Is there a shared pathophysiology for thrombotic thrombocytopenic purpura and hemolytic-uremic syndrome?

被引:25
作者
Desch, Karl
Motto, David
机构
[1] Univ Iowa, Dept Internal Med, Iowa City, IA 52242 USA
[2] Univ Iowa, Dept Pediat, Iowa City, IA 52242 USA
[3] Univ Michigan, Inst Life Sci, Dept Pediat, Ann Arbor, MI 48109 USA
来源
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY | 2007年 / 18卷 / 09期
关键词
D O I
10.1681/ASN.2007010062
中图分类号
R5 [内科学]; R69 [泌尿科学(泌尿生殖系疾病)];
学科分类号
1002 ; 100201 ;
摘要
Thrombotic microangiopathy is characterized by microvascular thrombosis coupled with thrombocytopenia, hemolytic anemia, and red blood cell fragmentation. Familiar to nephrologists and hematologists alike, classically associated with thrombotic microangiopathy are the hemolytic-uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP), the histories and presentations of which are closely intertwined. Not surprising, these two disorders are considered by many to be manifestations of the same disease process, whereas others consider HUS and TTP to be distinct clinical and pathologic entities. Herein are reviewed HUS and TTP along with recent progress shedding new light on possible shared pathophysiologic mechanisms for these two intriguing disorders.
引用
收藏
页码:2457 / 2460
页数:4
相关论文
共 15 条
[1]   Prothrombotic coagulation abnormalities preceding the hemolytic-uremic syndrome [J].
Chandler, WL ;
Jelacic, S ;
Boster, DR ;
Ciol, MA ;
Williams, GD ;
Watkins, SL ;
Igarashi, T ;
Tarr, PI .
NEW ENGLAND JOURNAL OF MEDICINE, 2002, 346 (01) :23-32
[2]   Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome [J].
de Jorge, Elena Goicoechea ;
Harris, Claire L. ;
Esparza-Gordillo, Jorge ;
Carreras, Luis ;
Arranz, Elena Aller ;
Garrido, Cynthia Abarrategui ;
Lopez-Trascasa, Margarita ;
Sanchez-Corral, Pilar ;
Morgan, B. Paul ;
Rodriguez de Cordoba, Santiago .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2007, 104 (01) :240-245
[3]   Platelet activation by Shiga toxin and circulatory factors as a pathogenetic mechanism in the hemolytic uremic syndrome [J].
Karpman, D ;
Papadopoulou, D ;
Nilsson, K ;
Sjögren, AC ;
Mikaelsson, C ;
Lethagen, S .
BLOOD, 2001, 97 (10) :3100-3108
[4]   Atypical haemolytic uraemic syndrome [J].
Kavanagh, David ;
Goodship, Timothy H. J. ;
Richards, Anna .
BRITISH MEDICAL BULLETIN, 2006, 77-78 :5-22
[5]   ADAMTS13 turns 3 [J].
Levy, GG ;
Motto, DG ;
Ginsburg, D .
BLOOD, 2005, 106 (01) :11-17
[6]   Cleavage of von Willebrand factor by ADAMTS-13 on endothelial cells [J].
López, JA ;
Dong, JF .
SEMINARS IN HEMATOLOGY, 2004, 41 (01) :15-23
[7]   Mechanisms of disease - Thrombotic microangiopathies [J].
Moake, JL .
NEW ENGLAND JOURNAL OF MEDICINE, 2002, 347 (08) :589-600
[8]   Shigatoxin triggers thrombotic thrombocytopenic purpura in genetically susceptible ADAMTS13-deficient mice [J].
Motto, DG ;
Chauhan, AK ;
Zhu, GJ ;
Homeister, J ;
Lamb, CB ;
Desch, KC ;
Zhang, WR ;
Tsai, HM ;
Wagner, DD ;
Ginsburg, D .
JOURNAL OF CLINICAL INVESTIGATION, 2005, 115 (10) :2752-2761
[9]   Hemolytic uremic syndrome-associated Shiga toxins promote endothelial-cell secretion and impair ADAMTS13 cleavage of unusually large von Willebrand factor multimers [J].
Nolasco, LH ;
Turner, NA ;
Bernardo, A ;
Tao, ZY ;
Cleary, TG ;
Dong, JF ;
Moake, JL .
BLOOD, 2005, 106 (13) :4199-4209
[10]   Complement factor H mutation in familial thrombotic thrombocytopenic purpura with ADAMTS13 deficiency and renal involvement [J].
Noris, M ;
Bucchioni, S ;
Galbusera, M ;
Donadelli, R ;
Bresin, E ;
Castelletti, F ;
Caprioli, J ;
Brioschi, S ;
Scheiflinger, F ;
Remuzzi, G .
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, 2005, 16 (05) :1177-1183