Non-Traumatic Fat Embolism Syndrome in Two HbSS Sickle Cell Disease Patients: A Case Series and Review

Mohammed Amouri Alzayer,1 Salah Abohelaika,2,3 Mousa Alhaddad,4 Mazen Al Zayer,5 Ahmed J Alali,6 Abdullah Mohammad Al Abbas,1 Nabeel J Al Eid,1 Fatimah A Al Saeed,1 Hussain H Al Saeed1 1Hematology and Inherited Blood Disorders Center, Prince Mohammed bin Fahad Hospital of Inherited Blood Disorders, Qatif, 32654, Saudi Arabia; 2Research Department, Qatif Central Hospital, Qatif, 32654, Saudi Arabia; 3Pharmacy Department, Qatif Central Hospital, Qatif, 32654, Saudi Arabia; 4Adult Hematology and Stem Cell Transplantation Department, King Fahad Specialist Hospital, Dammam, 32253, Saudi Arabia; 5Adult Intensive Care Department, Qatif Central Hospital, Qatif, 32654, Saudi Arabia; 6Adult Intensive Care Department, Prince Mohammed bin Fahad Hospital of Inherited Blood Disorders, Qatif, 32654, Saudi ArabiaCorrespondence: Mohammed Amouri Alzayer, Hematology and Inherited Blood Disorders Center, Prince Mohammed bin Fahad Hospital of Inherited Blood Disorders, Qatif, 32654, Saudi Arabia, Email [email protected] Salah Abohelaika, Research Department, Qatif Central Hospital, Qatif, 32654, Saudi Arabia, Email [email protected]: Non-traumatic fat embolism syndrome (FES) in sickle cell disease (SCD) is associated with high morbidity and mortality. This severe complication arises from bone marrow necrosis, which subsequently can lead to fat embolism syndrome and multi-organ failure. A key challenge in diagnosing and managing this syndrome lies in the absence of established diagnostic criteria and the variability of its clinical presentation. Instant red cell exchange transfusion could be lifesaving once the syndrome is suspected, and the use of therapeutic plasma exchange, as suggested in the literature, can improve outcomes. Here, we report two cases of SCD with FES that were managed successfully with blood transfusion and plasma exchange. Both of them rapidly deteriorated and required intensive care unit admission, where they received red cell exchange followed by plasma exchange. They both made a good recovery and were discharged from the hospital within two weeks.Keywords: sickle cell disease, bone marrow necrosis, fat embolism syndrome, multiorgan failure, blood transfusion, plasma exchange