1.Coagulation profile PT, FBG, FDP, D-D as disease predictors of RA and pSS inflammatory immunity.
Wenwen MIN ; Lei WAN ; Feng LI ; Yu ZHANG ; Ying WANG ; Siyu LIANG
Chinese Journal of Cellular and Molecular Immunology 2025;41(10):895-904
Objective To explore the expression of coagulation indexes in rheumatoid arthritis (RA) and dry syndrome (pSS) and their relationships with inflammation and immune function. Methods A total of 61 patients with RA who were hospitalized in the Department of Rheumatology of Anhui Provincial Hospital of Traditional Chinese Medicine from March 12 to September 9, 2024 were selected as the RA group. And 61 patients with pSS who were hospitalized in the Department of Rheumatology of the same hospital September 4, 2023, to August 17, 2024, were selected as the pSS group. 61 healthy individuals who underwent routine medical checkups at the Physical Examination Center of Anhui Provincial Hospital of Traditional Chinese Medicine during the same period were included as the control group. Baseline clinical indexes before treatment were collected from patients in each group, including prothrombin time(PT), international normalized ratio(INR), thrombia time(TT), fibrinogen(FBG), activated partial thromboplastin time(APTT), fibrin (ogen) degradation products(FDP) and D-Dimer(D-D). Results The expression levels of PT, FBG, TT, FDP, and D-D in the RA group, the pSS group, and the normal group were significantly different. The expression levels of PT, FBG, FDP, and D-D in the RA group were all higher than those in the pSS group and the control group, respectively. And the expression level of TT in the pSS group was lower than that in control group. ROC curve analysis showed that the AUC of PT was 0.638, the AUC of FBG was 0.899, the AUC of FDP was 0.866, and the AUC of D-D was 0.919 in the RA group compared with the normal group. And the AUC of coagulation indexes for joint diagnosis of RA was higher than that of the indexes detected individually. pSS group had an AUC of PT of 0.618 compared with that of the normal group. The AUC of TT was 0.645, and the AUC of coagulation indexes for the joint diagnosis of pSS was higher than the AUC of each index detected separately. Association rule analysis showed that elevated D-D in RA patients had a significant correlation with elevated hs-CRP, CCP and RF, and elevated FBG had a significant correlation with elevated hs-CRP, ESR, RF and CCP. Elevated D-D in pSS patients had a correlation with elevated hs-CRP and anti-SSA, and elevated INR has correlation with elevated hs-CRP, anti-SSA and anti-SSB. Correlation analysis showed that PT, INR, FBG, FDP, and D-D were positively correlated with CRP and ESR, and TT was negatively correlated with CRP and ESR in the RA group. FBG, FDP, and D-D were positively correlated with CRP and ESR in the pSS group. Moreover, coagulation indexes were positively correlated with immune indexes in RA group and pSS group which were all significant. The results of multiple linear regression analysis showed that FBG was a positive correlate of hs-CRP and ESR in RA patients. For PSS patients, FBG and FDP were positive correlates of hs-CRP. APTT and FBG were positive correlates of ESR. Conclusion Compared with pSS, coagulation indexes (especially PT, FBG, FDP and D-D) are more informative for the early diagnosis of RA and the judgment of the degree of the disease, and can be used as an important predictor for the confirmation of the diagnosis of RA.
Humans
;
Female
;
Male
;
Arthritis, Rheumatoid/diagnosis*
;
Middle Aged
;
Fibrin Fibrinogen Degradation Products/analysis*
;
Blood Coagulation
;
Adult
;
Fibrinogen/metabolism*
;
Partial Thromboplastin Time
;
Prothrombin Time
;
Aged
;
Inflammation/immunology*
;
ROC Curve
2.Application value of thromboelastography in assessing coagulation function in children with severe hemophilia A after emicizumab therapy: a single-center study.
Dong PENG ; Ying WANG ; Gui-Chi ZHOU ; Qian LI ; Mei-Zhu LUO ; Li-Ping LUO ; Ya-Xian KUANG ; Xiao-Ying FU
Chinese Journal of Contemporary Pediatrics 2025;27(3):293-299
OBJECTIVES:
To investigate the application value of thromboelastography (TEG) in assessing coagulation function in children with severe hemophilia A (HA) after emicizumab (EMI) therapy.
METHODS:
A retrospective analysis was performed on the activated partial thromboplastin time (APTT) and TEG testing results of 17 children with severe HA before and after EMI treatment at Shenzhen Children's Hospital from January 2023 to July 2024. Correlation analysis was conducted between coagulation factor VIII (FVIII) equivalent activity and reaction time (R value) measured by TEG.
RESULTS:
After EMI treatment, the mean bleeding rate for children with severe HA was 1.6 events per year, with 15 children (88%) without spontaneous bleeding or joint bleeding. The children with severe HA showed a significant reduction in APTT after EMI treatment (P<0.05), with a significantly shorter APTT than the normal control group (P<0.05). There was no correlation between APTT and FVIII equivalent activity after treatment (P>0.05). After EMI treatment, TEG parameters, including R value, kinetic time, alpha angle (α), maximum amplitude, clot strength, and coagulation index, shifted from a hypocoagulable state before treatment to a nearly normal state after treatment (P<0.05). The R value demonstrated a strong negative correlation with FVIII equivalent activity (r=-0.758, P<0.05).
CONCLUSIONS
The bleeding condition of children with severe HA can be effectively controlled after EMI treatment. Routine APTT testing cannot reflect true coagulation function, whereas TEG testing is clinically valuable in assessing the coagulation function of children with severe HA undergoing EMI treatment.
Humans
;
Thrombelastography
;
Hemophilia A/physiopathology*
;
Male
;
Child
;
Antibodies, Bispecific/therapeutic use*
;
Antibodies, Monoclonal, Humanized/therapeutic use*
;
Blood Coagulation/drug effects*
;
Child, Preschool
;
Retrospective Studies
;
Female
;
Partial Thromboplastin Time
;
Adolescent
;
Infant
3.The Effect of p.Thr241Asn and p.Cys389Gly Mutations on Coagulation Factor VII Structure and Function.
Journal of Experimental Hematology 2025;33(3):853-857
OBJECTIVE:
To identify F7 gene mutations in one pedigree with congenital coagulation factor VII (FVII) deficiency and explore the effect of F7 gene mutations on the structure and function of FVII.
METHODS:
Prothrombin time (PT) and activated partial thromboplastin time (APTT) were measured based on the one-stage assay, and PT-based one stage assay was used to detect the activity of FII, V, VII and X. Genomic DNA was extracted from the peripheral blood of family members. The sequences of all the exons and exon-intron boundaries of F7 were amplified by polymerase chain reaction (PCR) using specific primers followed by Sanger sequencing. PolyPhen-2, PROVEAN and Swiss-Pdb Viewer software were used to analyze the effect of mutations on the structure and function of FVII.
RESULTS:
The proband had a prolonged PT (33.8 s) due to low FVII activity (6.6%) and normal APTT, and had a history of epistaxis. The proband's mother and father displayed a slightly prolonged PT (13.2 and 13.9 s, respectively), and their FVII activity was 40.3% and 38.3%, respectively. The compound heterozygous c.722C>A (p.Thr241Asn) in exon 7 and c.1165T>G (p.Cys389Gly) in exon 8 of F7 gene were identified in the proband, and inherited from his father and mother, respectively. The p.Thr241Asn and p.Cys389Gly missense change were likely to have a damaging effect predicted by polyphen-2 and PROVEAN software. In silico modeling analysis showed that there was one hydrogen bond formed between wild-type Thr241 and Val249, two hydrogen bonds formed between mutant Asn241 and Val249 and between mutant Asn241 and Leu242, as well as one hydrogen bond and one disulfide bond formed between wild-type Cys389 and Leu370 and between wild-type Cys389 and Cys375, respectively. The hydrogen bond formed between mutant Gly389 and Leu370 and disulfide bond formed between mutant Gly389 and Cys375 both broke.
CONCLUSIONS
FVII deficiency in this family is caused by p.Thr241Asn and p.Cys389Gly mutation. In silico modeling may be a valuable tool for understanding amino acid residues from variants leading to congenital FVII deficiency.
Humans
;
Factor VII/genetics*
;
Mutation
;
Pedigree
;
Factor VII Deficiency/genetics*
;
Prothrombin Time
;
Partial Thromboplastin Time
;
Male
;
Female
4.Application value of pediatric sepsis-induced coagulopathy score and mean platelet volume/platelet count ratio in children with sepsis.
Jie HAN ; Xifeng ZHANG ; Zhenying WANG ; Guixia XU
Chinese Critical Care Medicine 2025;37(4):361-366
OBJECTIVE:
To investigate the application value of pediatric sepsis-induced coagulation (pSIC) score and mean platelet volume/platelet count (MPV/PLT) ratio in the diagnosis of pediatric sepsis and the determination of critical pediatric sepsis.
METHODS:
A retrospective cohort study was conducted, selecting 112 children with sepsis (sepsis group) admitted to pediatric intensive care unit (PICU) of Liaocheng Second People's Hospital from January 2020 to December 2023 as the study objects, and 50 children without sepsis admitted to the pediatric surgery department of our hospital during the same period for elective surgery due to inguinal hernia as the control (control group). The children with sepsis were divided into two groups according to the pediatric critical case score (PCIS). The children with PCIS score of ≤ 80 were classified as critically ill group, and those with PCIS score of > 80 was classified as non-critically ill group. pSIC score, coagulation indicators [prothrombin time (PT), international normalized ratio (INR), activated partial thromboplastin time (APTT), and fibrinogen (FIB)], and platelet related indicators (PLT, MPV, and MPV/PLT ratio) were collected. Pearson correlation method was used to analyze the correlation between pSIC score and MPV/PLT ratio as well as their correlation with coagulation indicators. Multivariate Logistic regression analysis was used to screen the independent risk factors for pediatric sepsis and critical pediatric sepsis. Receiver operator characteristic curve (ROC curve) was drawn to evaluate the application value of the above independent risk factors on the diagnosis of pediatric sepsis and the determination of critical pediatric sepsis.
RESULTS:
112 children with sepsis and 50 children without sepsis were enrolled in the final analysis. pSIC score, PT, INR, APTT, FIB, MPV, and MPV/PLT ratio in the sepsis group were significantly higher than those in the control group [pSIC score: 0.93±0.10 vs. 0.06±0.03, PT (s): 14.76±0.38 vs. 12.23±0.15, INR: 1.26±0.03 vs. 1.06±0.01, APTT (s): 40.08±0.94 vs. 32.47±0.54, FIB (g/L): 3.51±0.11 vs. 2.31±0.06, MPV (fL): 8.86±0.14 vs. 7.62±0.11, MPV/PLT ratio: 0.037±0.003 vs. 0.022±0.001, all P < 0.01], and PLT was slightly lower than that in the control group (×109/L: 306.00±11.01 vs. 345.90±10.57, P > 0.05). Among 112 children with sepsis, 46 were critically ill and 66 were non-critically ill. pSIC score, PT, INR, APTT, MPV, and MPV/PLT ratio in the critically ill group were significantly higher than those in the non-critically ill group [pSIC score: 1.74±0.17 vs. 0.36±0.07, PT (s): 16.55±0.80 vs. 13.52±0.23, INR: 1.39±0.07 vs. 1.17±0.02, APTT (s): 43.83±1.72 vs. 37.77±0.95, MPV (fL): 9.31±0.23 vs. 8.55±0.16, MPV/PLT ratio: 0.051±0.006 vs. 0.027±0.001, all P < 0.05], PLT was significantly lower than that in the non-critically ill group (×109/L: 260.50±18.89 vs. 337.70±11.90, P < 0.01), and FIB was slightly lower than that in the non-critically ill group (g/L: 3.28±0.19 vs. 3.67±0.14, P > 0.05). Correlation analysis showed that pSIC score was significantly positively correlated with MPV/PLT ratio and coagulation indicators including PT, APTT and INR in pediatric sepsis (r value was 0.583, 0.571, 0.296 and 0.518, respectively, all P < 0.01), and MPV/PLT ratio was also significantly positively correlated with PT, APTT and INR (r value was 0.300, 0.203 and 0.307, respectively, all P < 0.05). Multivariate Logistic regression analysis showed that pSIC score and MPV/PLT ratio were independent risk factors for pediatric sepsis and critical pediatric sepsis [pediatric sepsis: odds ratio (OR) and 95% confidence interval (95%CI) for pSIC score was 14.117 (4.190-47.555), and the OR value and 95%CI for MPV/PLT ratio was 1.128 (1.059-1.202), both P < 0.01; critical pediatric sepsis: the OR value and 95%CI for pSIC score was 8.142 (3.672-18.050), and the OR value and 95%CI for MPV/PLT ratio was 1.068 (1.028-1.109), all P < 0.01]. ROC curve analysis showed that pSIC score and MPV/PLT ratio had certain application value in the diagnosis of pediatric sepsis [area under the ROC curve (AUC) and 95%CI was 0.754 (0.700-0.808) and 0.720 (0.643-0.798), respectively] and the determination of critical pediatric sepsis [AUC and 95%CI was 0.849 (0.778-0.919) and 0.731 (0.632-0.830)], and the combined AUC of the two indictors was 0.815 (95%CI was 0.751-0.879) and 0.872 (95%CI was 0.806-0.938), respectively.
CONCLUSIONS
pSIC score and MPV/PLT ratio have potential application value in the diagnosis of pediatric sepsis and the determination of critical pediatric sepsis, and the combined application of both is more valuable.
Humans
;
Sepsis/complications*
;
Platelet Count
;
Mean Platelet Volume
;
Retrospective Studies
;
Child
;
Blood Coagulation Disorders/diagnosis*
;
Intensive Care Units, Pediatric
;
Male
;
Female
;
Partial Thromboplastin Time
;
Child, Preschool
;
Blood Coagulation
;
International Normalized Ratio
;
Infant
5.Quality of care among patients with acute heart failure at the emergency room and adherence of physicians at the University of the Philippines – Philippine General Hospital to the division of cardiovascular medicine – heart failure pathway:A retrospective cohort study.
Mark John D. SABANDO ; Felix Eduardo R. PUNZALAN ; Frances Dominique V. HO ; Tam Adrian P. AYA-AY ; Kevin Paul Da. ENRIQUEZ ; Marie Kirk A. MARAMARA ; Ronald Allan B. RODEROS ; Lauren Kay M. EVANGELISTA
Acta Medica Philippina 2025;60(2):22-32
OBJECTIVES
Clinical pathways (CPs) ensure adherence to heart failure (HF) management guidelines. To optimize quality care in a low resource setting, an evidence-based care pathway for the management of acute HF was implemented at the emergency department (ED) of the Philippine General Hospital (PGH), the designated national tertiary hospital and referral center. This study aimed to describe the characteristics of adults with acute HF admitted at the ED and evaluate the quality of care they received, measured using physician adherence to the hospital’s acute heart failure CP.
METHODSThis was a retrospective, descriptive cohort study. We reviewed the inpatient charts of all adult patients with acute HF admitted to the ED of the PGH and referred to the Division of Cardiovascular Medicine between December 1, 2022 and May 31, 2023. Quality of care was assessed based on adherence to quality indicators adapted from routine and conditional order sets detailed in the pathway. Descriptive statistics was utilized to describe patient characteristics, quality of care, and outcomes.
RESULTSTwo hundred thirty-six (236) patients were included, with a mean age of 51.8 years. Majority were male (53.4%); hypertension (61.4%) and ischemic heart disease (53.8%) were the most common comorbidities, and infection the most common precipitant of decompensation (60.6%). There were optimal adherence rates to routine orders, which included referrals to Internal Medicine and Cardiology, baseline vital signs monitoring, fluid intake and output monitoring, chest radiograph, complete blood count, blood urea nitrogen, sodium, potassium, prothrombin time, partial thromboplastin time, arterial blood gas, urinalysis, and N-terminal pro b-type natriuretic peptide. Conditional orders, such as oxygen support, focused echocardiography, thyroid - stimulating hormone, and the use of vasopressors, diuretics, and venous thromboembolism prophylactic agents, were optimally performed when warranted. However, we noted suboptimal adherence to certain resource-intensive conditional orders, such as hourly monitoring of urine output (61.4%), hooking to cardiac monitor (53.8%), and performance of 12-lead ECG within 10 minutes (56.8%). Further, only 43.9% of patients were referred to the intensive care unit. Troponin I, calcium, magnesium, and albumin were ordered in excess.
CONCLUSIONOverall adherence rate of physicians to the hospital’s Acute Heart Failure Pathway was satisfactory. Work is needed to improve adherence to hourly urine output monitoring, consistent hooking to cardiac monitor, and timely performance of 12-lead ECG – an effort that begins with expanding in-hospital diagnostic equipment and human resource supply. We recommend continuous pathway implementation with periodic evaluation and stakeholder feedback to further improve quality of care.
Human ; Male ; Female ; Middle Aged: 45-64 Yrs Old ; Adult ; Albumins ; Blood ; Blood Urea Nitrogen ; Calcium ; Cardiology ; Chart ; Charts ; Cohort Studies ; Critical Care ; Critical Pathways ; Diagnostic Equipment ; Disease ; Diuretics ; Echocardiography ; Electrocardiography ; Emergencies ; Emergency Service, Hospital ; Equipment And Supplies ; Evaluation Studies As Topic ; Feedback ; Heart ; Heart Diseases ; Heart Failure ; Hormones ; Hospitals ; Hospitals, General ; Humans ; Hypertension ; Indicators And Reagents ; Infection ; Infections ; Inpatients ; Intensive Care Units ; Internal Medicine ; Lead ; Magnesium ; Male ; Medicine ; Myocardial Ischemia ; Natriuretic Peptide, Brain ; Natriuretic Peptides ; Nitrogen ; Overall ; Oxygen ; Partial Thromboplastin Time ; Patients ; Peptides ; Philippines ; Physicians ; Potassium ; Prothrombin ; Prothrombin Time ; Quality Of Health Care ; Referral And Consultation ; Sodium ; Statistics ; Tertiary Care Centers ; Thorax ; Thromboembolism ; Thromboplastin ; Thyroid Gland ; Time ; Troponin ; Troponin I ; Universities ; Urea ; Urinalysis ; Urine ; Venous Thromboembolism ; Vital Signs ; Work ; Workforce
6.Coagulation and platelet profiles of COVID-19 patients admitted to a COVID Referral Center from March 2020 to December 2022
Ivana Ungajan-Galapon ; Karen Damian ; Nelson Geraldino
Philippine Journal of Pathology 2024;9(1):11-16
Objective:
This study aimed to determine the demographic profiles of admitted COVID-19 patients, the association of coagulation and platelet tests on COVID-19 severity and compare the coagulation and platelet profile across the spectrum of the disease in terms of severity among adult COVID-19 patients admitted to the Philippine General Hospital from March 2020 to December 2022.
:
Methodology. Medical records of a sample of adult COVID-19 patients admitted to the emergency room of the Philippine General Hospital from March 2020 to December 2022 were reviewed. The demographics, initial COVID-19 diagnosis and initial coagulation and platelet test results were gathered and tabulated. Comparison of the initial coagulation and initial platelet results were made per disease category.
Results:
Three hundred eighty-five (385) patients were included; 194 were males, and 191 were females. The mean age of all patients was 56.18 years old. There was a total of 30 patients classified as mild and 105 patients are under moderate category. 141 patients were classified as severe, whereas 109 patients were classified as critical. Platelet count test and Activated Partial Thromboplastin Time (APTT) were mostly normal in all disease categories. Prothrombin time was normal in a majority of patients from the mild and severe categories. INR and D-dimer were all elevated mostly in all disease categories.
Conclusion
Platelet counts and APTT were mostly normal in all disease categories. Prothrombin time and D-dimer had a significant association with disease severity. Platelet count, APTT and INR did not show significant association with disease severity. Prothrombin time, APTT, INR and D-dimer means had significant differences versus disease categories.
Partial Thromboplastin Time
;
OVID-19
;
Patient Acuity
7.Analysis of a Chinese pedigree affected with Hereditary coagulation factor Ⅺ deficiency due to variant of F11 gene.
Huanhuan WANG ; Suting JIANG ; Huinan XIA ; Lihong YANG ; Yanhui JIN ; Mingshan WANG
Chinese Journal of Medical Genetics 2023;40(11):1319-1323
OBJECTIVE:
To explore the molecular pathogenesis of a Chinese pedigree affected with Hereditary coagulation factor Ⅺ (FⅪ) deficiency due to variants of the F11 gene.
METHODS:
A male proband with Hereditary coagulation factor Ⅺ deficiency who was admitted to the First Affiliated Hospital of Wenzhou Medical University due to urinary calculi on November 30, 2020 and his family members (7 individuals from 3 generations in total) were selected as the study subjects. Clinical data of the proband were collected, and relevant coagulation indices of the proband and his family members were determined. Genomic DNA of peripheral blood samples was extracted for PCR amplification. All exons, flanking sequences, and 5' and 3' untranslated regions of the F11 gene of the proband were analyzed by direct sequencing. And the corresponding sites were subjected to sequencing in other family members. The conservation of amino acid variation sites was analyzed by bioinformatic software, and the effect of the variant on the protein function was analyzed. Variants were graded based on the guidelines from the American College of Medical Genetics and Genomics (ACMG).
RESULTS:
The proband was a 36-year-old male. His activated partial thromboplastin time (APTT) was 89.2s, which was significantly prolonged. The FⅪ activity (FⅪ:C) and FⅪ antigen (FⅪ:Ag) were 2.0% and 3.5%, respectively, which were extremely reduced. Both the proband and his sister were found to harbor compound heterozygous variants of the F11 gene, including a c.689G>T (p.Cys230Phe) missense variant in exon 7 from their father and a c.1556G>A (p.Trp519*) nonsense variant in exon 13 from their mother. Conservation analysis indicated the Cys230 site to be highly conserved. The c.1556G>A (p.Trp519*) variant was known to be pathogenic, whilst the c.689G>T variant was classified as likely pathogenic (PM2+PM5+PP1+PP3+PP4) based on the ACMG guidelines.
CONCLUSION
The c.689G>T and c.1556G>A compound heterozygous variants of the F11 gene probably underlay the pathogenesis of FⅪ deficiency in this pedigree.
Adult
;
Humans
;
Male
;
3' Untranslated Regions
;
East Asian People
;
Factor XI/genetics*
;
Factor XI Deficiency/genetics*
;
Partial Thromboplastin Time
;
Pedigree
8.Analysis of APTT Mixing Test Results in Factor Ⅷ Inhibitor-Positive Hemophilia Patients.
Li-Hua ZHANG ; Zhi-Qiang XIE ; He ZHUANG ; Mei-Hua WANG ; Ying-Ping CAO
Journal of Experimental Hematology 2023;31(6):1791-1796
OBJECTIVE:
To analyze the results of activated partial thromboplastin time (APTT) mixing test in coagulation factor Ⅷ inhibitor-positive hemophilia patients, so as to increase the value of APTT mixing test in the screen of factor Ⅷ inhibitor.
METHODS:
Eighty plasmas samples with different titers of coagulation factor Ⅷ inhibitors had been collected and diluted for routine immediate APTT mixing test and at 37 ℃ 2 hours incubation APTT mixing test. Fifteen samples were selected for immediate and normal temperature incubation for 15 min, 30min, 1 hour, 2 hours and 37 ℃ for 30 min, 1 hour, 2 hours APTT mixing test.
RESULTS:
The results of APTT mixing test were significantly correlated with the titers of coagulation factor Ⅷ inhibitors. The ROC curve result showed that the best diagnostic cut-off value for 2 hours incubation APTT mixing test at 37 ℃ to determine the presence or absence of coagulation factor Ⅷ inhibitors was 43.8 s (sensitivity and specificity was 85.90% and 100%, respectively), while the best diagnostic cut-off value for distinguishing high-titer and low-titer Ⅷ inhibitors was 52.4 s (sensitivity and specificity was 98.18% and 95.65%, respectively). The critical coagulation factor Ⅷ inhibitor titer that could not be corrected by immediate APTT was 5.14 BU/ml, while that could not be corrected by 37 ℃ 2 hours incubation APTT was 1.31 BU/ml. Paired samples t -test was performed on the APTT mixing test results at different times and temperatures, and the differences were statistically significant (P < 0.05).
CONCLUSIONS
The APTT mixing test can be used as a screening index for coagulation factor Ⅷ inhibitors. APTT mixing test result shows a significant time-temperature dependence with lower titers of coagulation factor Ⅷ inhibitor. Patients with hemophilia who cannot be corrected by immediate APTT mixing test should be alert to the possibility of high titer of coagulation factor Ⅷ.
Humans
;
Factor VIII
;
Hemophilia A/diagnosis*
;
Blood Coagulation Tests/methods*
;
Partial Thromboplastin Time
;
Blood Coagulation Factors
9.Analysis of High-Risk Clinical Factors of Early Death in Secondary Hemophagocytic Lymphohistiocytosis.
Jin-Li ZHU ; Fan WU ; Lin-Hui HU ; Zhi-Min ZHAI
Journal of Experimental Hematology 2023;31(6):1866-1871
OBJECTIVE:
To explore the high-risk clinical factors of early death in patients with secondary hemophagocytic lymphohistiocytosis (sHLH), and further identify the clinical factors related to the rapid progression of sHLH in the short term.
METHODS:
The clinical manifestations, laboratory examination and prognosis of sHLH patients were retrospectively analyzed. Continuous variables were grouped by median, univariate and multivariate Cox regression analysis and Kaplan-Meier survival curve were used to explore the risk factors affecting early death of sHLH. Then, a nomogram model was established with independent risk factors, Bootstrap resampling method was used for verification, and consistency index (C-index) and calibration curve were used to detect the prediction accuracy.
RESULTS:
A total of 126 sHLH patients were enrolled, with a median age of 48.5(16-88) years, including 74 males and 52 females. Fifty-five patients (43.6%) died within 30 days, including 39 males and 16 females. Univariate regression analysis showed that lymphocyte count <0.45×109/L, platelet count (PLT) <39.5×109/L, prothrombin time (PT)≥13.3 s, activated partial thromboplastin time (APTT)≥39.7 s, albumin (ALB) <25.9 g/L, lactate dehydrogenase (LDH)≥811 U/L, creatinine (Cr) ≥67 μmol/L and procalcitonin (PCT)≥0.61 ng/ml were risk factors for death within 30 days in sHLH patients. Multivariate regression analysis showed that lymphocyte count <0.45×109/L, APTT≥39.7 s and ALB <25.9 g/L were independent risk factors for death within 30 days in sHLH patients. A nomogram model was established based on the above three risk factors, its C-index was 0.683, and the calibration chart showed good agreement between the observed and predicted values of sHLH.
CONCLUSIONS
Lymphopenia, prolonged APTT, and hypoalbuminemia are risk factors for early death of sHLH patients. Early identification and positive intervention are expected to reduce early mortality in sHLH patients. The nomogram model based on the above risk factors provides a method for clinicians to evaluate sHLH.
Male
;
Female
;
Humans
;
Middle Aged
;
Aged
;
Aged, 80 and over
;
Lymphohistiocytosis, Hemophagocytic/complications*
;
Retrospective Studies
;
Prognosis
;
Risk Factors
;
Partial Thromboplastin Time
;
Albumins
10.Recurrent epistaxis with coagulation disorders in a boy aged 2 years.
Jia-Zhuo LI ; Xin TIAN ; Chu-Shu LIAO ; Xiang-Ling HE ; Cheng-Guang ZHU
Chinese Journal of Contemporary Pediatrics 2022;24(7):817-820
A boy, aged 2 years and 5 months, had recurrent epistaxis, and the coagulation function examination showed that activated partial thromboplastin time (APTT) was significantly prolonged. Further laboratory examinations showed that the prolonged APTT was not immediately corrected in the APTT correction test, with positive lupus anticoagulant and low prothrombin activity. The boy was diagnosed with hypoprothrombinemia-lupus anticoagulant syndrome. The condition was improved after treatment with glucocorticoid, immunoglobulin, and vitamin K1. The boy has been followed up for 6 months, and no epistaxis was observed. Prothrombin activity returned to normal, and lupus anticoagulant remained positive. This is a relatively rare disease, and for patients with bleeding symptoms and coagulation disorders, it is recommended to perform the tests such as APTT correction test, lupus anticoagulant testing, and coagulation factor dilution test, which can improve the detection rate of this disease, so as to achieve early diagnosis, provide rational treatment in the early stage, and improve the prognosis.
Antiphospholipid Syndrome/diagnosis*
;
Blood Coagulation Disorders
;
Child, Preschool
;
Epistaxis/etiology*
;
Humans
;
Hypoprothrombinemias/diagnosis*
;
Lupus Coagulation Inhibitor
;
Male
;
Partial Thromboplastin Time
;
Prothrombin


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