Systemic fat embolism and pulmonary hypertension in sickle cell disease

Hematol Oncol Clin North Am. 1996 Dec;10(6):1289-303. doi: 10.1016/s0889-8588(05)70401-9.


Systemic fat embolism, a relatively rare complication of sickle cell disease, is difficult to diagnose and it is often fatal. A high index of suspicion and early transfusion therapy may provide the best chance for recovery. Sickle cell-related pulmonary hypertension can be documented by cardiac catheterization but has no proven treatment. Patients with this complication are usually adults, have a poor prognosis, and may be considered for hydroxyurea treatment. Administration of vasodilators, anticoagulation, or oxygen may be beneficial in selected individuals.

Publication types

  • Review

MeSH terms

  • Anemia, Sickle Cell / complications*
  • Anemia, Sickle Cell / physiopathology
  • Embolism, Fat / diagnosis
  • Embolism, Fat / etiology*
  • Embolism, Fat / physiopathology
  • Embolism, Fat / therapy
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Pulmonary / physiopathology
  • Hypertension, Pulmonary / therapy
  • Prevalence