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Objective:To study the morphology of hemophagocytosis (HPC) in bone marrow smears of patients with infection-associated hemophagocytic lymphohistiocytosis (IAHLH), and further analyse if there were differences in the clinical and laboratory features, the cytokines level and prognosisMethods:24 patients newly diagnosed with IAHLH from 2016-Dec-1 to 2021-Dec-31 in Beijing Friendship Hospital were included as study group, and 20 patients with infectious disease as non-HLH control group. In IAHLH group, mean age was 34±13 years, including 17(71%) males and7(29%) females. In Non-HLH group, mean agewas 43±16 years, including 14 (70%) males and6 (30%) females. Depending on re-checking phagocytic cell type on the initial bone marrow smear, the HPCs were divided into HPC-1, phagocytizing non-nucleated cells (mature erythrocyte or platelets), and HPC-2, phagocytizing nucleated cells. The differences in clinical presentations covered in HLH-2004 criteria, cytokines value(IL-6, IL-10, IL-18, IFN-γ) recommended in HLH-2022-China guideline, and the mortality within 1 year of diagnosis, were compared between IAHLH and non-HLH groups, between patients with or without HPC, and between patients with HPC-2 or only with HPC-1. For categorical variables, two groups were compared with the use of either the chi-square test or Fisher′s exact test. For non-normal distribution continuous variables, the difference between two groups variation was performed by using Mann-Whitney U test, and for normal distribution continuous variables, the difference was by the Independent Samples t-test.Results:The positive rates of fever, hepatomegaly and splenomegalyand the motrtality in IAHLH were 100% (24/24), 63% (15/24), 92% (22/24) and 46% (11/24), respectivelyin non-HLH were 55%(11/20),0(0/20),25% (5/20),0(0/20),and the differences between two groups were all statistically significant( P<0.01), but thedifferences between groups with and without HPC and between IAHLH patients with HPC-2 or only with HPC-1 were no statistically significanlly, ( P>0.05).In IAHLH group, IFN-γ in patients with HPC-2 was 400(246, 532)ng/L, significantly higher than 146(38, 180)ng/L in patients only with HPC-1 [ P=0.02, 95% CI was 233(75.8 to 397)], andthe other test parameters and cytokines level showed no obvious differences ( P>0.05).
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Objective:To analyze the clinical manifestations, laboratory examination characteristics and treatment of secondary hemophagocytic lymphohistiocytosis (HLH) in children with different etiologies, and to further explore the stratified diagnosis and treatment of pediatric secondary HLH, in an attempt to improve pediatricians′ understanding of this disease.Methods:The clinical data of 5 children with secondary HLH treated at Beijing Children′s Hospital, Capital Medical University from January 2018 to December 2019 were retrospectively analyzed, to explore the diagnosis and subsequent treatment of the primary HLH.Results:Five patients (including 3 boys and 2 girls) had a median onset age of 1.5 (0.5-12.7) years.At the early stage of the disease, all patients had fever and hemocytopenia.After admission, HLH-related examinations were performed and secondary HLH was diagnosed.The underlying primary diseases were lymphoma, hepatitis-associated aplastic anemia, Langerhans cell histiocytosis, systemic autoinflammatory disease and hereditary metabolic disease.Except for controlling HLH activation, the treatment of primary disease was also of great significance.Conclusions:Pediatric HLH is a group of syndrome with complex underlying diseases.Therefore, pediatricians should pay more attention to the stratified diagnosis of underlying primary diseases of pediatric secondary HLH.Related laboratory tests should be performed as soon as possible to determine the etiology after HLH is diagnosed.
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The term hemophagocytosis describes the pathologic finding of activated macrophages, engulfing erythrocytes, leukocytes, platelets and their precursor cells. This phenomenon is an important finding in patients with hemophagocytic syndrome, more properly referred to as hemophagocytic lymphohistiocytosis (HLH). HLH is a distinct clinical entity characterized by fever, pancytopenia, splenomegaly and hemophagocytosis in bone marrow, liver or lymph nodes. It has been associated with a variety of viral, bacterial, fungal and parasitic infections, as well as collagen-vascular diseases and malignancies and is uniformly fatal if left untreated. We report Staphylococcal aureus-induced hemophagocytic lymphohistiocytosis in a 3-month-old girl presenting with respiratory distress, sepsis and multiorgan failure. This case report may at least in part guide pediatricians and other physicians to recognize this rare entity of infection triggering fatal HLH and thus proper treatment may be instituted in those affected with this disease at the earliest.