Consumption Coagulopathy Treatment & Management: Medical Care, Surgical Care, Consultations
The most important concept in disseminated intravascular coagulation (DIC) is that it is a secondary manifestation of an underlying disorder.
The most important therapeutic maneuver is treating the initiating disorder. Without this, supportive measures ultimately fail.
Shock is a frequent underlying factor, and important supportive measures include ventilatory support, volume support, and pressor support as well as close monitoring of neurologic and renal function. Dialysis may be needed.
Involve a pediatric surgeon, as the underlying disorder indicates.
Surgical complications may include thrombotic occlusion of an artery with imminent loss of limb or organ function, bleeding, or compartment syndrome.
DIC can result in bleeding at any surgical site.
DIC is a complex pediatric disease that is best treated in tertiary care centers by using a multidisciplinary approach. Involving many services may be appropriate.
Treatment involves complex decisions regarding differential diagnosis and treatment options.
Involve a pediatric hematologist early.
If DIC is thought to be secondary to malignancy, a pediatric oncologist can expedite diagnosis.
Most children with DIC are critically ill and require monitoring available in the pediatric ICU.
Many children develop shock and respiratory failure and require ventilatory support.
Treatment of patients may involve blood products.
Blood bank specialists can provide resource advice on treatment decisions.
Many children with DIC have underlying sepsis that requires aggressive management.
Renal derangement is not uncommon because thrombosis and shock interfere with renal perfusion.
DIC may cause neurologic symptoms related to CNS thrombosis, infarction, or hemorrhage.
Medication
Alexander Gozman, MD Assistant Professor, Department of Pediatrics, Division of Hematology/Oncology, Albany Medical Center
Alexander Gozman, MD is a member of the following medical societies: American Medical Association, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, American Society of Clinical Oncology
Coauthor(s)
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, American Society of Pediatric Hematology/Oncology
Vikramjit S Kanwar, MBBS, MBA MRCP(UK), FAAP, Associate Professor and Division Chief of Pediatric Hematology and Oncology, Department of Pediatrics, Albany Medical Center
Vikramjit S Kanwar, MBBS, MBA is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, Royal College of Physicians, Children's Oncology Group
Disclosure: Received honoraria from Jazz Pharmaceutical for speaking and teaching.
Specialty Editor Board
Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Gary D Crouch, MD Associate Professor, Program Director of Pediatric Hematology-Oncology Fellowship, Department of Pediatrics, Uniformed Services University of the Health Sciences
Gary D Crouch, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology
Chief Editor
Robert J Arceci, MD, PhD Director, Children’s Center for Cancer and Blood Disorders, Department of Hematology/Oncology, Co-Director of the Ron Matricaria Institute of Molecular Medicine, Phoenix Children’s Hospital; Editor-in-Chief, Pediatric Blood and Cancer; Professor, Department of Child Health, University of Arizona College of Medicine
Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Association for Cancer Research, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology
Acknowledgements
Jennifer Boden Cerone, MD Resident Physician, Department of Pediatrics, The Children’s Hospital at Albany Medical Center
Jennifer Boden Cerone is a member of the following medical societies: American Academy of Pediatrics
Paul J Galardy, MD Instructor, Department of Pediatrics, Massachusetts General Hospital for Children and Harvard Medical School
Eric Grabowski, MD, ScD Director of Cardiovascular Thrombosis Laboratory, Massachusetts General Hospital for Children; Associate Professor, Department of Pediatrics, Division of Hematology-Oncology, Harvard Medical School
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
References
Purpura fulminans.
Peripheral blood of a child with disseminated intravascular coagulation demonstrates thrombocytopenia and many schistocytes (Wright stain, original magnification X 1000).
Table. DIC Scoring System
Medical Care
The most important concept in disseminated intravascular coagulation (DIC) is that it is a secondary manifestation of an underlying disorder.
The most important therapeutic maneuver is treating the initiating disorder. Without this, supportive measures ultimately fail.
Shock is a frequent underlying factor, and important supportive measures include ventilatory support, volume support, and pressor support as well as close monitoring of neurologic and renal function. Dialysis may be needed.
Surgical Care
Involve a pediatric surgeon, as the underlying disorder indicates.
Surgical complications may include thrombotic occlusion of an artery with imminent loss of limb or organ function, bleeding, or compartment syndrome.
DIC can result in bleeding at any surgical site.
Consultations
DIC is a complex pediatric disease that is best treated in tertiary care centers by using a multidisciplinary approach. Involving many services may be appropriate.
Hematologist-oncologist
Treatment involves complex decisions regarding differential diagnosis and treatment options.
Involve a pediatric hematologist early.
If DIC is thought to be secondary to malignancy, a pediatric oncologist can expedite diagnosis.
Intensivist
Most children with DIC are critically ill and require monitoring available in the pediatric ICU.
Many children develop shock and respiratory failure and require ventilatory support.
Blood bank specialist
Treatment of patients may involve blood products.
Blood bank specialists can provide resource advice on treatment decisions.
Infectious disease specialist
Many children with DIC have underlying sepsis that requires aggressive management.
Nephrologist
Renal derangement is not uncommon because thrombosis and shock interfere with renal perfusion.
Neurologist
DIC may cause neurologic symptoms related to CNS thrombosis, infarction, or hemorrhage.
Medication
Alexander Gozman, MD Assistant Professor, Department of Pediatrics, Division of Hematology/Oncology, Albany Medical Center
Alexander Gozman, MD is a member of the following medical societies: American Medical Association, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, American Society of Clinical Oncology
Coauthor(s)
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, American Society of Pediatric Hematology/Oncology
Vikramjit S Kanwar, MBBS, MBA MRCP(UK), FAAP, Associate Professor and Division Chief of Pediatric Hematology and Oncology, Department of Pediatrics, Albany Medical Center
Vikramjit S Kanwar, MBBS, MBA is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, Royal College of Physicians, Children's Oncology Group
Disclosure: Received honoraria from Jazz Pharmaceutical for speaking and teaching.
Specialty Editor Board
Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Gary D Crouch, MD Associate Professor, Program Director of Pediatric Hematology-Oncology Fellowship, Department of Pediatrics, Uniformed Services University of the Health Sciences
Gary D Crouch, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology
Chief Editor
Robert J Arceci, MD, PhD Director, Children’s Center for Cancer and Blood Disorders, Department of Hematology/Oncology, Co-Director of the Ron Matricaria Institute of Molecular Medicine, Phoenix Children’s Hospital; Editor-in-Chief, Pediatric Blood and Cancer; Professor, Department of Child Health, University of Arizona College of Medicine
Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Association for Cancer Research, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology
Acknowledgements
Jennifer Boden Cerone, MD Resident Physician, Department of Pediatrics, The Children’s Hospital at Albany Medical Center
Jennifer Boden Cerone is a member of the following medical societies: American Academy of Pediatrics
Paul J Galardy, MD Instructor, Department of Pediatrics, Massachusetts General Hospital for Children and Harvard Medical School
Eric Grabowski, MD, ScD Director of Cardiovascular Thrombosis Laboratory, Massachusetts General Hospital for Children; Associate Professor, Department of Pediatrics, Division of Hematology-Oncology, Harvard Medical School
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
References
- Levi M, Ten Cate H. Disseminated intravascular coagulation. N Engl J Med. 1999 Aug 19. 341(8):586-92. [Medline].
- 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. 1997 Aug. 55(4):169-74. [Medline].
- Nieuwland R, Berckmans RJ, McGregor S, Boing AN, Romijn FP, Westendorp RG. Cellular origin and procoagulant properties of microparticles in meningococcal sepsis. Blood. 2000 Feb 1. 95(3):930-5. [Medline].
- Morel N, Morel O, Petit L, et al. Generation of procoagulant microparticles in cerebrospinal fluid and peripheral blood after traumatic brain injury. J Trauma. 2008 Mar. 64(3):698-704. [Medline].
- 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. 2008 Jun. 87(6):451-7. [Medline].
- Arbuthnot C, Wilde JT. Haemostatic problems in acute promyelocytic leukaemia. Blood Rev. 2006 Jun 3. [Medline].
- 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. 2001 Jan. 12(1):1-8. [Medline].
- Grewal PK, Uchiyama S, Ditto D, Varki N, Le DT, Nizet V. The Ashwell receptor mitigates the lethal coagulopathy of sepsis. Nat Med. 2008 Jun. 14(6):648-55. [Medline].
- 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. 2005 Dec. 22(8):679-88. [Medline].
- Wada H. Disseminated intravascular coagulation. Clin Chim Acta. 2004 Jun. 344(1-2):13-21. [Medline].
- Chi S, Ikezoe T. Disseminated intravascular coagulation in non-Hodgkin lymphoma. Int J Hematol. 2015 Oct. 102 (4):413-9. [Medline].
- Cunningham FG, Nelson DB. Disseminated Intravascular Coagulation Syndromes in Obstetrics. Obstet Gynecol. 2015 Oct 5. [Medline].
- Levi M, de Jonge E, Meijers J. The diagnosis of disseminated intravascular coagulation. Blood Rev. 2002 Dec. 16(4):217-23. [Medline].
- Hjorleifsson E, Sigurdsson MI, Gudmundsdottir BR, Sigurdsson GH, Onundarson PT. Prediction of survival in patients suspected of disseminated intravascular coagulation. Acta Anaesthesiol Scand. 2015 Aug. 59 (7):870-80. [Medline].
- Taylor FB, Toh CH, Hoots WK, et al. Towards definition, clinical and laboratory criteria, and a scoring system for disseminated intravascular coagulation. Thromb Haemost. 2001 Nov. 86(5):1327-30. [Medline].
- 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. 2004 Apr. 91(4):812-8. [Medline].
- 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. 2006 Sep. 17(6):445-51. [Medline].
- Soundar EP, Jariwala P, Nguyen TC, Eldin KW, Teruya J. Evaluation of the international society on thrombosis and haemostasis and institutional diagnostic criteria of disseminated intravascular coagulation in pediatric patients. Am J Clin Pathol. 2013 Jun. 139(6):812-6. [Medline].
- 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].
- 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. 2000 Dec 1. 96(12):3719-24. [Medline].
- Bernard GR, Vincent JL, Laterre PF, et al. Efficacy and safety of recombinant human activated protein C for severe sepsis. N Engl J Med. 2001 Mar 8. 344(10):699-709. [Medline].
- 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. 2006 May. 90(4):294-301. [Medline].
- 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. 2008 Jun. 25(4):301-11. [Medline].
- 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. 2008 May. 30(5):337-42. [Medline].
- Nadel S, Goldstein B, Williams MD, et al. Drotrecogin alfa (activated) in children with severe sepsis: a multicentre phase III randomised controlled trial. Lancet. 2007 Mar 10. 369(9564):836-43. [Medline].
- Marti-Carvajal AJ, Comunian-Carrasco G, Pena-Marti GE. Haematological interventions for treating disseminated intravascular coagulation during pregnancy and postpartum. Cochrane Database Syst Rev. 2011 Mar 16. 3:CD008577. [Medline].
- 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. 2008 Sep. 34(9):1707-12. [Medline].
- Levi M. Disseminated intravascular coagulation: What's new?. Crit Care Clin. 2005 Jul. 21(3):449-67. [Medline].
- Sallah S, Husain A, Nguyen NP. Recombinant activated factor VII in patients with cancer and hemorrhagic disseminated intravascular coagulation. Blood Coagul Fibrinolysis. 2004 Oct. 15(7):577-82. [Medline].
- Wang ZY, Chen Z. Acute promyelocytic leukemia: from highly fatal to highly curable. Blood. 2008 Mar 1. 111(5):2505-15. [Medline].
Purpura fulminans.
Peripheral blood of a child with disseminated intravascular coagulation demonstrates thrombocytopenia and many schistocytes (Wright stain, original magnification X 1000).
- Table. DIC Scoring System
Table. DIC Scoring System
| Measure | Score | |||
| 0 | 1 | 2 | 3 | |
| Platelet count | >100 X 10/L | < 100 X 10/L | < 50 X 10/L | NA |
| PT prolongation, s | 0-3 | 3-6 | 6 | NA |
| Fibrinogen level (mg/dL) | >100 | < 100 | NA | NA |
| Fibrin split products | NA | NA | + | +++ |
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