Consumption Coagulopathy Clinical Presentation

  • Author: Vikramjit S Kanwar, MD, MBA, MRCP(UK), FAAP; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Oct 26, 2011
 

History

The history should be tailored to the age of the child. Important historical aspects in disseminated intravascular coagulation (DIC) are the presence or suspected presence of any known predisposing conditions, especially sepsis. With meningococcal and pneumococcal sepsis, the prodrome may be limited, and the first indication of problems may be a purpuric rash with fever and hypotension.

Obtain appropriate historical facts, as follows:

  • History of fever
  • Behavior changes: Alterations in mental status may be indicative of CNS infection, an encephalopathic condition, or CNS insult such as thrombosis, hemorrhage, or infarction.
  • Feeding patterns: Alteration of feeding patterns may indicate illness in the infant or nonverbal child.
  • Urine output, as a measure of hydration status as well as cardiovascular and renal function
  • Sick contacts, exposure to potential bacterial or viral agents that are known causes of DIC in the pediatric population
  • Recent travel, exposure to fungal or parasitic agents endemic to particular areas

Obtain a birth history, including the following:

  • Perinatal course (eg, placental abruption or eclampsia)
  • Prenatal testing
  • Neonatal risk factors of sepsis (eg, premature rupture of membranes, maternal fever, fetal tachycardia, maternal group B streptococcal status, perinatal antibiotic therapy)
  • Immediate postnatal course, especially neonatal illnesses
  • Sepsis evaluation
  • Antibiotic therapy

Obtain other history, as follows:

  • Recent illness
  • Recent bruising - Indicates an underlying hematologic disorder
  • Fatigue
  • Frequent infections
  • Weight loss - May indicate the presence of underlying chronic illness or malignancy
  • Menstrual history - To evaluate likelihood of pregnancy in female adolescents
  • Use of any legal or illegal drugs
  • Family history suggestive of an inherited thrombotic disorder or cancer syndrome
  • Chronic illnesses, including malignancy, vascular malformations (eg, Kasabach-Merritt syndrome, Klippel-Trenaunay syndrome), and inherited or acquired immunodeficiencies
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Physical

Clinical manifestations depend on whether the onset is acute or chronic.

Acute onset (Minutes to days)

The patient's general appearance is frequently toxic.

The clinical picture is commonly one of bleeding with signs of shock out of proportion to the amount of blood loss, with poor perfusion, cold extremities, and poor tone in the neonate.

Bleeding may range in severity from petechiae, purpura, subconjunctival or mucosal hemorrhages and extravasation from past venipuncture or surgical sites, to severe life-threatening hemorrhage.

Coexisting signs of bleeding and thrombosis may be present.

Purpura fulminans (see the image below) is severe, extensive hemorrhage into the skin associated with fever and hypotension.

Purpura fulminans. Purpura fulminans.

It may be caused by infections, such as meningococcemia and varicella, or by protein C deficiency. Cutaneous purpuric or hemorrhagic lesions rapidly develop and spread and may progress to frank gangrene.

In addition to these signs, renal, hepatic, pulmonary, or CNS manifestations often accompany DIC. Most patients are critically ill.

The clinical appearance of each patient heavily depends on the underlying cause.

In many instances, determining if clinical manifestations are a result of DIC or an underlying disorder is difficult.

Chronic onset (Days to weeks)

Patients with specific underlying disorders may develop a chronic form of DIC.

Chronic onset occurs in children with large vascular malformations and in women with intrauterine fetal demise, chronic inflammation, and certain forms of malignancy (eg, acute promyelocytic leukemia, metastatic alveolar rhabdomyosarcoma). These patients have a low, constant rate of thrombin formation that does not outstrip the body's ability to compensate.

Patients with chronic DIC may not have obvious clinical manifestations. Patients may develop slowly resolving ecchymoses or have prolonged bleeding from internal or cutaneous wounds.

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Causes

DIC has numerous causes from conditions in many organ systems. The abbreviated list below emphasizes the pediatric causes of DIC.

Infections, as follows:

Obstetric complications, as follows:

  • Placental abruption
  • Amniotic fluid embolism
  • Intrauterine fetal demise

Malignancies, as follows:

  • Acute leukemia - Promyelocytic (M3), myelomonocytic (M4), monocytic (M5), lymphoblastic (T cell), and lymphoblastic (Philadelphia-chromosome positive)
  • Metastatic tumors -Neuroblastoma, alveolar rhabdomyosarcoma

Collagen vascular disorders, as follows:

Trauma, as follows:

  • Massive head trauma
  • Burn injuries
  • Major surgery
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Contributor Information and Disclosures
Author

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.

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, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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 and American Society of Hematology

Disclosure: Nothing to disclose.

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, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at 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.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors Paul J Galardy, MD, Eric Grabowski, MD, ScD, and Jennifer Boden Cerone, MD, to the development and writing of this article.

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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
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++++
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