Diagnosing and treating hemophagocytic lymphohistiocytosis in the tropics: systematic review from the Indian subcontinent.

Hemophagocytic lymphohistiocytosis (HLH) is a catastrophic syndrome of unrestrained immune activation. Evaluation and management of HLH in the tropics is challenging. To examine the reported etiologies and management of HLH reported from the sub-continent. Systematic review of all published cases from the Indian sub-continent. We found only 156 published cases of HLH from the sub-continent. HLH was reported from the immediate perinatal period to 46 years of age. Infection-associated HLH (IAHS) constituted 46.8% of all cases of HLH (44% and 51% in children and adults respectively). In adults, tropical infections triggered 51% of these cases of IAHS. Steroids were used in 47% of children and 10% of adults. Etoposide and/or cyclosporine were used in 8% children and 8% of adults only. Intravenous immunoglobulin was used in another 30% of children and 4% of the adults. HLH-related mortality occurred in 31.8% and 28% of children and adults respectively. HLH is under-reported in the sub-continent and has high mortality. Cyclosporine and etoposide are seldom administered early despite diagnosis of HLH. Larger cohorts with IAHS triggered by tropical infections are urgently needed to understand its natural history and implications of this differing prescription pattern on mortality.

Rajagopala S ，Singh N 《-》

Experience with hemophagocytic lymphohistiocytosis/macrophage activation syndrome at a single institution.

Gupta AA ，Tyrrell P ，Valani R ，Benseler S ，Abdelhaleem M ，Weitzman S ... -  《-》

Profile of hemophagocytic lymphohistiocytosis; efficacy of intravenous immunoglobulin therapy.

Rajajee S ，Ashok I ，Manwani N ，Rajkumar J ，Gowrishankar K ，Subbiah E ... -  《-》

Hyperferritinemia in the critically ill child with secondary hemophagocytic lymphohistiocytosis/sepsis/multiple organ dysfunction syndrome/macrophage activation syndrome: what is the treatment?

Demirkol D ，Yildizdas D ，Bayrakci B ，Karapinar B ，Kendirli T ，Koroglu TF ，Dursun O ，Erkek N ，Gedik H ，Citak A ，Kesici S ，Karabocuoglu M ，Carcillo JA ，Turkish Secondary HLH/MAS Critical Care Study Group ... -  《CRITICAL CARE》

Hemophagocytic lymphohistiocytosis associated with acute otitis media: A case report.

Hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening syndrome for which early recognition and treatment are essential for improving outcomes. HLH is characterized by uncontrolled immune activation leading to fever, cytopenias, hepatosplenomegaly, coagulation abnormalities, and elevated typical markers. This condition can be genetic or secondary, with the latter often triggered by infections. Here, we present a unique case of HLH secondary to acute otitis media (AOM), a common ear infection. We describe a 4-year-old boy who initially presented with a high fever and otalgia, later diagnosed with bilateral AOM. Despite antibiotic treatment, his condition deteriorated. The patient fulfilled diagnostic criteria for HLH. Aggressive treatment by using combination therapy with immunoglobulins, intravenous steroids (dexamethasone), cyclosporine, and etoposide was performed. After 1 month of treatment, improvement in the otologic symptoms was observed, and hematological findings gradually improved and normalized. The link between AOM and HLH may be associated with inflammatory responses and immunological mechanisms, highlighting the importance of considering HLH in severe infection cases. This case emphasizes the need for prompt diagnosis and management, especially in secondary HLH scenarios, to improve patient outcomes. It is imperative to be aware of the potential correlation between these 2 conditions, and healthcare professionals should consider the likelihood of HLH.

Chung DH ，Lee KY ，Kim JY ，Jung DJ ... -  《-》