients with nonfamilial TTP and hemolytic-uremic syndrome (HUS) to examine the relationship between the clinical variables and vWF-CPase activity. RESULTS: Eight of nine patients with HUS had more than 40 percent of vWF-CPase activity, whereas one had 28 percent of the normal level at the acute phase. Ten of 12 TTP patients with a good outcome had a severe deficiency of vWF-CPase activity and its inhibitor, whereas four of six patients with a poor outcome had a moderate deficiency of vWF-CPase activity along with a lack of the inhibitor. PE produced normalization of the vWF-CPase activity and neutralization of the inhibitor in TTP patients with a good outcome; however, some TTP patients with vWF-CPase inhibitor had relapsed and required an immunosuppressive therapy. The response to the combination therapy with PE and immunosuppressive treatment was poor in TTP patients without a severe deficiency of vWF-CPase activity. CONCLUSION: Assays of vWF-CPase activity and its inhibitor may be useful for predicting the response to therapy and the outcome of patients with TTP. In some patients, nonfamilial TTP with a poor prognosis may not be caused by a constitutional or acquired deficiency of vWF-CPase with its inhibitor. Although PE and immunosuppressive therapy are effective in patients with nonfamilial TTP and a vWF-CPase inhibitor, other therapeutic modalities may be needed for nonfamilial TTP with unknown etiology.
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Authors | Yoshitaka Mori, Hideo Wada, Esteban C Gabazza, Nobuyuki Minami, Tsutomu Nobori, Hiroshi Shiku, Hideo Yagi, Hiromichi Ishizashi, Masanori Matsumoto, Yoshihiro Fujimura |
Journal | Transfusion
(Transfusion)
Vol. 42
Issue 5
Pg. 572-80
(May 2002)
ISSN: 0041-1132 [Print] United States |
PMID | 12084165
(Publication Type: Comparative Study, Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Autoantibodies
- Biomarkers
- Immunosuppressive Agents
- von Willebrand Factor
- ADAM Proteins
- Metalloendopeptidases
- ADAMTS13 Protein
- ADAMTS13 protein, human
- Methylprednisolone
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Topics |
- ADAM Proteins
- ADAMTS13 Protein
- Adolescent
- Adult
- Aged
- Antibody Specificity
- Autoantibodies
(blood, immunology)
- Autoimmune Diseases
(blood, enzymology, etiology, therapy)
- Biomarkers
- Combined Modality Therapy
- Female
- Hemolytic-Uremic Syndrome
(blood, chemically induced, enzymology, therapy)
- Humans
- Immunosuppressive Agents
(therapeutic use)
- Male
- Metalloendopeptidases
(antagonists & inhibitors, blood, deficiency, immunology)
- Methylprednisolone
(therapeutic use)
- Middle Aged
- Plasma Exchange
- Prognosis
- Purpura, Thrombotic Thrombocytopenic
(blood, drug therapy, enzymology, therapy)
- Recurrence
- Retrospective Studies
- Severity of Illness Index
- Treatment Failure
- von Willebrand Factor
(metabolism)
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