Defibrotide in Sickle Cell Disease-Related Acute Chest Syndrome

Inclusion Criteria:

- SCD-associated ACS with the presence of any two or more of the following signs not
explained by other etiologies: Fever, Chest pain, Cough, Dyspnea, Tachypnea for age,
Pulmonary infiltrate on CXR and/or Chest CT scan, Decreased O2 saturation with or
without oxygen supplement;

- Age 2 to 40 years of age;

- Homozygous Hemoglobin S Disease, Hemoglobin SC Disease or Hemoglobin S 0/+
thalassemia;

- Informed consent/assent;

- Consent of patient/parent within ≤72 hours after inpatient admission for
SCD-associated ACS.

- Females of childbearing age will have a negative pregnancy test.

Exclusion Criteria:

- Current Grade III or IV hemorrhage;

- Previous hypersensitivity reaction to defibrotide;

- Current systemic anti-coagulant therapy and/or fibrinolytic therapy;

- Consent of patient/parent greater than 72 hours of inpatient admission for
SCD-associated ACS;

- No signed informed consent