eMedicine Specialties > Pediatrics: General Medicine > Hematology

Consumption Coagulopathy: Differential Diagnoses & Workup

Author: Jennifer Boden Cerone, MD, Resident Physician, Department of Pediatrics, The Children's Hospital at Albany Medical Center
Coauthor(s): Vikramjit S Kanwar, MD, MBA, MRCP(UK), FAAP, Associate Professor of Pediatric Hematology and Oncology, Department of Pediatrics, Albany Medical Center; Faculty, Alden March Bioethics Institute; Richard H Sills, MD, Professor of Pediatrics, Upstate Medical University
Contributor Information and Disclosures

Updated: Jan 6, 2009

Differential Diagnoses

Fulminant Hepatic Failure
Hemolytic-Uremic Syndrome
Hemorrhagic Disease of Newborn
Lymphohistiocytosis

Other Problems to Be Considered

Postbypass pump syndrome
Posttransfusion purpura
Thrombotic thrombocytopenic purpura

Workup

Laboratory Studies

  • Screening
    • No single test or combination of tests is adequate to diagnose disseminated intravascular coagulation (DIC).11 Perform screening tests in all patients, such as platelet count, prothrombin time (PT), activated partial thromboplastin time (aPTT), thrombin time, and fibrin degradation products or soluble fibrin monomers. Additional tests that may be useful in aiding in the diagnosis include antithrombin III levels, protein C, D-dimer, fibrinogen, measurements of specific coagulation factors such as factor V and VIII, and plasminogen activator inhibitor type I (PAI-1). Clinical judgment in conjunction with these tests provide a means of working towards a diagnosis of DIC, although no single test results alone are confirmatory.  
    • Thrombocytopenia is an almost universal finding, and the CBC count with smear review may reveal findings suggestive of DIC, such as increased platelet size, schistocytes, and helmet cells (see Media file 2).
    • The PT and the aPTT are usually prolonged on screening tests but may be normal in an individual in the early phase of DIC ("nonovert DIC").
    • Fibrin or fibrinogen degradation products and D-dimers are usually elevated due to the rapid generation of fibrin and breakdown of cross-linked fibrin polymers. Although sensitive, these tests are not specific.
    • Fibrinogen levels are often decreased, as is antithrombin III.
  • The International Society on Thrombosis and Hemostasis (ISTH) DIC scoring system can help in diagnosis.12 In the presence of an underlying cause, key tests are performed, and the results are scored as shown in the Table below. A total score of 5 or greater is diagnostic of DIC. DIC Scoring System

    Open table in new window

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    Table
    MeasureScore
    0123
    Platelet count>100 X 109/L<100 X 109/L<50 X 109/LNA
    PT prolongation, s0-33-66NA
    Fibrinogen level (mg/dL)>100<100NANA
    Fibrin split productsNANA++++
    MeasureScore
    0123
    Platelet count>100 X 109/L<100 X 109/L<50 X 109/LNA
    PT prolongation, s0-33-66NA
    Fibrinogen level (mg/dL)>100<100NANA
    Fibrin split productsNANA++++
  • The most important prognostic factor is the ability to correct the underlying cause and arrest the ongoing derangement of the coagulation system.
  • A similar scoring system proposed by the Japanese Ministry of Health and Welfare (JMHW) in 1979 used more specific criteria. They numerically categorized the extent of fibrin degradation product, included underlying disease and clinical symptoms as part of the overall scoring system; whereas underlying disease is an essential component in the ISTH system.10  The ISTH system is useful for predicting overt DIC.
  • The ISTH DIC scoring system does not precisely define  the extent of the increase in fibrin split products or which marker is being measured.   Specifying these fibrin products as either D-dimers or soluble fibrin monomers and clearly defining numerical parameters of these products within the scoring system makes the score a more powerful prognostic indicator.13,14   
  • In a retrospective study evaluating prognostic factors influencing mortality in pediatric patients with DIC, multiorgan dysfunction syndrome, acute respiratory distress syndrome, and cardiovascular and respiratory system dysfunction were associated with increased mortality.9

Other Tests

  • Because DIC is not the primary disease but a manifestation of underlying illness, diagnosing the initiating disorder is crucial.

More on Consumption Coagulopathy

Overview: Consumption Coagulopathy
Differential Diagnoses & Workup: Consumption Coagulopathy
Treatment & Medication: Consumption Coagulopathy
Follow-up: Consumption Coagulopathy
Multimedia: Consumption Coagulopathy
References
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References

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  2. Shimura M, Wada H, Wakita Y, et al. Plasma tissue factor and tissue factor pathway inhibitor levels in patients with disseminated intravascular coagulation. Am J Hematol. Aug 1997;55(4):169-74. [Medline].

  3. Nieuwland R, Berckmans RJ, McGregor S, Boing AN, Romijn FP, Westendorp RG. Cellular origin and procoagulant properties of microparticles in meningococcal sepsis. Blood. Feb 1 2000;95(3):930-5. [Medline].

  4. Morel N, Morel O, Petit L, et al. Generation of procoagulant microparticles in cerebrospinal fluid and peripheral blood after traumatic brain injury. J Trauma. Mar 2008;64(3):698-704. [Medline].

  5. Langer F, Spath B, Haubold K, Holstein K, Marx G, Wierecky J. Tissue factor procoagulant activity of plasma microparticles in patients with cancer-associated disseminated intravascular coagulation. Ann Hematol. Jun 2008;87(6):451-7. [Medline].

  6. Arbuthnot C, Wilde JT. Haemostatic problems in acute promyelocytic leukaemia. Blood Rev. Jun 3 2006;[Medline].

  7. Asakura H, Ontachi Y, Mizutani T, et al. Elevated levels of free tissue factor pathway inhibitor antigen in cases of disseminated intravascular coagulation caused by various underlying diseases. Blood Coagul Fibrinolysis. Jan 2001;12(1):1-8. [Medline].

  8. Grewal PK, Uchiyama S, Ditto D, Varki N, Le DT, Nizet V. The Ashwell receptor mitigates the lethal coagulopathy of sepsis. Nat Med. Jun 2008;14(6):648-55. [Medline].

  9. Oren H, Cingoz I, Duman M. Disseminated intravascular coagulation in pediatric patients: clinical and laboratory features and prognostic factors influencing the survival. Pediatr Hematol Oncol. Dec 2005;22(8):679-88. [Medline].

  10. Wada H. Disseminated intravascular coagulation. Clin Chim Acta. Jun 2004;344(1-2):13-21. [Medline].

  11. Levi M, de Jonge E, Meijers J. The diagnosis of disseminated intravascular coagulation. Blood Rev. Dec 2002;16(4):217-23. [Medline].

  12. Taylor FB, Toh CH, Hoots WK, et al. Towards definition, clinical and laboratory criteria, and a scoring system for disseminated intravascular coagulation. Thromb Haemost. Nov 2001;86(5):1327-30. [Medline].

  13. Dempfle CE, Wurst M, Smolinski M, et al. Use of soluble fibrin antigen instead of D-dimer as fibrin-related marker may enhance the prognostic power of the ISTH overt DIC score. Thromb Haemost. Apr 2004;91(4):812-8. [Medline].

  14. Voves C, Wuillemin WA, Zeerleder S. International Society on Thrombosis and Haemostasis score for overt disseminated intravascular coagulation predicts organ dysfunction and fatality in sepsis patients. Blood Coagul Fibrinolysis. Sep 2006;17(6):445-51. [Medline].

  15. Ettingshausen CE, Veldmann A, Beeg T, Schneider W, Jäger G, Kreuz W. Replacement therapy with protein C concentrate in infants and adolescents with meningococcal sepsis and purpura fulminans. Semin Thromb Hemost. 1999;25(6):537-41. [Medline].

  16. White B, Livingstone W, Murphy C, Hodgson A, Rafferty M, Smith OP. An open-label study of the role of adjuvant hemostatic support with protein C replacement therapy in purpura fulminans-associated meningococcemia. Blood. Dec 1 2000;96(12):3719-24. [Medline].

  17. Bernard GR, Vincent JL, Laterre PF, et al. Efficacy and safety of recombinant human activated protein C for severe sepsis. N Engl J Med. Mar 8 2001;344(10):699-709. [Medline].

  18. Schellongowski P, Bauer E, Holzinger U, Staudinger T, Frass M, Laczika K. Treatment of adult patients with sepsis-induced coagulopathy and purpura fulminans using a plasma-derived protein C concentrate (Ceprotin). Vox Sang. May 2006;90(4):294-301. [Medline].

  19. Yilmaz D, Karapinar B, Balkan C, Akisu M, Kavakli K. Single-center experience: use of recombinant factor VIIa for acute life-threatening bleeding in children without congenital hemorrhagic disorder. Pediatr Hematol Oncol. Jun 2008;25(4):301-11. [Medline].

  20. Fischer D, Schloesser R, Buxmann H, Veldman A. Recombinant activated Factor VII as a hemostatic agent in very low birth weight preterms with gastrointestinal hemorrhage and disseminated intravascular coagulation. J Pediatr Hematol Oncol. May 2008;30(5):337-42. [Medline].

  21. Baratto F, Michielan F, Meroni M, Dal Palu A, Boscolo A, Ori C. Protein C concentrate to restore physiological values in adult septic patients. Intensive Care Med. Sep 2008;34(9):1707-12. [Medline].

  22. Bick RL. Disseminated intravascular coagulation: objective clinical and laboratory diagnosis, treatment, and assessment of therapeutic response. Semin Thromb Hemost. 1996;22(1):69-88. [Medline].

  23. de Jonge E, Dekkers PE, Creasey AA, et al. Tissue factor pathway inhibitor dose-dependently inhibits coagulation activation without influencing the fibrinolytic and cytokine response during human endotoxemia. Blood. Feb 15 2000;95(4):1124-9. [Medline].

  24. Hazelzet JA, Risseeuw-Appel IM, Kornelisse RF, et al. Age-related differences in outcome and severity of DIC in children with septic shock and purpura. Thromb Haemost. Dec 1996;76(6):932-8. [Medline].

  25. Levi M. Disseminated intravascular coagulation: What's new?. Crit Care Clin. Jul 2005;21(3):449-67. [Medline].

  26. Sakuragawa N, Hasegawa H, Maki M, et al. Clinical evaluation of low-molecular-weight heparin (FR-860) on disseminated intravascular coagulation (DIC)--a multicenter co- operative double-blind trial in comparison with heparin. Thromb Res. Dec 15 1993;72(6):475-500. [Medline].

  27. Sallah S, Husain A, Nguyen NP. Recombinant activated factor VII in patients with cancer and hemorrhagic disseminated intravascular coagulation. Blood Coagul Fibrinolysis. Oct 2004;15(7):577-82. [Medline].

  28. Wang ZY, Chen Z. Acute promyelocytic leukemia: from highly fatal to highly curable. Blood. Mar 1 2008;111(5):2505-15. [Medline].

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Further Reading

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Keywords

consumption coagulopathy, disseminated intravascular coagulation, DIC, sepsis, sepsis-related DIC, trauma, massive head injuries, excess production of thrombin, brain trauma, acute promyelocytic leukemia, APL, hypotension, snake venom, snake bites, placental abruption, eclampsia, premature rupture of membranes, maternal fever, Kasabach-Merritt syndrome, Klippel-Trenaunay syndrome, vascular malformations, malignancy, thrombotic disorder, purpura fulminans, varicella, protein C deficiency, meningococcemia, Rocky Mountain spotted fever, herpes simplex, hepatitis, cytomegalovirus, CMV, Aspergillus infection, histoplasmosis, malaria, trypanosomiasis, neuroblastoma, systemic lupus erythematosus, juvenile rheumatoid arthritis

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Contributor Information and Disclosures

Author

Jennifer Boden Cerone, MD, Resident Physician, Department of Pediatrics, The Children's Hospital at Albany Medical Center
Jennifer Boden Cerone, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Coauthor(s)

Vikramjit S Kanwar, MD, MBA, MRCP(UK), FAAP, Associate Professor of Pediatric Hematology and Oncology, Department of Pediatrics, Albany Medical Center; Faculty, Alden March Bioethics Institute
Vikramjit S Kanwar, MD, MBA, MRCP(UK), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and Royal College of Physicians of the United Kingdom
Disclosure: Nothing to disclose.

Richard H Sills, MD, Professor of Pediatrics, Upstate Medical University
Richard H Sills, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Society of Hematology, and American Society of Pediatric Hematology/Oncology
Disclosure: Nothing to disclose.

Medical Editor

Gary R Jones, MD, Associate Medical Director, Clinical Development, Berlex Laboratories
Gary R Jones, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, and Western Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Gary D Crouch, MD, Program Director of Pediatric Hematology-Oncology Fellowship, Department of Pediatrics, Associate Professor, Uniformed Services University of the Health Sciences
Gary D Crouch, MD is a member of the following medical societies: American Academy of Pediatrics and American Society of Hematology
Disclosure: Nothing to disclose.

CME Editor

Samuel Gross, MD, Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University
Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD, King Fahd Professor of Pediatric Oncology, Department of Oncology, Division of Pediatric Oncology, Johns Hopkins University School of Medicine
Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology
Disclosure: Nothing to disclose.

 
 
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