The cardinal manifestations of HLH are prolonged fever, pancytopenia and hepatosplenomegaly.
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The full complex of HLH symptoms reflects an inability to control inflammatory responses. 5 This systemic infiltration can explain the vast array of clinical manifestations.Īlbeit rare, HLH is a potentially fatal condition. The main histopathological characteristic of HLH is the infiltration of lymphocytes and histiocytes with hemophagocytic activity observed in the reticuloendothelial system, bone marrow and central nervous system. 1, 2 The acquired form is more frequent than the familial forms and it is a result of disrupted function of the immune system, as a consequence of severe infection, immunodeficiency or malignancy. The familial form is rare and it underlies eight autosomal recessive gene defects that encode proteins related to the exocytosis of cytotoxic granules during apoptosis in natural killer (NK) cells. HLH comprises two different groups based on various inherited or acquired immune deficiencies. Hemophagocytic lymphohistiocytosis (HLH), also known as hemophagocytic syndrome, is a condition in which there is an inappropriate immune system activation.
In conclusion, HLH is a rare disease with a high mortality rate, especially in patients with disease reactivation and those with familial- or immunodeficiency-associated forms, which makes early recognition and genetic testing crucial for appropriate management and prompt SCT indication. These patients demonstrated an estimated 5-year OS of 25%, while all patients that did not reactivate were alive until the end of the follow-up. We observed a significant difference in prognosis associated with reactivation of HLH. The 5-year overall survival (OS) was 36.7%. The mortality rate was 57.1% and the mean time between diagnosis and death was 9.98 months. Fifteen (71.4%) patients presented resolution criteria and eight (53.3%) of them presented reactivation.
All patients were admitted to the intensive care unit (ICU) at some point. HLH mainly affected females, fever was the most frequent clinical sign and hyperferritinemia was the most prevalent laboratory abnormality. In this context, we described clinical and laboratory characteristics, therapeutic modality and outcome of 21 patients with HLH treated at a pediatric oncology hospital between January 2000 and February 2019.
Hemophagocytic lymphohistiocytosis comprises a systemic hyperactivation of macrophages that requires prompt recognition of symptoms and early treatment.
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