Objective: To investigate the expression and clinical significance of soluble B7-H3 (sB7-H3) in patients with secondary hemophagocytic lymphohistiocytosis (sHLH).
Methods: The plasma samples of 85 newly diagnosed sHLH patients from December 2012 to April 2018 were collected. The patients were divided into lymphoma-related HLH(LHLH)group and infection-related HLH(IHLH)group. The expression of sB7-H3 in plasma was detected by ELISA, and the clinical data were collected for analysis. Fifteen healthy people were chosen as control group.
Results: The expression level of sB7-H3 in lymphoma-related HLH and infection-related HLH group significant increased as compared with the control group, (P<0.05), and the expression level of sB7-H3 in lymphoma-related HLH group was significant higher than that in infection-related HLH group [(35.75± 9.90) vs (28.70±8.95) ng/ml)] (P<0.001). There were no significant statistical difference in the expression of some clinical factors (including age, fever, splenomegaly, ANC, Plt, FIB, calcium ion, serum albumin, LDH, serum ferritin, sCD25) in lymphoma-related HLH and infection-related HLH group (P>0.05). The evaluation of expression and significance of sB7-H3 in sHLH by using ROC curve, showed that the area under ROC curve comparison of patients in lymphoma-related HLH group and infection-related HLH group was 0.718 (95% CI 0.610-0.810) (P=0.0002), and predicting the sensitivity and specificity of the lymphoma-related HLH patients were 77.36% and 59.38%, respectively. The best cut-off value of patients in sB7-H3 was 29.81 ng/ml, the overall survival time of sB7-H3 high-expression group (≥29.81 ng/ml) was significant shorter than that in low-expression group (<29.81 ng/ml) (24 vs 440 d) (P<0.001). The clinical factors affecting the survival status of sHLH patients were neutrophils, albumin, serum ferritin, serum calcium ions and sB7-H3 levels.
Conclusion: sB7-H3 associates with poor prognosis of sHLH patients, and may be involved in disease progression.
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http://dx.doi.org/10.19746/j.cnki.issn.1009-2137.2020.03.048 | DOI Listing |
J Clin Immunol
December 2024
Department of Pediatrics, Division of Pediatric Hematology Oncology and Bone Marrow Transplantation, King Hussein Cancer Center, 202 Queen Rania Street, Amman, 11941, Jordan.
Inborn errors of immunity (IEI) are a heterogenous group of rare monogenic disorders that affect innate or adaptive immunity, resulting in susceptibility to life-threatening infections and autoimmunity. Allogeneic hematopoietic cell transplantation (HCT) is a valuable curative option for children with IEI. We conducted a retrospective single-center study on the outcome of HCT in children with IEI.
View Article and Find Full Text PDFFront Endocrinol (Lausanne)
December 2024
The Fertility Clinic, Skive Regional Hospital, Skive, Department of Clinical Medicine, Aarhus University, Skive, Denmark.
Background: In a global effort to assess expert perspectives on the use of recombinant gonadotropins, recombinant human luteinizing hormone (r-hLH) and recombinant human follicle-stimulating hormone (r-hFSH), a consensus meeting was held in Dubai. The key aim was to address three critical questions: What are the factors that influence follicle response to gonadotropins? Which categories of patients are most likely to benefit from LH supplementation? And what are the optimal management strategies for these patients?
Methods: A panel of thirty-six experts reviewed and refined the initial statements and references proposed by the Scientific Coordinator. Consensus was defined as agreement or disagreement by more than two-thirds (66%) of the panel members for each statement.
Cureus
November 2024
Internal Medicine and Infectiology, Universitair Ziekenhuis Brussel, Brussels, BEL.
A 53-year-old woman was hospitalized due to a fever of unknown origin for three weeks. Given the presence of fever and fatigue, the laboratory findings, and a bone marrow biopsy confirming hemophagocytic lymphohistiocytosis (HLH), a hematological malignancy was suspected. Peripheral lymphocytic typing, bone marrow biopsy, and imaging could not identify an underlying cause of HLH.
View Article and Find Full Text PDFInt J Hematol
December 2024
Children's Cancer Center, National Center for Child Health and Development, Tokyo, Japan.
Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) has a wide range of clinical presentations and is sometimes life-threatening. It is often treated with systemic corticosteroids and etoposide, but no optimal treatment has been identified. Dexamethasone palmitate (DP) contains a combination of dexamethasone and a lipid emulsion and is selectively taken up by activated macrophages.
View Article and Find Full Text PDFJ Clin Immunol
November 2024
Paediatric Immunology Department, Great Ormond Street Hospital for Children National Health Service (NHS) Foundation Trust, London, UK.
Griscelli syndrome type 2 (GS2) is a rare, life-threatening immunodysregulatory disorder characterised by impaired cytotoxic activity leading to susceptibility to haemophagocytic lymphohistiocytosis (HLH) and hypopigmentation. We completed a literature review and analysis of clinical data of 149 patients with GS2 including 8 new patients.We identified three founder mutations which show diverse phenotypic profiles (RAB27A c.
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