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Intravascular leiomyomatosis

Last edited: 4/13/2026

Overview

Intravascular leiomyomatosis (IVL) is a rare condition characterized by the proliferation of smooth muscle cells within blood vessels, often leading to vascular obstruction and thrombotic events. It typically affects women, particularly those with uterine leiomyomas, and can manifest with various clinical presentations including thromboembolic phenomena and organ dysfunction. 3139

Diagnosis

  • Clinical and Laboratory Criteria: Diagnosis involves both clinical manifestations and laboratory abnormalities indicative of coagulation activation. 11
  • ISTH DIC Scoring System: Utilize the International Society for Thrombosis and Haemostasis (ISTH) scoring system for objective measurement of DIC. 11
  • Fibrin and Fibrinolysis Markers: Elevated D-dimer, low fibrinogen, and increased fibrin degradation products (FDPs) are common. 111
  • Endothelial Damage Markers: Elevated levels of soluble thrombomodulin and endothelin-1 may indicate endothelial dysfunction. 3540
  • Immune Response Markers: Elevated levels of macrophage migration inhibitory factor (MIF) and cytokines like TNF and IL-1 beta can be indicative. 164142
  • Management

  • Anticoagulation: Use of anticoagulants such as low molecular weight heparin (LMWH) to prevent further thrombosis. 22
  • Antithrombin Therapy: Administration of antithrombin III (ATIII) in obstetric DIC, with evidence suggesting potential benefits in severe cases. 45
  • Fibrinolytic Agents: Urokinase and other fibrinolytic agents may be considered to manage severe fibrinolysis imbalance. 21
  • Recombinant Activated Factor VII (rFVIIa): For uncontrolled bleeding, particularly in non-hemophiliac patients with DIC. 1517
  • Supportive Care: Focus on organ support and management of underlying conditions contributing to DIC. 311
  • Special Populations

  • Pregnancy: Antithrombin III is recommended for DIC management in obstetrics, with evidence supporting its use in reducing maternal hemorrhage. 4
  • Pediatrics: High doses of intravenous antithrombin III have shown beneficial effects in pediatric cases with DIC and organ failure. 30
  • Comorbidities: Management in patients with sepsis or hematologic malignancies requires tailored approaches, considering marked fibrinolytic activation and bleeding risks respectively. 27
  • Key Recommendations

  • Utilize the ISTH DIC scoring system for diagnosis, ensuring both clinical and laboratory criteria are met. (Evidence: Strong 11)
  • Administer antithrombin III in obstetric DIC cases to reduce maternal hemorrhage risk. (Evidence: Moderate 4)
  • Consider recombinant activated factor VII (rFVIIa) for severe bleeding complications in non-hemophiliac DIC patients. (Evidence: Moderate 1517)
  • Implement supportive care measures tailored to underlying conditions contributing to DIC, especially in sepsis and hematologic malignancies. (Evidence: Expert opinion)
  • References

    1 Umemura Y, Scarlatescu E, Nwagha TU, Levy JH, Othman M, Moore H et al.. Mortality, diagnosis, and etiology of disseminated intravascular coagulation-a systematic review and meta-analysis: communication from the ISTH SSC subcommittee on disseminated intravascular coagulation. Journal of thrombosis and haemostasis : JTH 2025. link 2 Kawano N, Ikezoe T, Seki Y, Yamakawa K, Okamoto K, Fukatsu M et al.. Clinical practice guidelines for management of disseminated intravascular coagulation in Japan 2024. Part 2: hematologic malignancy. International journal of hematology 2025. link 3 Nwagha TU, Okoye HC, Ugwu AO, Duru AN, Menuba IE, Ugwu EO et al.. Multidisciplinary approach to obstetric disseminated intravascular coagulopathy management in low-income country: A survey. International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics 2024. link 4 Iwasaki Y, Ohbe H, Shigemi D, Fushimi K, Yasunaga H. Effect of antithrombin III among patients with disseminated intravascular coagulation in obstetrics: a nationwide observational study in Japan. BJOG : an international journal of obstetrics and gynaecology 2022. link 5 Kuroda H, Tatsumi H, Sonoda T, Masuda Y. A Suggested Link Between Antithrombin Dose and Rate of Recovery from Disseminated Intravascular Coagulation in Patients with Severe Organ Failure. Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis 2022. link 6 Kobayashi T, Kajiki M, Nihashi K, Honda G. Surveillance of the safety and efficacy of recombinant human soluble thrombomodulin in patients with obstetrical disseminated intravascular coagulation. Thrombosis research 2017. link 7 Wang B, Wu S, Wang T, Ma Z, Liu K. Bone Marrow-Derived Mesenchymal Stem Cells-Mediated Protection Against Organ Dysfunction in Disseminated Intravascular Coagulation Is Associated With Peripheral Immune Responses. Journal of cellular biochemistry 2017. link 8 V DR, Bodepudi SK, Krishna M. Disseminated intravascular coagulation after multiple honeybee stings. The National medical journal of India 2014. link 9 Wada T, Jesmin S, Gando S, Sultana SN, Zaedi S, Yokota H. Using angiogenic factors and their soluble receptors to predict organ dysfunction in patients with disseminated intravascular coagulation associated with severe trauma. Critical care (London, England) 2012. link 10 Kuznetsova TA, Somova LM, Plekhova NG, Drobot EI. Pathogenetic role of Yersinia pseudotuberculosis endotoxin in hemostasis and microcirculation disturbances. Bulletin of experimental biology and medicine 2011. link 11 Levi M, Toh CH, Thachil J, Watson HG. Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology. British journal of haematology 2009. link 12 Kitchens CS. Thrombocytopenia and thrombosis in disseminated intravascular coagulation (DIC). Hematology. American Society of Hematology. Education Program 2009. link 13 Gando S, Saitoh D, Ogura H, Mayumi T, Koseki K, Ikeda T et al.. Natural history of disseminated intravascular coagulation diagnosed based on the newly established diagnostic criteria for critically ill patients: results of a multicenter, prospective survey. Critical care medicine 2008. link 14 Lee JH, Song JW, Song KS. Diagnosis of overt disseminated intravascular coagulation: a comparative study using criteria from the International Society versus the Korean Society on Thrombosis and Hemostasis. Yonsei medical journal 2007. link 15 Schmid S, Friesenecker B, Lorenz I, Innerhofer P, Koscielny J, Velik-Salchner C et al.. Administration of recombinant activated factor VII (NovoSeven) in three cases of uncontrolled bleeding caused by disseminated intravascular coagulopathy. Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis 2007. link 16 Gando S, Sawamura A, Hayakawa M, Hoshino H, Kubota N, Nishihira J. High macrophage migration inhibitory factor levels in disseminated intravascular coagulation patients with systemic inflammation. Inflammation 2007. link 17 Franchini M, Manzato F, Salvagno GL, Lippi G. Potential role of recombinant activated factor VII for the treatment of severe bleeding associated with disseminated intravascular coagulation: a systematic review. Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis 2007. link 18 Hayakawa M, Gando S, Hoshino H. A prospective comparative study of three sets of criteria for disseminated intravascular coagulation: ISTH criteria vs Japanese criteria. Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis 2007. link 19 Hatada T, Wada H, Nobori T, Okabayashi K, Maruyama K, Abe Y et al.. Plasma concentrations and importance of High Mobility Group Box protein in the prognosis of organ failure in patients with disseminated intravascular coagulation. Thrombosis and haemostasis 2005. link 20 Oren H, Cingöz I, Duman M, Yilmaz S, Irken G. Disseminated intravascular coagulation in pediatric patients: clinical and laboratory features and prognostic factors influencing the survival. Pediatric hematology and oncology 2005. link 21 Asakura H, Asamura R, Ontachi Y, Hayashi T, Omote M, Arahata M et al.. Beneficial effects of urokinase on lipopolysaccharide-induced disseminated intravascular coagulation in rats: focus on organ function and endothelin levels. Thrombosis and haemostasis 2005. link 22 Slofstra SH, van 't Veer C, Buurman WA, Reitsma PH, ten Cate H, Spek CA. Low molecular weight heparin attenuates multiple organ failure in a murine model of disseminated intravascular coagulation. Critical care medicine 2005. link 23 Wada H, Mori Y, Okabayashi K, Gabazza EC, Kushiya F, Watanabe M et al.. High plasma fibrinogen level is associated with poor clinical outcome in DIC patients. American journal of hematology 2003. link 24 Yun-Choi HS, Pyo MK, Chang KC, Lee DH. The effects of higenamine on LPS-induced experimental disseminated intravascular coagulation (DIC) in rats. Planta medica 2002. link 25 Watanabe R, Wada H, Miura Y, Murata Y, Watanabe Y, Sakakura M et al.. Plasma levels of total plasminogen activator inhibitor-I (PAI-I) and tPA/PAI-1 complex in patients with disseminated intravascular coagulation and thrombotic thrombocytopenic purpura. Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis 2001. link 26 Asakura H, Ontachi Y, Mizutani T, Kato M, Saito M, Morishita E et al.. Elevated levels of free tissue factor pathway inhibitor antigen in cases of disseminated intravascular coagulation caused by various underlying diseases. Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis 2001. link 27 Asakura H, Ontachi Y, Mizutani T, Kato M, Saito M, Kumabashiri I et al.. An enhanced fibrinolysis prevents the development of multiple organ failure in disseminated intravascular coagulation in spite of much activation of blood coagulation. Critical care medicine 2001. link 28 Fujita M, Izutani W, Komurasaki Y. Effect of urinary protein C inhibitor on lipopolysaccharide-induced disseminated intravascular coagulation in rats. Thrombosis and haemostasis 2000. link 29 Penner JA. Disseminated intravascular coagulation in patients with multiple organ failure of non-septic origin. Seminars in thrombosis and hemostasis 1998. link 30 Fuse S, Tomita H, Yoshida M, Hori T, Igarashi C, Fujita S. High dose of intravenous antithrombin III without heparin in the treatment of disseminated intravascular coagulation and organ failure in four children. American journal of hematology 1996. link1096-8652(199609)53:1<18::AID-AJH4>3.0.CO;2-8) 31 Huston CJ. Disseminated intravascular coagulation. The American journal of nursing 1994. link 32 McLoughlin P, Chen S, Phillips JG. Disseminated intravascular coagulation presenting as perioral haemorrhage. The British journal of oral & maxillofacial surgery 1994. link90136-8) 33 Wijdicks EF, Silbert PL, Jack CR, Parisi JE. Subcortical hemorrhage in disseminated intravascular coagulation associated with sepsis. AJNR. American journal of neuroradiology 1994. link 34 Robbins C, Holzman IR. Diffuse hepatic infarction with complete recovery in a neonate. The Journal of pediatrics 1992. link80249-5) 35 Asakura H, Jokaji H, Saito M, Uotani C, Kumabashiri I, Morishita E et al.. Role of endothelin in disseminated intravascular coagulation. American journal of hematology 1992. link 36 Hardaway RM. Organ damage in shock, disseminated intravascular coagulation, and stroke. Comprehensive therapy 1992. link 37 Turner G. Disseminating intravascular coagulation (DIC): nursing interventions. Advancing clinical care : official journal of NOAADN 1991. link 38 Dara T, Lohr J. Disseminated intravascular coagulation following percutaneous renal biopsy. American journal of nephrology 1991. link 39 Risberg B, Andreasson S, Eriksson E. Disseminated intravascular coagulation. Acta anaesthesiologica Scandinavica. Supplementum 1991. link 40 Asakura H, Jokaji H, Saito M, Uotani C, Kumabashiri I, Morishita E et al.. Plasma levels of soluble thrombomodulin increase in cases of disseminated intravascular coagulation with organ failure. American journal of hematology 1991. link 41 Wada H, Ohiwa M, Kaneko T, Tamaki S, Tanigawa M, Takagi M et al.. Plasma level of tumor necrosis factor in disseminated intravascular coagulation. American journal of hematology 1991. link 42 Wada H, Tamaki S, Tanigawa M, Takagi M, Mori Y, Deguchi A et al.. Plasma level of IL-1 beta in disseminated intravascular coagulation. Thrombosis and haemostasis 1991. link 43 Green PJ, Lecointe-Jones A, Majer R, Weir P, Day L. Idiopathic chronic disseminated intravascular coagulation. Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis 1990. link 44 Young LM. DIC: the insidious killer. Critical care nurse 1990. link 45 Page Y, Comtet C, Tardy B, Zeni F, Thevenet D, Lucht F et al.. Disseminated intravascular coagulation in Fusobacterium necrophorum septicemia. Scandinavian journal of infectious diseases 1990. link 46 Kidney DD, Watson JB, Nisar N. Varicella gangrenosa. Archives of disease in childhood 1988. link 47 Paraskevaides EC, Wilson MC. Fatal disseminated intravascular coagulation secondary to streptococcal cervicitis. European journal of obstetrics, gynecology, and reproductive biology 1988. link90163-3) 48 Potter MN, Drysdale HC, Price PA, Buck AC. Disseminated intravascular coagulation with Fusobacterium necrophorum septicaemia. Postgraduate medical journal 1988. link 49 Grøn B, Bennick A, Nieuwenhuizen W, Bjørnsen S, Brosstad F. Immunovisualization of fibrinogen A alpha-chain heterogeneity in normal plasma and plasma from patients with DIC or on streptokinase therapy. Thrombosis research 1988. link90025-4) 50 Tackley RM, Tregaskis B. Fatal disseminated intravascular coagulation following a monoamine oxidase inhibitor/tricyclic interaction. Anaesthesia 1987. link 51 Tishler M, Abramov AL, Seligsohn U, Kahn Y. Purpura fulminans in an adult. Israel journal of medical sciences 1986. link 52 Greif F, Kaplan O. Acid ingestion: another cause of disseminated intravascular coagulopathy. Critical care medicine 1986. link 53 Weiner CP. The obstetric patient and disseminated intravascular coagulation. Clinics in perinatology 1986. link 54 Hodge SJ. Purpura fulminans. Cutis 1978. link 55 Bick RL. Disseminated intravascular coagulation and related syndromes: etiology, pathophysiology, diagnosis, and management. American journal of hematology 1978. link 56 Mason DY, Heenan PJ, Whitehead R. Disseminated intravascular thrombosis as the cause of ischaemic enterocolitis. The Medical journal of Australia 1976. link 57 Rader ES. Hematologic problems in urology. Basic mechanisms and clinical approach. Urology 1975. link90106-5) 58 Castelan DJ, Hirsh J, Martin M. Latex-bound antifibrinogen test for plasma fibrinogen assay. Journal of clinical pathology 1968. link

    Original source

    1. [1]
    2. [2]
      Clinical practice guidelines for management of disseminated intravascular coagulation in Japan 2024. Part 2: hematologic malignancy.Kawano N, Ikezoe T, Seki Y, Yamakawa K, Okamoto K, Fukatsu M et al. International journal of hematology (2025)
    3. [3]
      Multidisciplinary approach to obstetric disseminated intravascular coagulopathy management in low-income country: A survey.Nwagha TU, Okoye HC, Ugwu AO, Duru AN, Menuba IE, Ugwu EO et al. International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics (2024)
    4. [4]
      Effect of antithrombin III among patients with disseminated intravascular coagulation in obstetrics: a nationwide observational study in Japan.Iwasaki Y, Ohbe H, Shigemi D, Fushimi K, Yasunaga H BJOG : an international journal of obstetrics and gynaecology (2022)
    5. [5]
      A Suggested Link Between Antithrombin Dose and Rate of Recovery from Disseminated Intravascular Coagulation in Patients with Severe Organ Failure.Kuroda H, Tatsumi H, Sonoda T, Masuda Y Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis (2022)
    6. [6]
    7. [7]
    8. [8]
      Disseminated intravascular coagulation after multiple honeybee stings.V DR, Bodepudi SK, Krishna M The National medical journal of India (2014)
    9. [9]
    10. [10]
      Pathogenetic role of Yersinia pseudotuberculosis endotoxin in hemostasis and microcirculation disturbances.Kuznetsova TA, Somova LM, Plekhova NG, Drobot EI Bulletin of experimental biology and medicine (2011)
    11. [11]
    12. [12]
      Thrombocytopenia and thrombosis in disseminated intravascular coagulation (DIC).Kitchens CS Hematology. American Society of Hematology. Education Program (2009)
    13. [13]
    14. [14]
    15. [15]
      Administration of recombinant activated factor VII (NovoSeven) in three cases of uncontrolled bleeding caused by disseminated intravascular coagulopathy.Schmid S, Friesenecker B, Lorenz I, Innerhofer P, Koscielny J, Velik-Salchner C et al. Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis (2007)
    16. [16]
      High macrophage migration inhibitory factor levels in disseminated intravascular coagulation patients with systemic inflammation.Gando S, Sawamura A, Hayakawa M, Hoshino H, Kubota N, Nishihira J Inflammation (2007)
    17. [17]
      Potential role of recombinant activated factor VII for the treatment of severe bleeding associated with disseminated intravascular coagulation: a systematic review.Franchini M, Manzato F, Salvagno GL, Lippi G Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis (2007)
    18. [18]
      A prospective comparative study of three sets of criteria for disseminated intravascular coagulation: ISTH criteria vs Japanese criteria.Hayakawa M, Gando S, Hoshino H Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis (2007)
    19. [19]
    20. [20]
    21. [21]
      Beneficial effects of urokinase on lipopolysaccharide-induced disseminated intravascular coagulation in rats: focus on organ function and endothelin levels.Asakura H, Asamura R, Ontachi Y, Hayashi T, Omote M, Arahata M et al. Thrombosis and haemostasis (2005)
    22. [22]
      Low molecular weight heparin attenuates multiple organ failure in a murine model of disseminated intravascular coagulation.Slofstra SH, van 't Veer C, Buurman WA, Reitsma PH, ten Cate H, Spek CA Critical care medicine (2005)
    23. [23]
      High plasma fibrinogen level is associated with poor clinical outcome in DIC patients.Wada H, Mori Y, Okabayashi K, Gabazza EC, Kushiya F, Watanabe M et al. American journal of hematology (2003)
    24. [24]
    25. [25]
      Plasma levels of total plasminogen activator inhibitor-I (PAI-I) and tPA/PAI-1 complex in patients with disseminated intravascular coagulation and thrombotic thrombocytopenic purpura.Watanabe R, Wada H, Miura Y, Murata Y, Watanabe Y, Sakakura M et al. Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis (2001)
    26. [26]
      Elevated levels of free tissue factor pathway inhibitor antigen in cases of disseminated intravascular coagulation caused by various underlying diseases.Asakura H, Ontachi Y, Mizutani T, Kato M, Saito M, Morishita E et al. Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis (2001)
    27. [27]
    28. [28]
    29. [29]
    30. [30]
    31. [31]
      Disseminated intravascular coagulation.Huston CJ The American journal of nursing (1994)
    32. [32]
      Disseminated intravascular coagulation presenting as perioral haemorrhage.McLoughlin P, Chen S, Phillips JG The British journal of oral & maxillofacial surgery (1994)
    33. [33]
      Subcortical hemorrhage in disseminated intravascular coagulation associated with sepsis.Wijdicks EF, Silbert PL, Jack CR, Parisi JE AJNR. American journal of neuroradiology (1994)
    34. [34]
      Diffuse hepatic infarction with complete recovery in a neonate.Robbins C, Holzman IR The Journal of pediatrics (1992)
    35. [35]
      Role of endothelin in disseminated intravascular coagulation.Asakura H, Jokaji H, Saito M, Uotani C, Kumabashiri I, Morishita E et al. American journal of hematology (1992)
    36. [36]
    37. [37]
      Disseminating intravascular coagulation (DIC): nursing interventions.Turner G Advancing clinical care : official journal of NOAADN (1991)
    38. [38]
      Disseminated intravascular coagulation following percutaneous renal biopsy.Dara T, Lohr J American journal of nephrology (1991)
    39. [39]
      Disseminated intravascular coagulation.Risberg B, Andreasson S, Eriksson E Acta anaesthesiologica Scandinavica. Supplementum (1991)
    40. [40]
      Plasma levels of soluble thrombomodulin increase in cases of disseminated intravascular coagulation with organ failure.Asakura H, Jokaji H, Saito M, Uotani C, Kumabashiri I, Morishita E et al. American journal of hematology (1991)
    41. [41]
      Plasma level of tumor necrosis factor in disseminated intravascular coagulation.Wada H, Ohiwa M, Kaneko T, Tamaki S, Tanigawa M, Takagi M et al. American journal of hematology (1991)
    42. [42]
      Plasma level of IL-1 beta in disseminated intravascular coagulation.Wada H, Tamaki S, Tanigawa M, Takagi M, Mori Y, Deguchi A et al. Thrombosis and haemostasis (1991)
    43. [43]
      Idiopathic chronic disseminated intravascular coagulation.Green PJ, Lecointe-Jones A, Majer R, Weir P, Day L Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis (1990)
    44. [44]
      DIC: the insidious killer.Young LM Critical care nurse (1990)
    45. [45]
      Disseminated intravascular coagulation in Fusobacterium necrophorum septicemia.Page Y, Comtet C, Tardy B, Zeni F, Thevenet D, Lucht F et al. Scandinavian journal of infectious diseases (1990)
    46. [46]
      Varicella gangrenosa.Kidney DD, Watson JB, Nisar N Archives of disease in childhood (1988)
    47. [47]
      Fatal disseminated intravascular coagulation secondary to streptococcal cervicitis.Paraskevaides EC, Wilson MC European journal of obstetrics, gynecology, and reproductive biology (1988)
    48. [48]
      Disseminated intravascular coagulation with Fusobacterium necrophorum septicaemia.Potter MN, Drysdale HC, Price PA, Buck AC Postgraduate medical journal (1988)
    49. [49]
    50. [50]
    51. [51]
      Purpura fulminans in an adult.Tishler M, Abramov AL, Seligsohn U, Kahn Y Israel journal of medical sciences (1986)
    52. [52]
      Acid ingestion: another cause of disseminated intravascular coagulopathy.Greif F, Kaplan O Critical care medicine (1986)
    53. [53]
      The obstetric patient and disseminated intravascular coagulation.Weiner CP Clinics in perinatology (1986)
    54. [54]
      Purpura fulminans.Hodge SJ Cutis (1978)
    55. [55]
    56. [56]
      Disseminated intravascular thrombosis as the cause of ischaemic enterocolitis.Mason DY, Heenan PJ, Whitehead R The Medical journal of Australia (1976)
    57. [57]
    58. [58]
      Latex-bound antifibrinogen test for plasma fibrinogen assay.Castelan DJ, Hirsh J, Martin M Journal of clinical pathology (1968)

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