The thrombotic microangiopathies are microvascular occlusivedisorders characterized by systemic or intrarenal aggregationof platelets, thrombocytopenia, and mechanical injury to erythrocytes.In thrombotic thrombocytopenic purpura, systemic microvascularaggregation of platelets causes ischemia in the brain and otherorgans. In the hemolytic–uremic syndrome, platelet–fibrinthrombi occlude predominantly the renal circulation. Thromboticthrombocytopenic purpura was initially described by Moschcowitzin 1924,1 and the hemolytic–uremic syndrome by Gasseret al.2 in 1955. Both disorders remained mysterious until the1980s. In 1982, "unusually large" multimers of von Willebrandfactor released from endothelial cells were found to accumulatein the plasma of patients with chronic . . . [Full Text of this Article]
Clinical Presentations
Pathophysiology
Thrombotic Thrombocytopenic Purpura
The Hemolytic–Uremic Syndrome
Deficiency of Plasma Factor H
Renal or Systemic Thrombotic Microangiopathies of Unknown Causes
Therapy
Thrombotic Thrombocytopenic Purpura
The Hemolytic–Uremic Syndrome
Overview of Therapy
Source Information
From Baylor College of Medicine and Rice University — both in Houston.
Address reprint requests to Dr. Moake at the Hematology–Oncology Section, Methodist Hospital, MS 902, Main Bldg., 6565 Fannin, Houston, TX 77030, or at jmoake@rice.edu.
Turner, N. A., Nolasco, L., Ruggeri, Z. M., Moake, J. L.
(2009). Endothelial cell ADAMTS-13 and VWF: production, release, and VWF string cleavage. Blood
114: 5102-5111
[Abstract][Full Text]
Licht, C., Pluthero, F. G., Li, L., Christensen, H., Habbig, S., Hoppe, B., Geary, D. F., Zipfel, P. F., Kahr, W. H. A.
(2009). Platelet-associated complement factor H in healthy persons and patients with atypical HUS. Blood
114: 4538-4545
[Abstract][Full Text]
Ehsanipoor, R. M., Rajan, P., Holcombe, R. F., Wing, D. A.
(2009). Limitations of ADAMTS-13 Activity Level in Diagnosing Thrombotic Thrombocytopenic Purpura in Pregnancy. CLIN APPL THROMB HEMOST
15: 585-587
[Abstract]
Sucker, C., Schmitz, M., Hetzel, G. R., Grabensee, B., Maruhn-Debowski, B., Ostojic, L., Scharf, R. E., Zotz, R. B.
(2009). Are Prothrombotic Variants of Platelet Glycoprotein Receptor Polymorphisms Involved in the Pathogenesis of Thrombotic Microangiopathies?. CLIN APPL THROMB HEMOST
15: 402-407
[Abstract]
Delvaeye, M., Noris, M., De Vriese, A., Esmon, C. T., Esmon, N. L., Ferrell, G., Del-Favero, J., Plaisance, S., Claes, B., Lambrechts, D., Zoja, C., Remuzzi, G., Conway, E. M.
(2009). Thrombomodulin Mutations in Atypical Hemolytic-Uremic Syndrome. NEJM
361: 345-357
[Abstract][Full Text]
Kreitman, R. J., Stetler-Stevenson, M., Margulies, I., Noel, P., FitzGerald, D. J.P., Wilson, W. H., Pastan, I.
(2009). Phase II Trial of Recombinant Immunotoxin RFB4(dsFv)-PE38 (BL22) in Patients With Hairy Cell Leukemia. JCO
27: 2983-2990
[Abstract][Full Text]
Li, X., Zhang, W., Ren, H., Pan, X., Chen, N.
(2009). Malignant hypertension complicated by acute renal failure. BMJ Case Reports
2009: bcr1020081116-bcr1020081116
[Abstract][Full Text]
Sucker, C., Kurschat, C., Farokhzad, F., Hetzel, G. R., Grabensee, B., Maruhn-Debowski, B., Loncar, R., Scharf, R. E., Zotz, R. B.
(2009). The TT Genotype of the C677T Polymorphism in the Methylentetrahydrofolate Reductase as a Risk Factor in Thrombotic Microangiopathies: Results From a Pilot Study. CLIN APPL THROMB HEMOST
15: 283-288
[Abstract]
Sucker, C., Kurschat, C., Hetzel, G. R., Grabensee, B., Maruhn-Debowski, B., Loncar, R., Ostojic, L., Scharf, R. E., Zotz, R. B.
(2009). The G1691A Mutation of the Factor V Gene (Factor V Leiden) and the G20210A Mutation of the Prothrombin Gene as Risk Factors in Thrombotic Microangiopathies. CLIN APPL THROMB HEMOST
15: 360-363
[Abstract]
Taranta, A., Gianviti, A., Palma, A., De Luca, V., Mannucci, L., Procaccino, M. A., Ghiggeri, G. M., Caridi, G., Fruci, D., Ferracuti, S., Ferretti, A., Pecoraro, C., Gaido, M., Penza, R., Edefonti, A., Murer, L., Tozzi, A. E., Emma, F., (on behalf of the Italian HUS surveillance study),
(2009). Genetic risk factors in typical haemolytic uraemic syndrome. Nephrol Dial Transplant
24: 1851-1857
[Abstract][Full Text]
Sucker, C., Farokhzad, F., Kurschat, C., Grabensee, B., Scharf, R. E., Zotz, R. B., Maruhn-Debowski, B., Hetzel, G. R.
(2009). The Homozygous Leu Variant of the Factor XIII Val34Leu Polymorphism as a Risk Factor for the Manifestation of Thrombotic Microangiopathies. CLIN APPL THROMB HEMOST
15: 197-200
[Abstract]
Letchumanan, P., Ng, H.-J., Lee, L.-H., Thumboo, J.
(2009). A comparison of thrombotic thrombocytopenic purpura in an inception cohort of patients with and without systemic lupus erythematosus. Rheumatology (Oxford)
48: 399-403
[Abstract][Full Text]
Galbusera, M., Noris, M., Remuzzi, G.
(2009). Inherited thrombotic thrombocytopenic purpura. haematol
94: 166-170
[Full Text]
Garagiola, I., Valsecchi, C., Lavoretano, S., Oren, H., Bohm, M., Peyvandi, F.
(2008). Nonsense-mediated mRNA decay in the ADAMTS13 gene caused by a 29-nucleotide deletion. haematol
93: 1678-1685
[Abstract][Full Text]
Malbranque, S., Piercecchi-Marti, M. D., Thomas, L., Barbey, C., Courcier, D., Bucher, B., Ridarch, A., Smadja, D., Warrell, D. A.
(2008). Fatal Diffuse Thrombotic Microangiopathy after a Bite by the "Fer-de-Lance" Pit Viper (Bothrops lanceolatus) of Martinique. Am J Trop Med Hyg
78: 856-861
[Abstract][Full Text]
Denis, C. V.
(2008). Pas de deux between VWF and ADAMTS13. Blood
111: 3306-3307
[Full Text]
Chauhan, A. K., Walsh, M. T., Zhu, G., Ginsburg, D., Wagner, D. D., Motto, D. G.
(2008). The combined roles of ADAMTS13 and VWF in murine models of TTP, endotoxemia, and thrombosis. Blood
111: 3452-3457
[Abstract][Full Text]
Dickinson, C. E., Gould, D. H., Davidson, A. H., Avery, P. R., Legare, M. E., Hyatt, D. R., DebRoy, C.
(2008). Hemolytic-uremic syndrome in a postpartum mare concurrent with encephalopathy in the neonatal foal. jvdi
20: 239-242
[Abstract][Full Text]
Lammle, B., Kremer Hovinga, J. A., George, J. N.
(2008). Acquired thrombotic thrombocytopenic purpura: ADAMTS13 activity, anti-ADAMTS13 autoantibodies and risk of recurrent disease. haematol
93: 172-177
[Full Text]
Peyvandi, F., Lavoretano, S., Palla, R., Feys, H. B., Vanhoorelbeke, K., Battaglioli, T., Valsecchi, C., Canciani, M. T., Fabris, F., Zver, S., Reti, M., Mikovic, D., Karimi, M., Giuffrida, G., Laurenti, L., Mannucci, P. M.
(2008). ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission. haematol
93: 232-239
[Abstract][Full Text]
Fang, C. J., Fremeaux-Bacchi, V., Liszewski, M. K., Pianetti, G., Noris, M., Goodship, T. H. J., Atkinson, J. P.
(2008). Membrane cofactor protein mutations in atypical hemolytic uremic syndrome (aHUS), fatal Stx-HUS, C3 glomerulonephritis, and the HELLP syndrome. Blood
111: 624-632
[Abstract][Full Text]
Limal, N., Cacoub, P., Sene, D., Guichard, I., Piette, J.-C.
(2008). Letter to the Editor: Rituximab for the treatment of thrombotic thrombocytopenic purpura in systemic lupus erythematosus. Lupus
17: 69-71
Bockmeyer, C. L., Claus, R. A., Budde, U., Kentouche, K., Schneppenheim, R., Losche, W., Reinhart, K., Brunkhorst, F. M.
(2008). Inflammation-associated ADAMTS13 deficiency promotes formation of ultra-large von Willebrand factor. haematol
93: 137-140
[Abstract][Full Text]
Falk, R. J., Hamm, L. L., Josephson, M. A., Adler, S., Singh, A. K.
(2007). The Nephrology Quiz and Questionnaire: 2006. CJASN
2: 1375-1388
[Full Text]
Chang, A., Hingorani, S., Kowalewska, J., Flowers, M. E.D., Aneja, T., Smith, K. D., Meehan, S. M., Nicosia, R. F., Alpers, C. E.
(2007). Spectrum of Renal Pathology in Hematopoietic Cell Transplantation: A Series of 20 Patients and Review of the Literature. CJASN
2: 1014-1023
[Abstract][Full Text]
Desch, K., Motto, D.
(2007). Is There a Shared Pathophysiology for Thrombotic Thrombocytopenic Purpura and Hemolytic-Uremic Syndrome?. J. Am. Soc. Nephrol.
18: 2457-2460
[Abstract][Full Text]
Swisher, K. K., Doan, J. T., Vesely, S. K., Kwaan, H. C., Kim, B., Lammle, B., Kremer Hovinga, J. A., George, J. N.
(2007). Pancreatitis preceding acute episodes of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: report of five patients with a systematic review of published reports. haematol
92: 936-943
[Abstract][Full Text]
Riksen, N. P, Luken, B. M, Klasen, I. S, Voorberg, J., Crama, N., van Deuren, M.
(2007). Antibodies against the CUB1-2 domains of ADAMTS13 in a patient with benign monoclonal gammopathy: no causal relationship. haematol
92: e74-e76
[Abstract][Full Text]
Sucker, C., Hetzel, G. R., Farokhzad, F., Dahhan, F., Schmitz, M., Kurschat, C., Grabensee, B., Maruhn-Debowski, B., Zotz, R., Scharf, R.
(2007). Association of genotypes of thrombin-activatable fibrinolysis inhibitors with thrombotic microangiopathies--a pilot study. Nephrol Dial Transplant
22: 1347-1350
[Abstract][Full Text]
Ferrari, S., Scheiflinger, F., Rieger, M., Mudde, G., Wolf, M., Coppo, P., Girma, J.-P., Azoulay, E., Brun-Buisson, C., Fakhouri, F., Mira, J.-P., Oksenhendler, E., Poullin, P., Rondeau, E., Schleinitz, N., Schlemmer, B., Teboul, J.-L., Vanhille, P., Vernant, J.-P., Meyer, D., Veyradier, A., for the French and Clinical Biological Network on,
(2007). Prognostic value of anti-ADAMTS13 antibody features (Ig isotype, titer, and inhibitory effect) in a cohort of 35 adult French patients undergoing a first episode of thrombotic microangiopathy with undetectable ADAMTS13 activity. Blood
109: 2815-2822
[Abstract][Full Text]
George, J. N.
(2007). Evaluation and Management of Patients With Thrombotic Thrombocytopenic Purpura. J Intensive Care Med
22: 82-91
[Abstract]
Heinen, S., Jozsi, M., Hartmann, A., Noris, M., Remuzzi, G., Skerka, C., Zipfel, P. F.
(2007). Hemolytic Uremic Syndrome: A Factor H Mutation (E1172Stop) Causes Defective Complement Control at the Surface of Endothelial Cells. J. Am. Soc. Nephrol.
18: 506-514
[Abstract][Full Text]
Chee, C. E., Mohr, D. N.
(2007). 71-Year-Old Woman With Fever and Altered Mental Status. Mayo Clin Proc.
82: 237-240
[Full Text]
de Jorge, E. G., Harris, C. L., Esparza-Gordillo, J., Carreras, L., Arranz, E. A., Garrido, C. A., Lopez-Trascasa, M., Sanchez-Corral, P., Morgan, B. P., de Cordoba, S. R.
(2007). Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome. Proc. Natl. Acad. Sci. USA
104: 240-245
[Abstract][Full Text]
Crowther, M. A., Abshire, T. C.
(2007). Hemostasis and thrombosis. ASH-SAP
2007: 361-407
[Full Text]
Taylor, J. L., Palmer, S. M.
(2006). Critical Care Perspective on Immunotherapy in Lung Transplantation. J Intensive Care Med
21: 327-344
[Abstract]
Izzedine, H., Isnard-Bagnis, C., Launay-Vacher, V., Mercadal, L., Tostivint, I., Rixe, O., Brocheriou, I., Bourry, E., Karie, S., Saeb, S., Casimir, N., Billemont, B., Deray, G.
(2006). Gemcitabine-induced thrombotic microangiopathy: a systematic review. Nephrol Dial Transplant
21: 3038-3045
[Full Text]
Shang, D., Zheng, X. W., Niiya, M., Zheng, X. L.
(2006). Apical sorting of ADAMTS13 in vascular endothelial cells and Madin-Darby canine kidney cells depends on the CUB domains and their association with lipid rafts. Blood
108: 2207-2215
[Abstract][Full Text]
Nishi, H., Hanafusa, N., Kondo, Y., Nangaku, M., Sugawara, Y., Makuuchi, M., Noiri, E., Fujita, T.
(2006). Clinical Outcome of Thrombotic Microangiopathy after Living-Donor Liver Transplantation Treated with Plasma Exchange Therapy. CJASN
1: 811-819
[Abstract][Full Text]
Hingorani, S.
(2006). Chronic Kidney Disease in Long-Term Survivors of Hematopoietic Cell Transplantation: Epidemiology, Pathogenesis, and Treatment. J. Am. Soc. Nephrol.
17: 1995-2005
[Abstract][Full Text]
Fremeaux-Bacchi, V., Moulton, E. A., Kavanagh, D., Dragon-Durey, M.-A., Blouin, J., Caudy, A., Arzouk, N., Cleper, R., Francois, M., Guest, G., Pourrat, J., Seligman, R., Fridman, W. H., Loirat, C., Atkinson, J. P.
(2006). Genetic and Functional Analyses of Membrane Cofactor Protein (CD46) Mutations in Atypical Hemolytic Uremic Syndrome. J. Am. Soc. Nephrol.
17: 2017-2025
[Abstract][Full Text]
George, J. N.
(2006). Clinical practice. Thrombotic thrombocytopenic purpura.. NEJM
354: 1927-1935
[Full Text]
Banno, F., Kokame, K., Okuda, T., Honda, S., Miyata, S., Kato, H., Tomiyama, Y., Miyata, T.
(2006). Complete deficiency in ADAMTS13 is prothrombotic, but it alone is not sufficient to cause thrombotic thrombocytopenic purpura. Blood
107: 3161-3166
[Abstract][Full Text]
Chauhan, A. K., Motto, D. G., Lamb, C. B., Bergmeier, W., Dockal, M., Plaimauer, B., Scheiflinger, F., Ginsburg, D., Wagner, D. D.
(2006). Systemic antithrombotic effects of ADAMTS13. JEM
203: 767-776
[Abstract][Full Text]
Hassenpflug, W. A., Budde, U., Obser, T., Angerhaus, D., Drewke, E., Schneppenheim, S., Schneppenheim, R.
(2006). Impact of mutations in the von Willebrand factor A2 domain on ADAMTS13-dependent proteolysis. Blood
107: 2339-2345
[Abstract][Full Text]
Donadelli, R., Orje, J. N., Capoferri, C., Remuzzi, G., Ruggeri, Z. M.
(2006). Size regulation of von Willebrand factor-mediated platelet thrombi by ADAMTS13 in flowing blood. Blood
107: 1943-1950
[Abstract][Full Text]
Mullarky, I. K., Szaba, F. M., Berggren, K. N., Kummer, L. W., Wilhelm, L. B., Parent, M. A., Johnson, L. L., Smiley, S. T.
(2006). Tumor Necrosis Factor Alpha and Gamma Interferon, but Not Hemorrhage or Pathogen Burden, Dictate Levels of Protective Fibrin Deposition during Infection. Infect. Immun.
74: 1181-1188
[Abstract][Full Text]
Ono, T., Mimuro, J., Madoiwa, S., Soejima, K., Kashiwakura, Y., Ishiwata, A., Takano, K., Ohmori, T., Sakata, Y.
(2006). Severe secondary deficiency of von Willebrand factor-cleaving protease (ADAMTS13) in patients with sepsis-induced disseminated intravascular coagulation: its correlation with development of renal failure. Blood
107: 528-534
[Abstract][Full Text]
Desch, K., Motto, D.
(2005). ADAMTS13 and VWF: not just for TTP anymore?. Blood
106: 4024-4024
[Full Text]
Tao, Z., Peng, Y., Nolasco, L., Cal, S., Lopez-Otin, C., Li, R., Moake, J. L., Lopez, J. A., Dong, J.-f.
(2005). Recombinant CUB-1 domain polypeptide inhibits the cleavage of ULVWF strings by ADAMTS13 under flow conditions. Blood
106: 4139-4145
[Abstract][Full Text]
Nolasco, L. H., Turner, N. A., Bernardo, A., Tao, Z., Cleary, T. G., Dong, J.-f., Moake, J. L.
(2005). Hemolytic uremic syndrome-associated Shiga toxins promote endothelial-cell secretion and impair ADAMTS13 cleavage of unusually large von Willebrand factor multimers. Blood
106: 4199-4209
[Abstract][Full Text]
Guessous, F., Marcinkiewicz, M., Polanowska-Grabowska, R., Kongkhum, S., Heatherly, D., Obrig, T., Gear, A. R. L.
(2005). Shiga Toxin 2 and Lipopolysaccharide Induce Human Microvascular Endothelial Cells To Release Chemokines and Factors That Stimulate Platelet Function. Infect. Immun.
73: 8306-8316
[Abstract][Full Text]
Fremeaux-Bacchi, V, Kemp, E J, Goodship, J A, Dragon-Durey, M-A, Strain, L, Loirat, C, Deng, H-W, Goodship, T H J
(2005). The development of atypical haemolytic-uraemic syndrome is influenced by susceptibility factors in factor H and membrane cofactor protein: evidence from two independent cohorts. J. Med. Genet.
42: 852-856
[Abstract][Full Text]
Hulstein, J. J. J., de Groot, P. G., Silence, K., Veyradier, A., Fijnheer, R., Lenting, P. J.
(2005). A novel nanobody that detects the gain-of-function phenotype of von Willebrand factor in ADAMTS13 deficiency and von Willebrand disease type 2B. Blood
106: 3035-3042
[Abstract][Full Text]
Kreitman, R. J., Squires, D. R., Stetler-Stevenson, M., Noel, P., FitzGerald, D. J.P., Wilson, W. H., Pastan, I.
(2005). Phase I Trial of Recombinant Immunotoxin RFB4(dsFv)-PE38 (BL22) in Patients With B-Cell Malignancies. JCO
23: 6719-6729
[Abstract][Full Text]
Fuji, R. N., Patton, K. M., Steinbach, T. J., Schulman, F. Y., Bradley, G. A., Brown, T. T., Wilson, E. A., Summers, B. A.
(2005). Feline Systemic Reactive Angioendotheliomatosis: Eight Cases and Literature Review. Vet Pathol
42: 608-617
[Abstract][Full Text]
Rieger, M., Mannucci, P. M., Hovinga, J. A. K., Herzog, A., Gerstenbauer, G., Konetschny, C., Zimmermann, K., Scharrer, I., Peyvandi, F., Galbusera, M., Remuzzi, G., Bohm, M., Plaimauer, B., Lammle, B., Scheiflinger, F.
(2005). ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases. Blood
106: 1262-1267
[Abstract][Full Text]
Uemura, M., Tatsumi, K., Matsumoto, M., Fujimoto, M., Matsuyama, T., Ishikawa, M., Iwamoto, T.-a., Mori, T., Wanaka, A., Fukui, H., Fujimura, Y.
(2005). Localization of ADAMTS13 to the stellate cells of human liver. Blood
106: 922-924
[Abstract][Full Text]
Sakakura, M., Wada, H., Abe, Y., Nishioka, J., Tomatsu, H., Hamaguchi, Y., Oguni, S., Shiku, H., Nobori, T.
(2005). Usefulness of Measurement of Reticulated Platelets for Diagnosis of Idiopathic Thrombocytopenic Purpura. CLIN APPL THROMB HEMOST
11: 253-261
[Abstract]
Levy, G. G., Motto, D. G., Ginsburg, D.
(2005). ADAMTS13 turns 3. Blood
106: 11-17
[Abstract][Full Text]
Tao, Z., Wang, Y., Choi, H., Bernardo, A., Nishio, K., Sadler, J. E., Lopez, J. A., Dong, J.-f.
(2005). Cleavage of ultralarge multimers of von Willebrand factor by C-terminal-truncated mutants of ADAMTS-13 under flow. Blood
106: 141-143
[Abstract][Full Text]
Majerus, E. M., Anderson, P. J., Sadler, J. E.
(2005). Binding of ADAMTS13 to von Willebrand Factor. J. Biol. Chem.
280: 21773-21778
[Abstract][Full Text]
Noris, M., Bucchioni, S., Galbusera, M., Donadelli, R., Bresin, E., Castelletti, F., Caprioli, J., Brioschi, S., Scheiflinger, F., Remuzzi, G., for the International Registry of Recurrent and Fa,
(2005). Complement Factor H Mutation in Familial Thrombotic Thrombocytopenic Purpura with ADAMTS13 Deficiency and Renal Involvement. J. Am. Soc. Nephrol.
16: 1177-1183
[Abstract][Full Text]
Heemskerk, L., Scott-Douglas, N., Yilmaz, S., McLaughlin, K.
(2005). Resolution of thrombotic microangiopathy following renal transplant. Nephrol Dial Transplant
20: 639-641
[Full Text]
Linenberger, M. L., Price, T. H.
(2005). Use of Cellular and Plasma Apheresis in the Critically Ill Patient: Part II: Clinical Indications and Applications. J Intensive Care Med
20: 88-103
[Abstract]
Esparza-Gordillo, J., Jorge, E. G. d., Buil, A., Berges, L. C., Lopez-Trascasa, M., Sanchez-Corral, P., Cordoba, S. R. d.
(2005). Predisposition to atypical hemolytic uremic syndrome involves the concurrence of different susceptibility alleles in the regulators of complement activation gene cluster in 1q32. Hum Mol Genet
14: 703-712
[Abstract][Full Text]
Dragon-Durey, M.-A., Loirat, C., Cloarec, S., Macher, M.-A., Blouin, J., Nivet, H., Weiss, L., Fridman, W. H., Fremeaux-Bacchi, V.
(2005). Anti-Factor H Autoantibodies Associated with Atypical Hemolytic Uremic Syndrome. J. Am. Soc. Nephrol.
16: 555-563
[Abstract][Full Text]
Humphreys, B. D., Soiffer, R. J., Magee, C. C.
(2005). Renal Failure Associated with Cancer and Its Treatment: An Update. J. Am. Soc. Nephrol.
16: 151-161
[Full Text]
Wolf, G.
(2004). Not known from ADAM(TS-13)--novel insights into the pathophysiology of thrombotic microangiopathies. Nephrol Dial Transplant
19: 1687-1693
[Full Text]
Bernardo, A., Ball, C., Nolasco, L., Moake, J. F., Dong, J.-f.
(2004). Effects of inflammatory cytokines on the release and cleavage of the endothelial cell-derived ultralarge von Willebrand factor multimers under flow. Blood
104: 100-106
[Abstract][Full Text]
Klaus, C., Plaimauer, B., Studt, J.-D., Dorner, F., Lammle, B., Mannucci, P. M., Scheiflinger, F.
(2004). Epitope mapping of ADAMTS13 autoantibodies in acquired thrombotic thrombocytopenic purpura. Blood
103: 4514-4519
[Abstract][Full Text]
Fremeaux-Bacchi, V, Dragon-Durey, M-A, Blouin, J, Vigneau, C, Kuypers, D, Boudailliez, B, Loirat, C, Rondeau, E, Fridman, W H
(2004). Complement factor I: a susceptibility gene for atypical haemolytic uraemic syndrome. J. Med. Genet.
41: e84-e84
[Full Text]
Espinosa, G, Bucciarelli, S, Cervera, R, Lozano, M, Reverter, J-C, de la Red, G, Gil, V, Ingelmo, M, Font, J, Asherson, R A
(2004). Thrombotic microangiopathic haemolytic anaemia and antiphospholipid antibodies. Ann Rheum Dis
63: 730-736
[Abstract][Full Text]
Hertig, A., Rondeau, E.
(2004). Role of the Coagulation/Fibrinolysis System in Fibrin-Associated Glomerular Injury. J. Am. Soc. Nephrol.
15: 844-853
[Abstract][Full Text]
Padilla, A., Moake, J. L., Bernardo, A., Ball, C., Wang, Y., Arya, M., Nolasco, L., Turner, N., Berndt, M. C., Anvari, B., Lopez, J. A., Dong, J.-F.
(2004). P-selectin anchors newly released ultralarge von Willebrand factor multimers to the endothelial cell surface. Blood
103: 2150-2156
[Abstract][Full Text]
Zakarija, A., Bandarenko, N., Pandey, D. K., Auerbach, A., Raisch, D. W., Kim, B., Kwaan, H. C., McKoy, J. M., Schmitt, B. P., Davidson, C. J., Yarnold, P. R., Gorelick, P. B., Bennett, C. L.
(2004). Clopidogrel-Associated TTP: An Update of Pharmacovigilance Efforts Conducted by Independent Researchers, Pharmaceutical Suppliers, and the Food and Drug Administration. Stroke
35: 533-537
[Abstract][Full Text]
Lopez, J. A.
(2004). von Willebrand factor: polymorphism predicts prompt polypeptide proteolysis. Blood
103: 754-754
[Full Text]
Sadler, J. E., Moake, J. L., Miyata, T., George, J. N.
(2004). Recent Advances in Thrombotic Thrombocytopenic Purpura. ASH Education Book
2004: 407-423
[Abstract][Full Text]
Scheiflinger, F., Knobl, P., Trattner, B., Plaimauer, B., Mohr, G., Dockal, M., Dorner, F., Rieger, M.
(2003). Nonneutralizing IgM and IgG antibodies to von Willebrand factor-cleaving protease (ADAMTS-13) in a patient with thrombotic thrombocytopenic purpura. Blood
102: 3241-3243
[Abstract][Full Text]
Richards, A., Kemp, E. J., Liszewski, M. K., Goodship, J. A., Lampe, A. K., Decorte, R., Muslumanogglu, M. H., Kavukcu, S., Filler, G., Pirson, Y., Wen, L. S., Atkinson, J. P., Goodship, T. H. J.
(2003). Mutations in human complement regulator, membrane cofactor protein (CD46), predispose to development of familial hemolytic uremic syndrome. Proc. Natl. Acad. Sci. USA
100: 12966-12971
[Abstract][Full Text]
Neumann, H P H, Salzmann, M, Bohnert-Iwan, B, Mannuelian, T, Skerka, C, Lenk, D, Bender, B U, Cybulla, M, Riegler, P, Konigsrainer, A, Neyer, U, Bock, A, Widmer, U, Male, D A, Franke, G, Zipfel, P F
(2003). Haemolytic uraemic syndrome and mutations of the factor H gene: a registry-based study of German speaking countries. J. Med. Genet.
40: 676-681
[Abstract][Full Text]
Brass, L. F.
(2003). Thrombin and Platelet Activation. Chest
124
: 18S-25S
[Abstract][Full Text]
Dong, J.-f., Moake, J. L., Bernardo, A., Fujikawa, K., Ball, C., Nolasco, L., Lopez, J. A., Cruz, M. A.
(2003). ADAMTS-13 Metalloprotease Interacts with the Endothelial Cell-derived Ultra-large von Willebrand Factor. J. Biol. Chem.
278: 29633-29639
[Abstract][Full Text]
Bryant, A. E.
(2003). Biology and Pathogenesis of Thrombosis and Procoagulant Activity in Invasive Infections Caused by Group A Streptococci and Clostridium perfringens. Clin. Microbiol. Rev.
16: 451-462
[Abstract][Full Text]
Vesely, S. K., George, J. N., Lammle, B., Studt, J.-D., Alberio, L., El-Harake, M. A., Raskob, G. E.
(2003). ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood
102: 60-68
[Abstract][Full Text]
Ritchie, J. M., Wagner, P. L., Acheson, D. W. K., Waldor, M. K.
(2003). Comparison of Shiga Toxin Production by Hemolytic-Uremic Syndrome-Associated and Bovine-Associated Shiga Toxin-Producing Escherichia coli Isolates. Appl. Environ. Microbiol.
69: 1059-1066
[Abstract][Full Text]
George, J. N., Vesely, S. K.
(2003). Thrombotic Thrombocytopenic Purpura: From the Bench to the Bedside, but Not Yet to the Community. ANN INTERN MED
138: 152-153
[Full Text]
Rick, M. E., Walsh, C. E., Key, N. S.
(2003). Congenital Bleeding Disorders. ASH Education Book
2003: 559-574
[Abstract][Full Text]
Tarr, P. I., Tsai, H.-M., Chandler, W. L., Nzerue, C. M., Moake, J. L.
(2002). Thrombotic Microangiopathies. NEJM
347: 2171-2173
[Full Text]
O'Brien, S. J., Adak, G. K.
(2002). Escherichia coli O157:H7 -- Piecing Together the Jigsaw Puzzle. NEJM
347: 608-609
[Full Text]
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