title: Is it quinine TTP/HUS or quinine TMA? ADAMTS13 levels and implications for therapy.
abstract: 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.
authors.affiliation: Department of Pathology and Laboratory Medicine, University of North Carolina, Chapel Hill, North Carolina, USA.
authors.forename: Yara A
authors.initials: YA
authors.lastname: Park
authors.forename: Shauna N
authors.initials: SN
authors.lastname: Hay
authors.forename: Karen E
authors.initials: KE
authors.lastname: King
authors.forename: Karen
authors.initials: K
authors.lastname: Matevosyan
authors.forename: Jessica
authors.initials: J
authors.lastname: Poisson
authors.forename: Amy
authors.initials: A
authors.lastname: Powers
authors.forename: Ravindra
authors.initials: R
authors.lastname: Sarode
authors.forename: Beth
authors.initials: B
authors.lastname: Shaz
authors.forename: Mark E
authors.initials: ME
authors.lastname: Brecher
mesh_terms: D051722:ADAM Proteins / Q000097:blood*; D000071120:ADAMTS13 Protein; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D005338:Fibrin Fibrinogen Degradation Products; D006801:Humans; D008875:Middle Aged; D009120:Muscle Cramp / Q000097:blood / Q000188:drug therapy; D009125:Muscle Relaxants, Central / Q000008:administration & dosage / Q000009:adverse effects*; D010951:Plasma Exchange; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000097:blood* / Q000139:chemically induced* / Q000628:therapy*; D011803:Quinine / Q000008:administration & dosage / Q000009:adverse effects*; D006435:Renal Dialysis; D051437:Renal Insufficiency / Q000097:blood / Q000139:chemically induced / Q000628:therapy; D012189:Retrospective Studies
chemical_list: D005338:Fibrin Fibrinogen Degradation Products; D009125:Muscle Relaxants, Central; C036309:fibrin fragment D; D011803:Quinine; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human