thrombotic microangiopathies


Summary: Diseases that result in THROMBOSIS in MICROVASCULATURE. The two most prominent diseases are PURPURA, THROMBOTIC THROMBOCYTOPENIC; and HEMOLYTIC-UREMIC SYNDROME. Multiple etiological factors include VASCULAR ENDOTHELIAL CELL damage due to SHIGA TOXIN; FACTOR H deficiency; and aberrant VON WILLEBRAND FACTOR formation.

Top Publications

  1. Li A, Bendapudi P, Uhl L, Hamdan A, Kaufman R, Makar R. Clinical features and outcomes in patients with thrombotic microangiopathy not associated with severe ADAMTS13 deficiency. Transfusion. 2017;57:2151-2158 pubmed publisher
    ..The ADAMTS13 activity level in this group is not an independent predictor of poor outcomes but instead appears to be a marker of disease acuity. ..
  2. Gordon C, Chitalia V, Sloan J, Salant D, Coleman D, Quillen K, et al. Thrombotic Microangiopathy: A Multidisciplinary Team Approach. Am J Kidney Dis. 2017;70:715-721 pubmed publisher
    ..Facilitated by an Affinity Research Collaborative mechanism, we describe an interdisciplinary team dedicated to improving both clinical care and translational research in TMA. ..
  3. Hunt D, Kavanagh D, Drummond I, Weller B, Bellamy C, Overell J, et al. Thrombotic microangiopathy associated with interferon beta. N Engl J Med. 2014;370:1270-1 pubmed publisher
  4. Yamada Y, Abe R, Okano Y, Miyakawa Y. Long-term Eculizumab Treatment Contributes to Recovery from End-stage Renal Disease Caused by Atypical Hemolytic Uremic Syndrome. Intern Med. 2017;56:1085-1088 pubmed publisher
    ..Plasma exchange was ineffective; therefore, we initiated eculizumab after we excluded other thrombotic microangiopathies. Although long-term peritoneal dialysis was required, we successfully discontinued dialysis 18 months ..
  5. Chen Y, Ooi M, Lim S, Lin A, Lee J, Nagarajan C, et al. Thrombotic microangiopathy during carfilzomib use: case series in Singapore. Blood Cancer J. 2016;6:e450 pubmed publisher
  6. Chan K, Filshie R, Nandurkar H, Quach H. Thrombotic microangiopathy complicating bortezomib-based therapy for multiple myeloma. Leuk Lymphoma. 2015;56:2185-6 pubmed publisher
  7. Ameda S, Kuroda H, Yamada M, Sato K, Miura S, Sakano H, et al. Thrombotic microangiopathy due to malignant hypertension complicated with late-onset bleeding after renal biopsy. Rinsho Ketsueki. 2017;58:637-642 pubmed publisher
    ..Renal failure did not improve, and continuous hemodiafiltration was needed. This procedure stabilized blood pressure and improved the TMA...
  8. Gloude N, Khandelwal P, Luebbering N, Lounder D, Jodele S, Alder M, et al. Circulating dsDNA, endothelial injury, and complement activation in thrombotic microangiopathy and GVHD. Blood. 2017;130:1259-1266 pubmed publisher
    ..NETs may serve as a mechanistic link between endothelial injury and complement activation. NET formation may be one mechanism contributing to the clinical overlap between GVHD and TA-TMA. ..
  9. Page E, Little D, Vesely S, George J. Quinine-Induced Thrombotic Microangiopathy: A Report of 19 Patients. Am J Kidney Dis. 2017;70:686-695 pubmed publisher
    ..8 patients died. Patients for whom plasma exchange was not requested were not identified. Quinine-induced TMA causes severe acute kidney injury that commonly results in chronic kidney disease. ..

More Information


  1. Warren M, Jodele S, Dandoy C, Myers K, Wallace G, Nelson A, et al. A Complete Histologic Approach to Gastrointestinal Biopsy From Hematopoietic Stem Cell Transplant Patients With Evidence of Transplant-Associated Gastrointestinal Thrombotic Microangiopathy. Arch Pathol Lab Med. 2017;141:1558-1566 pubmed publisher
    ..Identification of features consistent with iTMA has immediate implications for clinical management that could potentially improve outcome and survival. ..
  2. Lu S, Liu S, Wietelmann A, Kojonazarov B, Atzberger A, Tang C, et al. Developmental vascular remodeling defects and postnatal kidney failure in mice lacking Gpr116 (Adgrf5) and Eltd1 (Adgrl4). PLoS ONE. 2017;12:e0183166 pubmed publisher
  3. Hattahara K, Hamada A, Oyama R, Masui K, Shichiri Y. [A Case of Pulmonary Tumor Thrombotic Microangiopathy in Castration-Resistant Prostate Cancer]. Hinyokika Kiyo. 2017;63:275-278 pubmed publisher
    ..The results of an autopsy indicated tumor emboli and stenosis of small pulmonary arteries with the fibrocellular intimal thickening, and therefore our final diagnosis was pulmonary tumor thrombotic microangiopathy. ..
  4. Palla R, Valsecchi C, Bajetta M, Spreafico M, De Cristofaro R, Peyvandi F. Evaluation of assay methods to measure plasma ADAMTS13 activity in thrombotic microangiopathies. Thromb Haemost. 2011;105:381-5 pubmed publisher
  5. Shih H, Lin C, Shiao Y. Pulmonary tumor thrombotic microangiopathy. Am J Emerg Med. 2011;29:241.e3-4 pubmed publisher
  6. Qi J, Wang J, Chen J, Su J, Tang Y, Wu X, et al. Plasma levels of complement activation fragments C3b and sC5b-9 significantly increased in patients with thrombotic microangiopathy after allogeneic stem cell transplantation. Ann Hematol. 2017;96:1849-1855 pubmed publisher
    ..ADAMTS13 activity is reduced in VOD, but the ADAMTS13/vWF axis appears to be unaffected in patients with TA-TMA. ..
  7. Horváth O, Prohaszka Z, Kállay K, Kassa C, Stréhn A, Csordás K, et al. [Changes in diagnostic criteria of thrombotic microangiopathy after stem cell transplantation]. Orv Hetil. 2017;158:1043-1050 pubmed publisher
    ..Orv Hetil. 2017; 158(27): 1043-1050. ..
  8. Wayne A, Shah N, Bhojwani D, Silverman L, Whitlock J, Stetler Stevenson M, et al. Phase 1 study of the anti-CD22 immunotoxin moxetumomab pasudotox for childhood acute lymphoblastic leukemia. Blood. 2017;130:1620-1627 pubmed publisher
    ..Moxetumomab pasudotox showed a manageable safety profile and evidence of activity in relapsed or refractory childhood ALL. This trial was registered at as #NCT00659425...
  9. Vaisbich M, Braga A, Gabrielle M, Bueno C, Piazzon F, Kok F. Thrombotic microangiopathy caused by methionine synthase deficiency: diagnosis and treatment pitfalls. Pediatr Nephrol. 2017;32:1089-1092 pubmed publisher
    ..We report the second case of MSD associated to TMA previously diagnosed as aHUS in which the patient had a poor response to eculizumab. ..
  10. Fukada I, Araki K, Minatsuki S, Fujino T, Hatano M, Numakura S, et al. Imatinib alleviated pulmonary hypertension caused by pulmonary tumor thrombotic microangiopathy in a patient with metastatic breast cancer. Clin Breast Cancer. 2015;15:e167-70 pubmed publisher
  11. George J, Nester C. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371:1847-8 pubmed publisher
  12. Alvarado A, Brodsky S, Nadasdy T, Singh N. Hemolytic uremic syndrome associated with Clostridium difficile infection. Clin Nephrol. 2014;81:302-6 pubmed publisher
    ..This case series suggest that Clostridium difficile infection may cause renal failure due to thrombotic microangiopathy (TMA) and should be considered in the differential diagnosis of diarrhea-associated HUS. ..
  13. Song D, Wu L, Wang F, Yang X, Zhu D, Chen M, et al. The spectrum of renal thrombotic microangiopathy in lupus nephritis. Arthritis Res Ther. 2013;15:R12 pubmed publisher
    ..007). There were various causes of renal TMA in lupus nephritis. Complement over-activation via both classical and alternative pathways might play an important role in the pathogenesis of renal TMA in lupus nephritis. ..
  14. Scully M, Hunt B, Benjamin S, Liesner R, Rose P, Peyvandi F, et al. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012;158:323-35 pubmed publisher
  15. Robier C, Neubauer M, Beham Schmid C, Sill H. Thrombotic microangiopathy and disseminated intravascular coagulation associated with carcinocythemia in a patient with breast cancer. J Clin Oncol. 2011;29:e825-6 pubmed publisher
  16. Cutolo M, Sulli A, Smith V. Evaluating microangiopathy in systemic sclerosis: what have we learnt and what is left to discover?. Expert Rev Clin Immunol. 2011;7:395-7 pubmed publisher
  17. Schweizer H, Boehm J, Winterer J, Wild D, Neumann H, Wiech T, et al. Phaeochromocytoma and thrombotic microangiopathy: favourable outcome despite advanced renal failure. J Clin Pathol. 2010;63:754-6 pubmed publisher
  18. Kissling S, Legallais C, Pruijm M, Teta D, Vogt B, Burnier M, et al. A new prescription model for regional citrate anticoagulation in therapeutic plasma exchanges. BMC Nephrol. 2017;18:81 pubmed publisher
    ..The immediately obtained regional anticoagulation protects against both the risk of coagulation of the membrane and the exposure to an excess of citrate. ..
  19. Mutneja A, Cossey L, Liapis H, CHEN Y. A rare case of renal thrombotic microangiopathy associated with Castleman's disease. BMC Nephrol. 2017;18:57 pubmed publisher
    ..IL-6 and VEGF are postulated to suppress glomerular VEGF expression, thereby causing renal TMA. Therapy directed against these inflammatory mediators may have important therapeutic implications. ..
  20. Okubo M, Shoda H, Bannai E, Kubo K, Kanda H, Fujio K, et al. Systemic lupus erythematosus with ADAMTS13 inhibitor-negative thrombotic microangiopathy treated with combination of mycophenolate mofetil, plasma exchange and steroid. Lupus. 2017;26:334-336 pubmed publisher
  21. Tomita M, Ochiai M, Shu S, Yamauchi Y, Shihara H, Ogata A, et al. [A case of thrombotic microangiopathy with glomerular subendothelial IgA deposition due to bevacizumab]. Nihon Jinzo Gakkai Shi. 2014;56:612-7 pubmed
    ..TMA was recently added to a section of "significant adverse effects" in the package insert of bevacizumab. Nephrologists should be fully aware of this drug-induced nephropathy. ..
  22. Thurman J, FRAZER ABEL A, Holers V. The Evolving Landscape for Complement Therapeutics in Rheumatic and Autoimmune Diseases. Arthritis Rheumatol. 2017;69:2102-2113 pubmed publisher
    ..Mechanisms of complement activation in rheumatoid arthritis, lupus, and thrombotic microangiopathies are also illustrated...
  23. Nakamura H, Kato M, Nakaya T, Kono M, Tanimura S, Sato T, et al. Decreased haptoglobin levels inversely correlated with pulmonary artery pressure in patients with pulmonary arterial hypertension: A cross-sectional study. Medicine (Baltimore). 2017;96:e8349 pubmed publisher
    ..Serum haptoglobin levels decreased in PAH patients and inversely correlated with pulmonary artery pressure in CTD-PAH patients, suggesting their potential as a surrogate marker for CTD-PAH...
  24. Chao Y, Chang Y, Wu H, Peng C, Weng T, Wu K. Everolimus for pediatric patients with acute graft-versus-host disease after hematopoietic stem cell transplantation: A pilot study. Medicine (Baltimore). 2017;96:e8464 pubmed publisher
    ..9% with a median follow-up of 3.4 years after HSCT (range 0.7-5.7). Accordingly, everolimus with steroids were feasible for patients with aGVHD after HSCT as primary treatment. Further large-scale studies are required...