Distinctive Acellular Lipid Emboli in Hemoglobin SC Disease following Bone Marrow Infarction with Parvovirus...

Area of Science:

• Hematology
• Pathology
• Pulmonology

Background:

• Sickle hemoglobinopathies, such as hemoglobin SC disease, are associated with various complications.
• Fat Embolism Syndrome (FES) is a rare but serious condition.
• The co-occurrence of sickle hemoglobinopathies and FES presents diagnostic challenges.

Purpose of the Study:

• To report a case of fatal fat embolism syndrome in an adolescent with hemoglobin SC disease.
• To highlight the pathological findings associated with this rare complication.
• To emphasize the importance of recognizing the association between sickle hemoglobinopathies and FES for premortem diagnosis.

Main Methods:

• Case report of an adolescent with hemoglobin SC disease.
• Clinical presentation including pelvic pain, respiratory, and neurologic deterioration.
• Autopsy findings including acellular fat emboli, marrow necrosis, aggregated sickle cells, and parvovirus immunostaining.

Main Results:

• The patient experienced rapid deterioration and death.
• Autopsy revealed extensive acellular fat emboli in the lung and brain.
• Evidence of marrow necrosis with sickle cell aggregation and parvovirus positivity was found in the spine.

Conclusions:

• This case underscores the association between sickle hemoglobinopathies and fat embolism syndrome.
• Fat embolism syndrome can be a cause of death in patients with sickle cell disease.
• A high index of suspicion and knowledge of FES symptoms are crucial for premortem diagnosis in patients with sickle hemoglobinopathy.