Treatment of secondary hemophagocytic syndrome

Introduction. Hemophagocytic syndrome (HPS) is a reaction of severe, excessive, but ineffective inflammation. HPS is divided into primary or as a complication of a different causes — secondary HPS (sHPS).

Aim: to analyze the effi cacy of different treatments in sHPS patients.

Materials and methods. For the retrospective analysis, the medical documentation of patients who were treated in the period from June 2009 to January 2023 was used. The H-Score and HLH-2004 criteria were used to verify sHPS. The results of clinical blood analysis and biochemical tests are presented. The survival was analyzed within two weeks after the verification of sHPS. The main treatment options for sHPS were etoposide, glucocorticosteroids (GCSs), anticancer therapy and intravenous immunoglobulin.

Results. The study included data from 130 patients, median age 56 years (18–90); 70 females and 60 males with sHPS. All patients received treatment with a drug change in cases of inefficiency: a total of 186 episodes. A stable response was achieved in 74 (56.9 %) patients. The median survival in patients without a response was 2 days. If the therapy was effective, the median survival was not reached. Positive dynamics were observed during the first day after the start of effective treatment, however, a few patients had transient worsening of some markers. The main factor in the negative prognosis was the degree of multiple organ failure during sHPS verification. In the group of patients with autoimmune diseases, GCSs were the most effective, with a response reached in 75 % of cases. For patients with resistance, as well as in patients with Epstein—Barr virus infection and blood malignancy, etoposide proved to be effective in 65.7 % of cases.

Conclusion. sHPS was accompanied by an increase in pancytopenia, cytolytic, cholestatic syndromes, hypocoagulation, azotemia, hypertriglyceridemia and excessive hyperferritinemia. After the initiation of effective therapy, persistent clinical and laboratory responses developed during the first day. Therapy by GCSs was effective in most patients with autoimmune diseases associated with sHPS. With other forms of sHPS in the studied group, etoposide had the most pronounced effect.

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