Diagnostic challenges of hemophagocytic lymphohistiocytosis in child with multiorgan dysfunction in a low-resource...

and importance: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome as a result of dysregulation of the immune system. Physicians in the intensive care unit (ICU), especially pediatricians, need to know how to recognize the diagnostic criteria and spectrum of HLH clinical presentations because early detection and timing of initial therapy affect the survival rate of the patient.

A 7-year-old female patient was referred to the pediatric ICU (PICU) at our tertiary hospital because of the suspicion of severe sepsis with shock and disseminated intravascular coagulation. On the fifth day of treatment, the patient was intubated and given a mechanical ventilator after experiencing respiratory failure. On the seventh day in the PICU, high fever persisted and the patient developed worsening acute kidney injury with oliguria. When worsening conditions continued, the patient experienced hypotension and cardiac arrest. The patient died on the 8th day of treatment at PICU due to severe shock and multiorgan failure.

HLH complications can be life-threatening with documented ICU mortality of 35%, even with an appropriate initial therapeutic approach. Patients with HLH can have rapid progression of disease and often require a significant amount of ICU supportive care, including vasopressor support, significant amount of blood products, ventilator support, and renal replacement therapy.

The diagnosis of HLH should be considered if there are cases with persistent, prolonged fever, organomegaly, cytopenias and evolving Multi Organ Dysfunction Syndrome. It is important for pediatricians to know the diagnostic criteria and possible clinical presentations of HLH.