Consumption Coagulopathy Clinical Presentation

Updated: Aug 24, 2017
  • Author: Alexander Gozman, MD; Chief Editor: Robert J Arceci, MD, PhD  more...
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Presentation

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 [14] :

  • 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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