Extracorporeal membrane oxygenation outcomes in children with hemophagocytic lymphohistiocytosis

Cashen, Katherine; Chu, Roland; Klein, Jessica; Rycus, Peter; Costello, John M.

doi:10.1177/0267659116667804

Cited by 12 publications

(25 citation statements)

References 18 publications

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“…Initiation of ECLS may allow time for immunosuppressive therapies to be effective. A recent study evaluating the survival of pediatric HLH patients on ECLS showed a survival rate of only 30% [94]. This is significantly lower than the general pediatric ECLS survival of 59% but is comparable to other immunocompromised pediatric cohort survival (31%) and neonatal cardiac and E-CPR (extracorporeal cardiopulmonary resuscitation) patients (38%) [94].…”

Section: Intensive Care Presentation and Managementmentioning

confidence: 88%

“…A recent study evaluating the survival of pediatric HLH patients on ECLS showed a survival rate of only 30% [94]. This is significantly lower than the general pediatric ECLS survival of 59% but is comparable to other immunocompromised pediatric cohort survival (31%) and neonatal cardiac and E-CPR (extracorporeal cardiopulmonary resuscitation) patients (38%) [94]. Indication for ECLS included respiratory failure, cardiac failure, and E-CPR with 63% being placed on venoarterial ECLS [94].…”

Section: Intensive Care Presentation and Managementmentioning

confidence: 89%

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Diagnosis, Treatment, and Management of Hemophagocytic Lymphohistiocytosis in the Critical Care Unit

Hines

Bhatt

Talano

2019

Critical Care of the Pediatric Immunocompromised Hematology/Oncology Patient

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Section: Intensive Care Presentation and Managementmentioning

confidence: 88%

Section: Intensive Care Presentation and Managementmentioning

confidence: 89%

Diagnosis, Treatment, and Management of Hemophagocytic Lymphohistiocytosis in the Critical Care Unit

Hines

Bhatt

Talano

2019

Critical Care of the Pediatric Immunocompromised Hematology/Oncology Patient

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“…In our patient, ECMO was initiated due to the development of bilateral massive pneumothorax. However, in a recent study on a small cohort of patients, survival of pediatric HLH patients on ECMO was worse than patients without HLH on ECMO ( 37 ). It should be noted that the severity of the clinical manifestations and the high mortality related to the lung involvement may themselves lead to a reduced estimation of the incidence of the lung involvement in FHL, since the patient may die without receiving a genetic diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Two Brothers with Atypical UNC13D-Related Hemophagocytic Lymphohistiocytosis Characterized by Massive Lung and Brain Involvement

et al. 2017

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Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyperinflammatory condition. Variants in different genes have been associated with the familial forms of the syndrome (FHL), usually presenting within the first 2 years of life. Due to increasing awareness of the signs and symptoms of HLH and a better understanding of the genetic basis of the disease, FHL has been increasingly diagnosed in patients presenting beyond infancy. Here, we report on two brothers with atypical, late-onset HLH in which whole exome sequencing revealed a homozygous pathogenic UNC13D variant. In the first brother, the clinical phenotype was dominated by a massive lung involvement. In the second brother a progressive neurological deterioration was observed. In both cases, the clinical manifestations at symptom onset were misleading, making the diagnosis difficult to achieve. This report expands the spectrum of clinical presentations of FLH3. Moreover, it highlights the importance to warn clinicians to keep a high level of suspicion in patients presenting with fever, cytopenia, splenomegaly of unknown origin, and unresponsiveness to conventional treatment even beyond early childhood. Moreover, this report emphasizes that insidious neurologic symptoms may represent the initial or sole presenting sign of FHL, even in the absence of peripheral signs of activation.

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“…In a case series describing 30 pediatric patients with HLH on ECMO, it was demonstrated that pediatric HLH patients on ECMO had worse survival than pediatric patients without HLH on ECMO (27% in HLH patients vs. 63% in non-HLH patients) [ 11 ]. This may be attributed to the fact that ECMO is a potent immunostimulator in and of itself.…”

Section: Discussionmentioning

confidence: 99%

Extracorporeal Membrane Oxygenation for Hemophagocytic Lymphohistiocytosis

et al. 2016

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Patient: Male, 21Final Diagnosis: Hemophagocytic LymphohistiocytosisSymptoms: Acute respiratory insufficiency • anemia • thrombocytopeniaMedication: —Clinical Procedure: Extracorporeal membrane oxygenationSpecialty: HematologyObjective:Rare diseaseBackground:Hemophagocytic lymphohistiocytosis (HLH) is a rare hematological disease characterized by an excessive inflammatory response to various triggers, resulting in rapid multi-organ failure. Its incidence may be underestimated due to its rarity, its variable clinical presentation, and its high mortality rate prior to diagnosis. Oftentimes, HLH is mistaken for refractory sepsis and improperly treated as such. Left untreated, the disease is universally fatal. With treatment, case series of adults with HLH report a 30-day mortality of up to 44% and an overall mortality of up to 75%.Case Report:We describe the use of extracorporeal membrane oxygenation (ECMO) in a previously healthy young man with HLH and acute respiratory distress syndrome (ARDS), a common sequela of HLH. ECMO was employed to provide temporary hemodynamic support, allowing for recovery of pulmonary function compromised during the initial cytokine storm. Additionally, and perhaps more importantly, implementation of ECMO provided the time necessary for the eventual diagnosis and treatment of HLH.Conclusions:Although limited case reports and case series suggest that the use of ECMO in pediatric patients with HLH is associated with high mortality, our experience suggests that ECMO should not be rejected as a supportive modality in adults with HLH who have potentially recoverable cardiopulmonary function. We believe that ECMO may be appropriately instituted in select patients with HLH, or in rapidly deteriorating patients with an unknown illness refractory to conventional therapy, to allow for end-organ recovery, to reach a diagnosis, and to administer appropriate therapy.

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Extracorporeal membrane oxygenation outcomes in children with hemophagocytic lymphohistiocytosis

Abstract: HLH is an uncommon indication for ECMO and these patients have increased mortality compared to the overall pediatric ECMO population. These data should be factored into decision-making when considering ECMO for pediatric HLH patients.

Cited by 12 publications

References 18 publications

Diagnosis, Treatment, and Management of Hemophagocytic Lymphohistiocytosis in the Critical Care Unit

Diagnosis, Treatment, and Management of Hemophagocytic Lymphohistiocytosis in the Critical Care Unit

Two Brothers with Atypical UNC13D-Related Hemophagocytic Lymphohistiocytosis Characterized by Massive Lung and Brain Involvement

Extracorporeal Membrane Oxygenation for Hemophagocytic Lymphohistiocytosis

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