Successful therapeutic plasma exchange for a patient with sickle cell disease and fat embolism syndrome after a failure of a response to red cell exchange transfusion

Abstract

Background: Fat embolism syndrome (FES) is a rare complication, which was reported mostly with milder forms of heterozygous sickle cell disease (SCD). It may present in a catastrophic way with multi-organ failure, particularly involving the pulmonary and neurological systems. Diagnosis is often missed or delayed; and the standard recommended treatment is red cell exchange (RCE) transfusion, which has sub-optimal results, such as debilitating long-term neurological complications. Recently, few reports suggested that the addition of Therapeutic Plasma Exchange (TPE) might further improve the outcome.

Fat Emboli with Thrombocytopenia in A Patient Undergoing Operation for A 1/3 Medial Humerus Oblique Fracture

“…Other complaints were denied. The patient also had no history of any disease and was immediately rushed to the emergency room after falling [8]. The patient has a habit of smoking 1 pack per day and works mostly in a sitting position [9].…”

Therapeutic plasma exchange for sickle cell disease acute complications: A systematic review

“…3,[7][8][9][10][11] Although RBCX removes sickled RBCs, progression of SCD-related complications may occur due to oxidative stress, inflammatory cytokines, and accumulation of acute phase proteins. 1,7,[12][13][14][15] Therefore, in selected patients with acute SCD complications, RBCX alone may not be enough. 1,2 In patients with continued deterioration despite RBCX, some case reports demonstrate improvement with therapeutic plasma exchange (TPE).…”

“…proteins. 7,[12][13][14]16 In recent years, the use of TPE in SCD has seen an increase. 3 This increase in reported cases of TPE use in SCD may have prompted its inclusion as a category III recommendation in the 2023 American Society for Apheresis (ASFA) guidelines.…”

“…17 However, despite the growing use of TPE, little is understood about TPE's impact on patient outcomes. 12 To better understand TPE outcomes in acute complications of SCD, we conducted a systematic review of the existing literature. As demonstrated in the sections below, although TPE may offer potential benefits for SCD patients who are critically ill, more evidence is needed.…”