Myocardial dysfunction is highly prevalent in PSS but is not associated with mortality. LV systolic dysfunction is associated with a higher severity of illness, use of vasoactives, and BNP, whereas RV systolic dysfunction is associated with cold shock. Further studies are needed to determine the utility of echo in the bedside management of patients with PSS.
Ehrlichiosis, caused by transmission of Ehrlichia chaffeensis to humans through the bite of an infected lone star tick, can lead to secondary hemophagocytic lymphohistiocytosis (HLH), a life-threatening condition caused by uncontrolled activation of the cellular immune system. We describe a child with HLH secondary to ehrlichiosis who developed multiorgan failure and was successfully managed with extracorporeal membrane oxygenation (ECMO). A 9-year-old boy developed headaches, fever, and sore throat after suspected tick exposure. He presented with pancytopenia, elevated ferritin, and soluble interleukin-2 receptor levels, all consistent with HLH. Bone marrow biopsy revealed hemophagocytosis. Polymerase chain reaction was positive for E chaffeensis He developed acute kidney injury, coagulation failure, hepatic insufficiency, and progressive respiratory failure requiring intubation. Due to refractory hypoxemia, he was cannulated for veno-venous ECMO. Continuous veno-venous hemofiltration was used to manage acute kidney injury and fluid overload. He received doxycycline and dexamethasone/etoposide for treatment of ehrlichiosis and HLH, respectively. Plasma exchange was used for thrombocytopenia-associated multiple organ failure. The patient was decannulated after 140 hours of ECMO and subsequently transferred for inpatient rehabilitation after extubation. Review of the Extracorporeal Life Support Organization Registry database identified 6 patients with tickborne diseases who received ECMO for organ support (survival in 3 of 6); ehrlichiosis was not reported in any of these cases. ECMO likely allowed a platform for stabilization and additional therapeutic interventions in this patient.
Respiratory failure associated with hematopoietic stem cell transplantation (HSCT) has been considered a contraindication for use of extracorporeal membrane oxygenation (ECMO) at many centers. We describe a child with neuroblastoma and hypoxemic respiratory failure following HSCT who was successfully managed with veno-venous (VV) ECMO. The patient was an 18-month-old female with high-risk neuroblastoma status post tumor resection, chemotherapy, autologous HSCT, and primary site radiation. On day 113 posttransplant while receiving maintenance immunotherapy, she had an acute respiratory decompensation because of rhinovirus, aspiration pneumonia, and capillary leak syndrome. The patient was intubated and transitioned to a high frequency oscillatory ventilation and inhaled nitric oxide. Because of refractory hypoxemia, she was cannulated for VV ECMO. She was weaned and decannulated after 7.5 days on ECMO, then subsequently transferred for inpatient rehabilitation. The most recent Extracorporeal Life Support Organization registry analysis showed low survival (3/29) in patients requiring ECMO after HSCT, and 2 of 3 survivors had nononcological diagnoses. However, our patient’s outcome suggests that HSCT status should not be an absolute contraindication. The presence of a reversible single organ failure and the absence of significant bleeding risk in an engrafted, neurologically intact, and non-neutropenic HSCT patient with a favorable prognosis can support the potential benefit of ECMO.
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