Analysis of clinical data of 3 cases of hemophagocytic syndrome and literature review / 中国基层医药

ABSTRACT

Objective:To analyze the clinical characteristics, diagnosis, treatment and prognosis of three children with hemophagocytic syndrome (HPS) and provide diagnosis and treatment clues for reasonable treatment and prevention of serious complications and reduction of misdiagnosis and mistreatment.Methods:The clinical data of three children with HPS who received treatment in Taiyuan Maternal and Child Health Care Hospital from March 2018 to March 2020 were retrospectively analyzed. Related literature was retrieved. The clinical data of the three children were summarized to analyze the outcomes.Results:Fever (≥ 39 ℃) was the first symptom in all three cases. In the end, red blood cell line, white blood cell line, and platelet line were reduced to different degrees in all three cases. Hepatosplenomegaly was found in two cases. Transaminase highly increased in two cases, and slightly increased in one case. Ferritin greatly increased, fibrinogen decreased, and hypertriglyceridemia did not occur in all three cases. Two cases had skin rash during fever. Skin rash appeared late in one of them.All three cases had different degrees of lymphadenopathy. Bone puncture examination showed reticulocyte phagocytosis in bone marrow in one case and leishmania in bone marrow smear in another case. These two cases were cured and discharged. One case died of multiple organ failure due to rapid disease progression, and adrenal masses were found at autopsy.Conclusion:HPS has diverse clinical manifestations, complex etiology and different clinical prognoses. HPS should be considered in case of unexplained fever with hemocytopenia. The medical history and living history should be inquired in detail and relevant auxiliary examinations should be improved as soon as possible.