Singer M, Deutschman CS, Seymour CW, Shankar-Hari M, Annane D, Bauer M, Bellomo R, Bernard GR, Chiche JD, Coopersmith CM, Hotchkiss RS, Levy MM, Marshall JC, Martin GS, Opal SM, Rubenfeld GD, van der Poll T, Vincent JL, Angus DC. The Third International Consensus Definitions for Sepsis and Septic Shock (Sepsis-3). JAMA. 2016;23(315):801–10.
Article CAS Google Scholar
Howell MD, Davis AM. Management of sepsis and septic shock. JAMA. 2017;317:847–8.
Article PubMed Google Scholar
Thiery-Antier N, Binquet C, Vinault S, Meziani F, Boisramé-Helms J, Quenot JP. Is thrombocytopenia an early prognostic marker in septic shock? Crit Care Med. 2016;44:764–72.
PubMed Google Scholar
Claushuis TA, van Vught LA, Scicluna BP, Wiewel MA, Klein Klouwenberg PM, Hoogendijk AJ, Ong DS, Cremer OL, Horn J, Franitza M, Toliat MR, Nürnberg P, Zwinderman AH, Bonten MJ, Schultz MJ, van der Poll T. Thrombocytopenia is associated with a dysregulated host response in critically ill sepsispatients. Blood. 2016;127:3062–72.
Article CAS PubMed Google Scholar
de Stoppelaar SF, van’t Veer C, van der Poll T. The role of platelets in sepsis. Thromb Haemost. 2014;112:666–77.
Article PubMed Google Scholar
Iba T, Levy JH. Inflammation and thrombosis: roles of neutrophils, platelets and endothelial cells and their interactions in thrombus formation during sepsis. J Thromb Haemost. 2018;16:231–41.
Article CAS PubMed Google Scholar
Bermejo-Martin JF, Martín-Fernandez M, López-Mestanza C, Duque P, Almansa R. Shared features of endothelial dysfunction between sepsis and its preceding risk factors. J Clin Med. 2018;7:400.
Article PubMed Central Google Scholar
Nishida O, Ogura H, Egi M, Fujishima S, Hayashi Y, Iba T, Imaizumi H, Inoue S, Kakihana Y, Kotani J, Kushimoto S, Masuda Y, Matsuda N, Matsushima A, Nakada TA, Nakagawa S, Nunomiya S, Sadahiro T, Shime N, Yatabe T, Hara Y, Hayashida K, Kondo Y, Sumi Y, Yasuda H, Aoyama K, Azuhata T, Doi K, Doi M, Fujimura N, Fuke R, Fukuda T, Goto K, Hasegawa R, Hashimoto S, Hatakeyama J, Hayakawa M, Hifumi T, Higashibeppu N, Hirai K, Hirose T, Ide K, Kaizuka Y, Kan'o T, Kawasaki T, Kuroda H, Matsuda A, Matsumoto S, Nagae M, Onodera M, Ohnuma T, Oshima K, Saito N, Sakamoto S, Sakuraya M, Sasano M, Sato N, Sawamura A, Shimizu K, Shirai K, Takei T, Takeuchi M, Takimoto K, Taniguchi T, Tatsumi H, Tsuruta R, Yama N, Yamakawa K, Yamashita C, Yamashita K, Yoshida T, Tanaka H, Oda S. The Japanese Clinical Practice Guidelines for Management of Sepsis and Septic Shock 2016 (J-SSCG 2016). Acute Med Surg. 2018;5:3–89.
Article PubMed PubMed Central Google Scholar
Iba T, Levy JH, Wada H, Thachil J, Warkentin TE, Levi M. Differential diagnoses for sepsis-induced disseminated intravascular coagulation. J Thromb Haemost. 2018. https://doi.org/10.1111/jth.14354.
Nguyen TC, Cruz MA, Carcillo JA. Thrombocytopenia-associated multiple organ failure and acute kidney injury. Crit Care Clin. 2015;31:661–74.
Article PubMed PubMed Central Google Scholar
George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371:654–66.
Article CAS PubMed Google Scholar
Ruggenenti P, Noris M, Remuzzi G. Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. Kidney Int. 2001;60:831–46.
Article CAS PubMed Google Scholar
Taylor FB Jr, Toh CH, Hoots WK, Wada H, Levi M. Towards definition, clinical and laboratory criteria, and a scoring system for disseminated intravascular coagulation. Thromb Haemost. 2001;86:1327–30.
Article CAS PubMed Google Scholar
Gando S, Iba T, Eguchi Y, Ohtomo Y, Okamoto K, Koseki K, Mayumi T, Murata A, Ikeda T, Ishikura H, Ueyama M, Ogura H, Kushimoto S, Saitoh D, Endo S, Shimazaki S. A multicenter, prospective validation of disseminated intravascular coagulation diagnostic criteria for critically ill patients: comparing current criteria. Crit Care Med. 2006;34:625–31.
Article PubMed Google Scholar
Vincent JL, Castro P, Hunt BJ, Jörres A, Praga M, Rojas-Suarez J, Watanabe E. Thrombocytopenia in the ICU: disseminated intravascular coagulation and thrombotic microangiopathies-what intensivists need to know. Crit Care. 2018;22:158.
Article PubMed PubMed Central Google Scholar
Semeraro N, Ammollo CT, Semeraro F, Colucci M. Sepsis, thrombosis and organ dysfunction. Thromb Res. 2012;129:290–5.
Article CAS PubMed Google Scholar
Liaw PC, Ito T, Iba T, Thachil J, Zeerleder S. DAMP and DIC: The role of extracellular DNA and DNA-binding proteins in the pathogenesis of DIC. Blood Rev. 2016;30:257–61.
Article CAS PubMed Google Scholar
Gando S, Saitoh D, Ogura H, Mayumi T, Koseki K, Ikeda T, Ishikura H, Iba T, Ueyama M, Eguchi Y, Ohtomo Y, Okamoto K, Kushimoto S, Endo S, Shimazaki S. Natural history of disseminated intravascular coagulation diagnosed based on the newly established diagnostic criteria for critically ill patients: results of a multicenter, prospective survey. Crit Care Med. 2008;36:145–50.
Article PubMed Google Scholar
Semeraro N, Ammollo CT, Semeraro F, Colucci M. Coagulopathy of acute Sepsis. Semin Thromb Hemost. 2015;41:650–8.
Article CAS PubMed Google Scholar
Engelmann B, Massberg S. Thrombosis as an intravascular effector of innate immunity. Nat Rev Immunol. 2013;13:34–45.
Article CAS PubMed Google Scholar
Corrigan JJ Jr, Ray WL, May N. Changes in the blood coagulation system associated with septicemia. N Engl J Med. 1968;279:851–6.
Article PubMed Google Scholar
Østerud B, Bjørklid E. The tissue factor pathway in disseminated intravascular coagulation. Semin Thromb Hemost. 2001;27:605–17.
Article PubMed Google Scholar
Nieman MT. Protease-activated receptors in hemostasis. Blood. 2016;128:169–77.
Article CAS PubMed PubMed Central Google Scholar
Ma R, Xie R, Yu C, Si Y, Wu X, Zhao L, Yao Z, Fang S, Chen H, Novakovic V, Gao C, Kou J, Bi Y, Thatte HS, Yu B, Yang S, Zhou J, Shi J. Phosphatidylserine-mediated platelet clearance by endothelium decreases platelet aggregates and procoagulant activity in sepsis. Sci Rep. 2017;7:4978.
Article CAS PubMed PubMed Central Google Scholar
Levi M, van der Poll T. Coagulation and sepsis. Thromb Res. 2017;149:38–44.
Article CAS PubMed Google Scholar
Moake JL. Thrombotic microangiopathies. N Engl J Med. 2002;347:589–600.
Article CAS PubMed Google Scholar
Scully M, Hunt BJ, Benjamin S, Liesner R, Rose P, Peyvandi F, Cheung B, Machin SJ. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012;158:323–35.
Article PubMed Google Scholar
Wada H, Matsumoto T, Suzuki K, Imai H, Katayama N, Iba T, Matsumoto M. Differences and similarities between disseminated intravascular coagulation and thrombotic microangiopathy. Thromb J. 2018;16:14.
Article PubMed PubMed Central Google Scholar
Thomas W, Cutler JA, Moore GW, McDonald V, Hunt BJ. The utility of a fast turnaround ADAMTS13 activity in the diagnosis and exclusion of thrombotic thrombocytopenic purpura. Br J Haematol. 2018. https://doi.org/10.1111/bjh.15219.
Levi M, Scully M, Singer M. The role of ADAMTS-13 in the coagulopathy of sepsis. J Thromb Haemost. 2018;16:646–51.
Article CAS PubMed Google Scholar
Groot E, Fijnheer R, Sebastian SA, de Groot PG, Lenting PJ. The active conformation of von Willebrand factor in patients with thrombotic thrombocytopenic purpura in remission. J Thromb Haemost. 2009;7:962–9.
Wada H, Matsumoto T, Hatada T. Diagnostic criteria and laboratory tests for disseminated intravascular coagulation. Expert Rev Hematol. 2012;5:643–52.
Article CAS PubMed Google Scholar
Wada H, Matsumoto T, Yamashita Y. Natural history of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Semin Thromb Hemost. 2014;40:866–73.
Article PubMed Google Scholar
Iba T, Gando S, Thachil J. Anticoagulant therapy for sepsis-associated disseminated intravascular coagulation: the view from Japan. J Thromb Haemost. 2014;12:1010–9.
Article CAS PubMed Google Scholar
Peyvandi F, Scully M, Kremer Hovinga JA, Cataland S, Knöbl P, Wu H, Artoni A, Westwood JP, Mansouri Taleghani M, Jilma B, Callewaert F, Ulrichts H, Duby C, Tersago D. Caplacizumab for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2016;374:511–22.
Article CAS PubMed Google Scholar
Scully M, Cataland SR, Peyvandi F, Coppo P, Knöbl P, Kremer Hovinga JA, Metjian A, de la Rubia J, Pavenski K, Callewaert F, Biswas D, De Winter H, Zeldin RK. Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2019;380:335–46.
Article CAS PubMed Google Scholar
Tersteeg C, Schiviz A, De Meyer SF, Plaimauer B, Scheiflinger F, Rottensteiner H, Vanhoorelbeke K. Potential for recombinant ADAMTS13 as an effective therapy for acquired thrombotic thrombocytopenic purpura. Arterioscler Thromb Vasc Biol. 2015;35:2336–42.
Article CAS PubMed Google Scholar
Talarico V, Aloe M, Monzani A, Miniero R, Bona G. Hemolytic uremic syndrome in children. Minerva Pediatr. 2016;68:441–55.
PubMed Google Scholar
Karmali MA. Factors in the emergence of serious human infections associated with highly pathogenic strains of shiga toxin-producing Escherichia coli. Int J Med Microbiol. 2018;308:1067–72.
Article CAS PubMed Google Scholar
Ingelbeen B, Bruyand M, Mariani-Kurkjian P, Le Hello S, Danis K, Sommen C, Bonacorsi S, de Valk H. Emerging Shiga-toxin-producing Escherichia coli serogroup O80 associated hemolytic and uremic syndrome in France, 2013-2016: differences with other serogroups. PLoS One. 2018;13:e0207492.
Article CAS PubMed PubMed Central Google Scholar
Liu F, Huang J, Sadler JE. Shiga toxin (Stx)1B and Stx2B induce von Willebrand factor secretion from human umbilical vein endothelial cells through different signaling pathways. Blood. 2011;118:3392–8.
Article CAS PubMed PubMed Central Google Scholar
Noris M, Mescia F, Remuzzi G. STEC-HUS, atypical HUS and TTP are all diseases of complement activation. Nat Rev Nephrol. 2012;8:622–33.
Article CAS PubMed Google Scholar
Kielstein JT, Beutel G, Fleig S, Steinhoff J, Meyer TN, Hafer C, Kuhlmann U, Bramstedt J, Panzer U, Vischedyk M, Busch V, Ries W, Mitzner S, Mees S, Stracke S, Nürnberger J, Gerke P, Wiesner M, Sucke B, Abu-Tair M, Kribben A, Klause N, Schindler R, Merkel F, Schnatter S, Dorresteijn EM, Samuelsson O, Brunkhorst R. Best supportive care and therapeutic plasma exchange with or without eculizumab in Shiga-toxin-producing E. coli O104:H4 induced haemolytic-uraemic syndrome: an analysis of the German STEC-HUS registry. Nephrol Dial Transplant. 2012;27:3807–15.
Article CAS PubMed Google Scholar
Jokiranta TS. HUS and atypical HUS. Blood. 2017;129:2847–56.
Article CAS PubMed PubMed Central Google Scholar
Spinale JM, Ruebner RL, Kaplan BS, Copelovitch L. Update on Streptococcus pneumoniae associated hemolytic uremic syndrome. Curr Opin Pediatr. 2013;25:203–8.
Article CAS PubMed Google Scholar
Meinel C, Spartà G, Dahse HM, Hörhold F, König R, Westermann M, Coldewey SM, Cseresnyés Z, Figge MT, Hammerschmidt S, Skerka C, Zipfel PF. Streptococcus pneumoniae from patients with hemolytic uremic syndrome binds human plasminogen via the surface protein PspC and uses plasmin to damage human endothelial cells. J Infect Dis. 2018;217:358–70.
Article CAS PubMed Google Scholar
Azoulay E, Knoebl P, Garnacho-Montero J, Rusinova K, Galstian G, Eggimann P, Abroug F, Benoit D, von Bergwelt-Baildon M, Wendon J, Scully M. Expert statements on the standard of care in critically ill adult patients with atypical hemolytic uremic syndrome. Chest. 2017;152:424–34.
Article PubMed Google Scholar
Nester CM, Thomas CP. Atypical hemolytic uremic syndrome: what is it, how is it diagnosed, and how is it treated? Hematology Am Soc Hematol Educ Program. 2012;2012:617–25.
PubMed Google Scholar
Noris M, Caprioli J, Bresin E, et al. Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype. Clin J Am Soc Nephrol. 2010;5:1844–59.
Article CAS PubMed PubMed Central Google Scholar
Scully M, Goodship T. How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome. Br J Haematol. 2014;164:759–66.
Article PubMed PubMed Central Google Scholar
Fujisawa M, Kato H, Yoshida Y, Usui T, Takata M, Fujimoto M, Wada H, Uchida Y, Kokame K, Matsumoto M, Fujimura Y, Miyata T, Nangaku M. Clinical characteristics and genetic backgrounds of Japanese patients with atypical hemolyticuremic syndrome. Clin Exp Nephrol. 2018;22:1088–99.
Article CAS PubMed PubMed Central Google Scholar
Legendre CM, Licht C, Muus P, Greenbaum LA, Babu S, Bedrosian C, Bingham C, Cohen DJ, Delmas Y, Douglas K, Eitner F, Feldkamp T, Fouque D, Furman RR, Gaber O, Herthelius M, Hourmant M, Karpman D, Lebranchu Y, Mariat C, et al. Terminal complement inhibitor eculizumab in atypical hemolytic- uremic syndrome. N Engl J Med. 2013;368:2169–81.
Article CAS PubMed Google Scholar
Larsen CP, Wilson JD, Best-Rocha A, Beggs ML, Hennigar RA. Genetic testing of complement and coagulation pathways in patients with severe hypertension and renal microangiopathy. Mod Pathol. 2018;31:488–94.
Article CAS PubMed Google Scholar
Cines DB, Levine LD. Thrombocytopenia in pregnancy. Hematology Am Soc Hematol Educ Program. 2017;2017:144–51.
Article PubMed PubMed Central Google Scholar
Thomas MR, Robinson S, Scully MA. How we manage thrombotic microangiopathies in pregnancy. Br J Haematol. 2016;173:821–30.
Article PubMed Google Scholar
Erez O. Disseminated intravascular coagulation in pregnancy-clinical phenotypes and diagnostic scores. Thromb Res. 2017;151:S56–60.
Article CAS PubMed Google Scholar
Abildgaard U, Heimdal K. Pathogenesis of the syndrome of hemolysis, elevated liver enzymes, and low platelet count (HELLP): a review. Eur J Obstet Gynecol Reprod Biol. 2013;166:117–23.
Article CAS PubMed Google Scholar
Hulstein JJ, van Runnard Heimel PJ, Franx A, Lenting PJ, Bruinse HW, Silence K, de Groot PG, Fijnheer R. Acute activation of the endothelium results in increased levels of active von Willebrand factor in hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome. J Thromb Haemost. 2006;4:2569–75.
Article CAS PubMed Google Scholar
Haram K, Mortensen JH, Mastrolia SA, Erez O. Disseminated intravascular coagulation in the HELLP syndrome: how much do we really know? J Matern Fetal Neonatal Med. 2017;30:779–88.
Article CAS PubMed Google Scholar
Lamprecht A, Morton A, Laurie J, Lee W. Acute fatty liver of pregnancy and concomitant medical conditions: a review of cases at a quaternary obstetric hospital. Obstet Med. 2018;11:178–81.
Article PubMed PubMed Central Google Scholar
Wu Z, Huang P, Gong Y, Wan J, Zou W. Treating acute fatty liver of pregnancy with artificial liver support therapy: Systematic review. Medicine. 2018;97:e12473.
Article PubMed PubMed Central Google Scholar
de Holanda MI, Pôrto LC, Wagner T, Christiani LF, Palma LMP. Use of eculizumab in a systemic lupus erythemathosus patient presenting thrombotic microangiopathy and heterozygous deletion in CFHR1-CFHR3. A case report and systematic review. Clin Rheumatol. 2017;36:2859–67.
Article PubMed Google Scholar
Song D, Wu LH, Wang FM, Yang XW, Zhu D, Chen M, Yu F, Liu G, Zhao MH. The spectrum of renal thrombotic microangiopathy in lupus nephritis. Arthritis Res Ther. 2013;15:R12.
Article CAS PubMed PubMed Central Google Scholar
Sun F, Wang X, Wu W, Wang K, Chen Z, Li T, Ye S. TMA secondary to SLE: rituximab improves overall but not renal survival. Clin Rheumatol. 2018;37:213–8.
Article CAS PubMed Google Scholar
Sciascia S, Radin M, Yazdany J, Tektonidou M, Cecchi I, Roccatello D, Dall'Era M. Expanding the therapeutic options for renal involvement in lupus: eculizumab, available evidence. Rheumatol Int. 2017;37:1249–55.
Article CAS PubMed Google Scholar
Groot N, de Graeff N, Avcin T, Bader-Meunier B, Dolezalova P, Feldman B, Kenet G, Koné-Paut I, Lahdenne P, Marks SD, McCann L, Pilkington CA, Ravelli A, van Royen-Kerkhof A, Uziel Y, Vastert SJ, Wulffraat NM, Ozen S, Brogan P, Kamphuis S, Beresford MW. European evidence-based recommendations for diagnosis and treatment of paediatric antiphospholipid syndrome: the SHARE initiative. Ann Rheum Dis. 2017;76:1637–41.
Article CAS PubMed Google Scholar
Garcia D, Erkan D. Diagnosis and management of the antiphospholipid syndrome. N Engl J Med. 2018;378:2010–21.
Article CAS PubMed Google Scholar
Hoxha A, Mattia E, Tonello M, Grava C, Pengo V, Ruffatti A. Antiphosphatidylserine/prothrombin antibodies as biomarkers to identify severe primary antiphospholipid syndrome. Clin Chem Lab Med. 2017;55:890–8.
Article CAS PubMed Google Scholar
Sciascia S, Sanna G, Murru V, Roccatello D, Khamashta MA, Bertolaccini ML. Anti-prothrombin (aPT) and anti-phosphatidylserine/prothrombin (aPS/PT) antibodies and the risk of thrombosis in the antiphospholipid syndrome. A systematic review. Thromb Haemost. 2014;111:354–64.
Article CAS PubMed Google Scholar
Espinosa G, Rodríguez-Pintó I, Cervera R. Catastrophic antiphospholipid syndrome: an update. Panminerva Med. 2017;59:254–68.
PubMed Google Scholar
Legault K, Schunemann H, Hillis C, Yeung C, Akl EA, Carrier M, Cervera R, Crowther M, Dentali F, Erkan D, Espinosa G, Khamashta M, Meerpohl JJ, Moffat K, O'Brien S, Pengo V, Rand JH, Rodriguez Pinto I, Thom L, Iorio A. McMaster RARE-Bestpractices clinical practice guideline on diagnosis and management of the catastrophic antiphospholipid syndrome. J Thromb Haemost. 2018. https://doi.org/10.1111/jth.14192.
Zeisbrich M, Becker N, Benner A, Radujkovic A, Schmitt K, Beimler J, Ho AD, Zeier M, Dreger P, Luft T. Transplant-associated thrombotic microangiopathy is an endothelial complication associated with refractoriness of acute GvHD. Bone Marrow Transplant. 2017;52:1399–405.
Article CAS PubMed Google Scholar
Gavriilaki E, Sakellari I, Anagnostopoulos A, Brodsky RA. Transplant-associated thrombotic microangiopathy: opening Pandora's box. Bone Marrow Transplant. 2017;52:1355–60.
Article CAS PubMed Google Scholar
Morton JM, George JN. Microangiopathic hemolytic anemia and thrombocytopenia in patients with cancer. J Oncol Pract. 2016;12:523–30.
Article PubMed Google Scholar
Izzedine H, Perazella MA. Thrombotic microangiopathy, cancer, and cancer drugs. Am J Kidney Dis. 2015;66:857–68.
Article CAS PubMed Google Scholar
Kheder El-Fekih R, Deltombe C, Izzedine H. Thrombotic microangiopathy and cancer. Nephrol Ther. 2017;13:439–47.
Article PubMed Google Scholar
Eremina V, Jefferson JA, Kowalewska J, Hochster H, Haas M, Weisstuch J, Richardson C, Kopp JB, Kabir MG, Backx PH, Gerber HP, Ferrara N, Barisoni L, Alpers CE, Quaggin SE. VEGF inhibition and renal thrombotic microangiopathy. N Engl J Med. 2008;358:1129–36.
Article CAS PubMed PubMed Central Google Scholar
Al-Nouri ZL, Reese JA, Terrell DR, Vesely SK, George JN. Drug-induced thrombotic microangiopathy: a systematic review of published reports. Blood. 2015;125:616–8.
Article CAS PubMed PubMed Central Google Scholar
Gottschall JL, Neahring B, McFarland JG, Wu GG, Weitekamp LA, Aster RH. Quinine-induced immune thrombocytopenia with hemolytic uremic syndrome: clinical and serological findings in nine patients and review of literature. Am J Hematol. 1994;47:283–9.
Kleinpell R, Aitken L, Schorr CA. Implications of the new international sepsis guidelines or nursing care. Am J Crit Care. 2013;22:212–22.
Article PubMed Google Scholar
Martel N, Lee J, Wells PS. Risk for heparin-induced thrombocytopenia with unfractionated and low-molecular-weight heparin thromboprophylaxis: a meta-analysis. Blood. 2005;106:2710–5.
Article CAS PubMed Google Scholar
Warkentin TE. Clinical picture of heparin-induced thrombocytopenia (HIT) and its differentiation from non-HIT thrombocytopenia. Thromb Haemost. 2016;116:813–22.
Article PubMed Google Scholar
Warkentin TE, Greinacher A, Gruel Y, Aster RH, Chong BH. Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. Laboratory testing for heparin-induced thrombocytopenia: a conceptual framework and implications for diagnosis. J Thromb Haemost. 2011;9:2498–500.
Article CAS PubMed Google Scholar
Poudel DR, Ghimire S, Dhital R, Forman D, Warkentin TE. Spontaneous HIT syndrome post-knee replacement surgery with delayed recovery of thrombocytopenia: a case report and literature review. Platelets. 2017;28:614–20.
Article CAS PubMed Google Scholar
Warkentin TE, Greinacher A. Management of heparin-induced thrombocytopenia. Curr Opin Hematol. 2016;23:462–70.
Warkentin TE. Ischemic limb gangrene with pulses. N Engl J Med. 2015;373:642–55.
Article CAS PubMed Google Scholar
Rodeghiero F, Stasi R, Gernsheimer T, Michel M, Provan D, Arnold DM, Bussel JB, Cines DB, Chong BH, Cooper N, Godeau B, Lechner K, Mazzucconi MG, McMillan R, Sanz MA, Imbach P, Blanchette V, Kühne T, Ruggeri M, George JN. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood. 2009;12(113):2386–93.
Article CAS Google Scholar
Liebman HA. Recognizing and treating secondary immune thrombocytopenic purpura associated with lymphoproliferative disorders. Semin Hematol. 2009;46:S33–6.
Article PubMed Google Scholar
Cines DB, Blanchette VS. Immune thrombocytopenic purpura. N Engl J Med. 2002;346:995–1008.
Article PubMed Google Scholar
Johnsen J. Pathogenesis in immune thrombocytopenia: new insights. Hematology Am Soc Hematol Educ Program. 2012;2012:306–12.
PubMed Google Scholar
Qu M, Liu Q, Zhao HG, Peng J, Ni H, Hou M, Jansen AJG. Low platelet count as risk factor for infections in patients with primary immune thrombocytopenia: a retrospective evaluation. Ann Hematol. 2018;97:1701–6.
Article CAS PubMed PubMed Central Google Scholar
Neunert CE, Cooper N. Evidence-based management of immune thrombocytopenia: ASH guideline update. Hematology Am Soc Hematol Educ Program. 2018;2018:568–75.
Article PubMed PubMed Central Google Scholar
Provan D, Stasi R, Newland AC, Blanchette VS, Bolton-Maggs P, Bussel JB, Chong BH, Cines DB, Gernsheimer TB, Godeau B, Grainger J, Greer I, Hunt BJ, Imbach PA, Lyons G, McMillan R, Rodeghiero F, Sanz MA, Tarantino M, Watson S, Young J, Kuter DJ. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood. 2010;115:168–86.
Article CAS PubMed Google Scholar
Ghanima W, Godeau B, Cines DB, Bussel JB. How I treat immune thrombocytopenia: the choice between splenectomy or a medical therapy as a second-line treatment. Blood. 2012;120:960–9.
Article CAS PubMed Google Scholar
Ramachandran S, Zaidi F, Aggarwal A, Gera R. Recent advances in diagnostic and therapeutic guidelines for primary and secondary hemophagocytic lymphohistiocytosis. Blood Cells Mol Dis. 2017;64:53–7.
Article PubMed Google Scholar
Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, Ladisch S, McClain K, Webb D, Winiarski J, Janka G. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48:124–31.
Article PubMed Google Scholar
Kleynberg RL, Schiller GJ. Secondary hemophagocytic lymphohistiocytosis in adults: an update on diagnosis and therapy. Clin Adv Hematol Oncol. 2012;10:726–32.
PubMed Google Scholar
Chalmers E. Purpura fulminans: recognition, diagnosis and management. Arch Dis Child. 2011;96:1066–71.
Article CAS PubMed Google Scholar
Colling ME, Bendapudi PK. Purpura fulminans: mechanism and management of dysregulated hemostasis. Transfus Med Rev. 2018;32:69–76.
Article PubMed Google Scholar
Bendapudi PK, Robbins A, LeBoeuf N, Pozdnyakova O, Bhatt A, Duke F, Sells R, McQuiston J, Humrighouse B, Rouaisnel B, Colling M, Stephenson KE, Saavedra A, Losman JA. Persistence of endothelial thrombomodulin in a patient with infectious purpura fulminans treated with protein C concentrate. Blood Adv. 2018;2(21):2917–21.
Article PubMed PubMed Central Google Scholar
Sakashita K, Murata K, Takamori M. TAFRO syndrome: current perspectives. J Blood Med. 2018;9:15–23.
Article PubMed PubMed Central Google Scholar
Kawabata H, Takai K, Kojima M, Nakamura N, Aoki S, Nakamura S, Kinoshita T, Masaki Y. Castleman-Kojima disease (TAFRO syndrome): a novel systemic inflammatory disease characterized by a constellation of symptoms, namely, thrombocytopenia, ascites (anasarca), microcytic anemia, myelofibrosis, renal dysfunction, and organomegaly : a status report and summary of Fukushima (6 June, 2012) and Nagoya meetings (22 September, 2012). J Clin Exp Hematop. 2013;53:57–61.
Article PubMed Google Scholar
Semra P. Tafro syndrome: critical review for clinicians and pathologists. Crit Rev Oncol Hematol. 2018;128:88–95.
Article Google Scholar
Louis C, Vijgen S, Samii K, Chalandon Y, Terriou L, Launay D, Fajgenbaum DC, Seebach JD, Muller YD. TAFRO syndrome in Caucasians: a case report and review of the literature. Front Med. 2017;4:149.
Article Google Scholar
Guo CT, Lu QB, Ding SJ, Hu CY, Hu JG, Wo Y, Fan YD, Wang XJ, Qin SL, Cui N, Yang ZD, Zhang XA, Liu W, Cao WC. Epidemiological and clinical characteristics of severe fever with thrombocytopeniasyndrome (SFTS) in China: an integrated data analysis. Epidemiol Infect. 2016;144:1345–54.
Article CAS PubMed Google Scholar
Oh WS, Yoo JR, Kwon KT, Kim HI, Lee SJ, Jun JB, Ryu SY, Kim HA, Hur J, Wi YM, Lim MH, Heo ST. Effect of early plasma exchange on survival in patients with severe fever with thrombocytopenia syndrome: a multicenter study. Yonsei Med J. 2017;58:867–71.
Article PubMed PubMed Central Google Scholar
Afdhal NH, Giannini EG, Tayyab G, Mohsin A, Lee JW, Andriulli A, Jeffers L, McHutchison J, Chen PJ, Han KH, Campbell F, Hyde D, Brainsky A, Theodore D. Eltrombopag before procedures in patients with cirrhosis and thrombocytopenia. N Engl J Med. 2012;367:716–24.
Article CAS PubMed Google Scholar
Loudin M, Ahn J. Portal vein thrombosis in cirrhosis. J Clin Gastroenterol. 2017;51:579–85.
Article PubMed Google Scholar
Page 2
Category
Disease
Cause
Clinical features
Treatment
DIC
Infection-induced expression of tissue factor and phosphatidylserine of the cellular membrane
Thrombotic phenotype of coagulation disorder with fibrinolysis suppression
Management of infectious focus, potentially anticoagulant therapy
TMA
TTP (acquired)
Autoantibody inhibition of ADAMTS13 activity
TTP pentad (thrombocytopenia, MAHA, fluctuating neurological signs, renal impairment and fever)
Plasma exchange, immunosuppressive therapy, recombinant ADAMTS13 if possible
STEC-HUS
Shiga toxin-producing Escherichia coli
Hemorrhagic enterocolitis, fever, thrombocytopenia, MAHA, acute kidney injury
Avoiding antibiotic therapy and supportive care
aHUS
Uncontrolled activity of alternative complement pathway.
Thrombocytopenia, MAHA, acute kidney injury
Plasma exchange, and anti-C5 monoclonal antibody (eculizumab)
Secondary TMA
HELLP syndrome
Inadequate placentation secondary to maternal immune response to invading trophoblast.
Hemolysis, elevated liver enzymes, and low platelets
Timely delivery
APS/CAPS (primary)
Antiphospholipid antibodies (β2-glycoprotein I)
Multiple venous and arterial thrombosis, repeated miscarriage, multi-organ failure (CAPS)