The pathophysiology of ES is not clear, but is thought to involve an excess of pro-inflammatory cytokines, such as tumor necrosis factor and interleukin-1, which in turn may activate cytotoxic T-cells.
ES is typically seen after autologous transplantation, though has been reported after allogeneic transplantation as well. Potential risk factors for ES include exposure to granulocyte colony-stimulating factor (G-CSF), certain conditioning regimens (such as cyclophosphamide), and select chemotherapeutic agents, such as proteasome inhibitors. Large, multicenter trials are needed to confirm and identify additional risk factors for ES.
There are similar occurrence rates of ES between children and adults, and they also share a similar presentation. However, there is a higher nonrelapse mortality rate in children with ES.
T86.5 – Complications of stem cell transplant
426768001 – Engraftment syndrome
Differential Diagnosis & Pitfalls
- Sepsis – Diagnostic workup will reveal an infectious source for fever.
- Drug rash – There will be a culprit drug that can be identified on history. Additionally, fever may not be present, and pulmonary edema and end-organ dysfunction would not be expected.
- Acute graft-versus-host disease (GVHD) – Often, acute GVHD does not start within 1-4 days of neutrophil recovery, instead starting a couple weeks later. Additionally, acute GVHD is not associated with pulmonary edema and typically persists, whereas ES resolves.