| Is it quinine TTP/HUS or quinine TMA? ADAMTS13 levels and implications for therapy. | |
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MedLine Citation:
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PMID: 19260037 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Thrombocytopenia with or without microangiopathy following quinine is often referred to as quinine "hypersensitivity." When schistocytes are present it is frequently termed "quinine-associated TTP/HUS." A severe deficiency of the vWF-cleaving protease, ADAMTS13, is associated with idiopathic TTP. A previous study of patients with "quinine-associated TTP/HUS" found that ADAMTS13 activities were not abnormal in 12/12 patients. A retrospective review of TTP patients with quinine-associated thrombotic microangiopathy (TMA) for whom ADAMTS13 was measured before plasma exchange was performed. Six patients were identified. All were females (age range: 43 to 73, mean = 61.7 years) and had taken quinine for leg cramps. Four of the six experienced renal failure requiring dialysis. Five of the patients had D-Dimers levels measured, all were elevated. In four patients the levels were > or = 18 times the upper limit of normal. ADAMTS13 was normal in four patients and mildly decreased in two patients. We conclude that while thrombocytopenia and schistocytosis can be seen in quinine-associated TTP/HUS, the pathophysiology seems to be distinct from that seen in most cases of idiopathic TTP (i.e., severely decreased ADAMTS13 with an inhibitor). We recommend that a TMA in association with quinine be consistently referred to as quinine-associated thrombotic microangiopathy (quinine-TMA) to better distinguish this entity from idiopathic TTP. The use of plasma exchange in quinine-TMA is called into question. |
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Authors:
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Yara A Park; Shauna N Hay; Karen E King; Karen Matevosyan; Jessica Poisson; Amy Powers; Ravindra Sarode; Beth Shaz; Mark E Brecher |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Journal of clinical apheresis Volume: 24 ISSN: 1098-1101 ISO Abbreviation: J Clin Apher Publication Date: 2009 |
Date Detail:
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Created Date: 2009-06-11 Completed Date: 2009-08-18 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8216305 Medline TA: J Clin Apher Country: United States |
Other Details:
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Languages: eng Pagination: 115-9 Citation Subset: IM |
Affiliation:
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Department of Pathology and Laboratory Medicine, University of North Carolina, Chapel Hill, North Carolina, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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ADAM Proteins
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blood* Adult Aged Aged, 80 and over Female Fibrin Fibrinogen Degradation Products Humans Kidney Failure / blood, chemically induced, therapy Middle Aged Muscle Cramp / blood, drug therapy Muscle Relaxants, Central / administration & dosage, adverse effects* Plasma Exchange* Purpura, Thrombocytopenic, Idiopathic / blood*, chemically induced*, therapy* Quinine / administration & dosage, adverse effects* Renal Dialysis Retrospective Studies |
| Chemical | |
Reg. No./Substance:
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0/Fibrin Fibrinogen Degradation Products; 0/Muscle Relaxants, Central; 0/fibrin fragment D; 130-95-0/Quinine; EC 3.4.24.-/ADAM Proteins; EC 3.4.24.-/ADAMTS13 protein, human |
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