Disseminated intravascular coagulation (DIC) is an acquired syndrome characterized by excessive coagulation leading to both hemorrhage and thrombosis, occurring in 30-50% of sepsis patients. It has a variety of causes including infections, injuries, malignancies, and congenital disorders, with significant risk factors in neonates due to their immature coagulation systems. Early identification and management are crucial as DIC can rapidly progress to life-threatening multiorgan failure.

1. DISSEMINATED INTRAVASCULAR COAGULATION (DIC)
Dr Beatrice kyomugisa
Paediatrician
Fort portal RRH

2. Introduction/Definition
• Disseminated intravascular coagulation (DIC) is an acquired syndrome
characterized by excessive systemic activation of coagulation, resulting in
both hemorrhage and thrombosis.
OR
• Is a condition in which blood clots form throughout the body, blocking small
blood vessels
• DIC can progress rapidly into life-threatening multiorgan failure; thus,
identifying the underlying etiology is paramount to management

3. Epidemiology
• DIC may occur in 30-50% of patients with sepsis
• It develops in an estimated 1% of all hospitalized patients
• DIC occurs at all ages and in all races, and no particular sex predisposition
has been noted.

4. Causes of disseminated intravascular coagulation in children
1. Infection
• Bacteria: Meningococcus, gram-
positive and-negative bacterial
sepsis
• Virus: HIV, varicella-zoster,
cytomegalovirus, Dengue fever,
Ebola virus
• Fungal: Candida, Aspergillus
• Rickettsia : Rocky Mountain
Spotted fever
• Parasites: Malaria
2. Injury
• Brain injury
• Crush injury
• Massive burns
• Extensive surgery
3. Malignancy
• Acute promyelocytic leukemia
• Acute lymphoblastic leukemia
4. Microangiopathic disorders
• Giant hemangioma – Kasabach-
Merritt syndrome

5. Cont…..
5. Neonatal causes
• Birth asphyxia
• Respiratory distress syndrome
• Meconium aspiration
• Amniotic fluid aspiration
• Necrotizing enterocolitis
• Congenital infections: Neonatal
cytomegalovirus, herpes simplex
virus, bacterial or fungal
infections
6. Gastrointestinal disease
• Acute and chronic liver disease
• Reye syndrome
7. Congenital thrombotic disorders
• Homozygous deficiencies of
proteins C and S
• Antithrombin III deficiency

6. Risk factors of DIC in neonates
• Extreme prematurity
• Extremely low birth weight
• Low APGAR scores

7. Why is DIC common in neonates
• Platelets and coagulation factors are first detectable at 5 and 10 weeks of
gestation, respectively.
• Platelets reach normal range of 150–450×109 /L at 22 weeks of gestation
and most coagulation factors achieve adult values by 6 months of postnatal
age, with a few notable exceptions.
• Platelets are found to be hyporeactive and aggregation is impaired due to
deficiency of α-adrenergic receptors on platelet membrane, especially in
preterm infants

8. Coagulation pathway

9. Stages of DIC
Stage 1:
Overactive clotting leads to formation of blood clots throughout the blood
vessels. The clots can reduce or block blood flow, which can damage vital
organs.
Stage 2:
As DIC progresses, the overactive clotting uses up platelets and clotting
factors that help the blood to clot. Without these platelets and clotting
factors, DIC leads to bleeding just beneath the skin, in the nose or mouth, or
deeper tissues.

10. Pathogenesis of DIC
Intravascular activation of coagulation:
• Tissue damage from the initiating underlying disease releases procoagulants
into the bloodstream, resulting in intravascular activation of coagulation.
Examples of procoagulants include lipopolysaccharides from bacteria,
phospholipids from damaged vascular endothelium, or the formation of
neutrophil extracellular traps.
Formation of fibrin in the circulation:
• Tissue procoagulants activate hemostasis primarily through the interaction of
tissue factor and factor VII, thereby promoting fibrin formation and
deposition within the microcirculation.

11. Cont….
Fibrinolysis
• Fibrin formation activates the fibrinolytic pathway, which produces plasmin
that cleaves fibrinogen and fibrin, thereby generating fibrin degradation
products (FDPs). FDPs interfere with fibrin polymerization and impair platelet
aggregation.
Consumption of clotting factors and platelets:
• Ongoing activation of the coagulation system and fibrin deposition consume
clotting factors and platelets.

12. Cont….
Hemolysis:
• Intravascular fibrin strands cause mechanical shearing of red blood cells,
resulting in microangiopathic hemolytic anemia.
End-organ damage:
• Deposition of fibrin into the microcirculation of organs results in tissue
ischemia and damage.

13. Pathogenesis of DIC

15. Clinical features of DIC
• Bleeding venipuncture sites
• Oozing of blood from indwelling
catheters
• Spontaneous or minimal trauma-
related generalized ecchymoses
• Development of large, bullous
hemorrhagic skin lesions on previous
viral exanthematous sites
• Mucosal bleeding from gingiva,
gastrointestinal or renal tracts
• Unexpected and major bleeding
around drain sites or surgical wounds
post surgery in postoperative states
• Thrombophlebitis at unusual sites
• Renal impairment in the absence of other
explanations
• Fluctuating central nervous system
disturbances like confusion, and seizures
—consistent with the microcirculatory
ischemia
• Respiratory distress syndrome with no
obvious explanation
• Dermal infarcts and skin necrosis
• Greyish discoloration of finger tips, toes
or ear lobes, which has been termed
‘acral cyanosis’; usually seen in extreme
cases

17. Complications of DIC
• Multi organ failure e.g acute renal failure
• Life threatening hemorrhage e.g. Pulmonary embolism and hemorrhage
• Gangrene
• Venous thromboembolism
• Shock
• Stroke
• Acute respiratory distress syndrome

18. Differential diagnosis
• Dysfibrinogenemia
• Hemolytic uremic syndrome
• Heparin-induced thrombocytopenia
• Immune thrombocytopenia (ITP)
• Thrombotic thrombocytopenic purpura (TTP)
• Hepatic failure
• Vit K deficiency

19. Laboratory diagnosis of acute DIC
Lab Test DIC
CBC: Platelet count Decreased
PT/ (INR) Usually prolonged
aPPT Usually prolonged
Fibrinogen Usually decreased
Peripheral blood smear Microangiopathic hemolytic anemia with
schistocytes, helmet cells, and large platelets
Factor V and VIII levels Decreased
Confirmatory test
Fibrin degradation products(FDP) Positive more than 40 µg/mL
D-dimer Positive

20. Peripheral smear in microangiopathic hemolytic anemia
showing presence of schistocytes
• Multiple helmet cells (arrows)
• Fragmented red cells (small
arrowhead)
• Microspherocytes are also seen (large
arrowheads).
• The platelet number is reduced; the
large platelet in the center (dashed
arrow) suggests that the
thrombocytopenia is due to enhanced
destruction.

21. Diagnosis
Consider the diagnosis of DIC if all the following criteria are met:
The patient has an underlying condition that predisposes them to DIC (eg,
infection, trauma, malignancy)
Laboratory testing is consistent with DIC (ie, thrombocytopenia plus
coagulation factor consumption [prolonged PT and aPTT, low fibrinogen] and
fibrinolysis [elevated D-dimer])
No other etiology for these findings has been identified
• Clinical evidence of bleeding or thrombosis are supportive of the diagnosis but
are not required for diagnosis.

22. Management
• Treat an underlying condition that predisposes them to DIC (eg, infection,
trauma, malignancy). Antibiotics for severe sepsis
• Transfusion of platelets (10 to 15mls/kg)
• Replacement of clotting factors: Clotting factors can be replaced by either FFP
or cryoprecipitate. Repeat transfusions may be necessary. FFP dose of 10 to 15
mL/kg per infusion. Cryoprecipitate (factor VIII, von Willebrand factor, and
fibrinogen). It is administered as needed at a dose of 1 to 2 units per 10 kg (the
volume of a unit will vary; maximum 15 mL)
• Limited role of anticoagulation: Therapeutic anticoagulation has a very limited
role in the management of DIC in infants and children

23. Indications for platelet transfusion in children
• Less than 50,000 cells/μL with severe bleeding, including disseminated
intravascular coagulation
• Less than 30,000 cells/μL when bleeding is not life-threatening or
considered severe
• Less than 100,000 cells/μL for bleeding in the context of multiple trauma or
intracranial bleeding
• Surgical patient with active bleeding and platelet count <50-100×106/μL
• During massive transfusions with platelet count <75×106/μL
• ITP with major and/or dangerous bleeding (e.g., severe intestinal,
intracranial or intraocular hemorrhage)

24. Prognosis
• Disseminated intravascular coagulation can quickly lead to multiorgan
failure and death, particularly if early recognition and treatment fail to
occur.
• A high index of suspicion of this high-mortality disease in critically ill
patients remains paramount to improve outcomes in patients with DIC