[The clinical significance of plasma PTX3 in patients with secondary hemophagocytic lymphohistiocytosis].

Objective: To investigate the significance of plasma pentraxin 3 (PTX3) in patients with secondary hemophagocytic lymphohistiocytosis (sHLH). Methods: Plasma PTX3 levels were tested by ELISA in 48 newly diagnosed sHLH patients, 18 healthy volunteers and 9 lymphoma controls in the First Affiliated Hospital of Nanjing Medical University from January 2017 to July 2019. Clinical parameters were collected, and the correlations with PTX3 levels were analyzed. Results: PTX3 level in newly diagnosed group was significantly higher than that of healthy control group [16.29(1.17-66.00) vs. 0.76(0.01-7.86) µg/L, P<0.01]. Patients with lymphoma-associated HLH(LHLH) had higher plasma level of PTX3 than Fhose with infection-associated HLH (IHLH) [24.29(3.36-66.00) vs. 9.56(1.17-36.50)µg/L, P<0.05]. Plasma PTX3 levels in 48 sHLH patients were positively correlated with serum ferritin (P<0.05). Receiver operating characteristic (ROC) curve for plasma PTX3 levels of sHLH and healthy controls produced a cutoff value at 3.9 µg/L, with its 86.7% sensitivity and 94.4% specificity. And ROC analysis showed that PTX3 17.5 µg/L was the critical value for diagnosis of LHLH from non-LHLH group, that the sensitivity and specificity were 63.0% and 76.2% respectively. The 1-year overall survival (OS) rate in patients with PTX3≥17.5 µg/L was significantly lower in those with PTX3<17.5 µg/L (18.5% vs. 75.8%, P<0.01). Conclusion: These results indicate the potential of PTX3 as a biomarker for diagnosis and prognosis in patients with sHLH.

[Clinical analysis of secondary hemophagocytic lymphohistiocytosis complicated with capillary leak syndrome].

Objective: To investigate the clinical characteristics of secondary hemophagocytic lymphohistiocytosis (sHLH) complicated with capillary leak syndrome (CLS) . Methods: The clinical and laboratory data of 87 sHLH patients, who were treated in our hospital between January 2015 and December 2017, were retrospectively analyzed. Depending on whether they were complicated with CLS, 21 sHLH patients were classified as the CLS-sHLH group, while 66 were classified as the non-CLS-sHLH group. The differences of clinical manifestations, laboratory tests, treatment and prognosis between the two groups were compared. Results: There was no significant difference in the etiology of sHLH between the CLS-sHLH group and the non-CLS-sHLH group (P>0.05) . The neutrophil, fibrinogen and albumin levels in the CLS-sHLH group were lower than those in the non-CLS-sHLH group, while the triacylglycerol levels were higher than those in the non-CLS-sHLH group (P<0.05) . Varying degrees of edema, weight gain, hypotension, hypoproteinemia, oliguria and multiple serous effusions were observed in the CLS-sHLH group. Among them, there were 15 patients that CLS get improved, and the medial time of improvement was 7 (5-14) days. The other 6 patients did not get remission, while they died within 6-30 days. The median overall survival of the CLS-sHLH group was lower than that of the non-CLS-sHLH group (75 days vs not reached, P=0.031) . Conclusions: There may be no correlation between the cause of sHLH and the occurrence of CLS. Severity of neutropenia, fibrinogen and albumin levels, and triglyceride levels may be accompanied for sHLH patients complicated with CLS. Patients with sHLH who complicated with CLS have a poor prognosis. Active treatment of HLH and its primary disease, reasonable fluid replacement and oxygen supply are crucial, which can effectively control disease progression.

[The mediation mechanism of coronary artery lesions in both male and female patients with Kawasaki disease].

Objective: To explore the mediation mechanism of coronary artery lesion among both male and female Kawasaki disease (KD) children. Methods: Children with KD that hospitalized in the Wenzhou Medical University affiliated Yuying Children's Hospital from January 2009 to December 2014, were included in this study. Differences on demographical characteristics, clinical manifestations, laboratory indicators, regimen and time of treatment, results from pre/post echocardiography and treatment between male and female patients, were compared. The independent effect of gender on the risk of coronary artery lesions (CAL) was evaluated, and the mediating effect of BMI, visiting time and KD type on the association between gender and CAL were also studied. Results: The average BMI level of male patients was higher than that of female patients. The difference was statistically significant (P<0.001). The prevalence of overweight among male patients (20.9%) was higher than female (14.1%). The difference was statistically significant (P=0.011). Data from the multivariate logistic regression analysis confirmed that the incidence of CAL in male patients was higher than that in female patients (aOR=1.50, 95%CI: 1.06-2.12) but the CAL was mainly different before on the immunoglobulin therapy. Results from the mediation analysis showed that BMI was an important mediator in the association between gender and CAL, with the indirect effect as 1.05 (95%CI: 1.01- 1.10) and the proportion mediated as 13.0%. Conclusions: Male patients presented higher incidence of CAL but was mainly reflected in the difference of CAL before the treatment. BMI was probably an important mediator related to the association between gender and CAL.

[Clinical significance of serum calcium and albumin in patients with secondary hemophagocytic lymphohistiocytosis].

Objective: To investigate the clinical significance of serum calcium concentrations and albumin levels in patients with secondary hemophagocytic lymphohistiocytosis (sHLH) . Methods: The clinical and laboratory data of 68 patients with newly diagnosed sHLH from April 2013 to April 2016, and 20 healthy controls were retrospectively analyzed. The patients were classified as the clinical remission group and the progression group according to the response criteria. To explore the changes of serum calcium concentrations and albumin levels in the patients, its correlations with other laboratory indexes and the impact on survival. Results: ①The serum calcium concentrations and albumin levels in lymphoma-related HLH group and non-tumor HLH group were lower than control group (P<0.05) ; The serum calcium concentrations and albumin levels in the clinical remission group after treatment was higher than that before treatment (P<0.01) , while in the progression group the serum calcium concentrations after treatment decreased (P=0.002) , the albumin levels did not changed significantly (P=0.086) .②The serum calcium concentrations in newly diagnosed sHLH patients was positively correlated with ANC, PLT, HGB (P<0.05) , and negatively with ferritin (P<0.05) ; The overall survival (OS) was significantly longer in patients with serum calcium concentration≥1.92 mmol/L than that<1.92 mmol/L (266 d vs 95 d, P=0.006) . ③The serum albumin levels in newly diagnosed sHLH patients was positively correlated with ANC, HGB (P<0.05) , and negatively with ferritin (P<0.05) ; The overall survival was longer in patients with serum albumin level ≥ 26.7 g/L than that<26.7 g/L (263 d vs 95 d, P=0.023) . ④The serum calcium concentrations in patients before treatment was positively correlated with serum albumin levels (P<0.001) , and no significant correlation was found in control group (P=0.079) . Conclusion: The serum calcium concentrations and albumin levels have clinical significance for disease diagnosis, decision-making therapy and treatment outcomes in the patients with sHLH.

Clinical analysis of cases of neonatal Streptococcus agalactiae sepsis.

With the advent of antibiotic resistance, pathogenic bacteria have become a major threat in cases of neonatal sepsis; however, guidelines for treatment have not yet been standardized. In this study, 15 cases of neonatal Streptococcus agalactiae sepsis from our hospital were retrospectively analyzed. Of these, nine cases showed early-onset and six cases showed late-onset sepsis. Pathogens were characterized by genotyping and antibiotic sensitivity tests on blood cultures. Results demonstrated that in cases with early-onset sepsis, clinical manifestations affected mainly the respiratory tract, while late-onset sepsis was accompanied by intracranial infection. Therefore, we suggest including a cerebrospinal fluid examination when diagnosing neonatal sepsis. Bacterial genotyping indicated the bacteria were mainly type Ib, Ia, and III S. agalactiae. We recommend treatment with penicillin or ampicillin, since bacteria were resistant to clindamycin and tetracycline. In conclusion, our results provide valuable information for the clinical treatment of S. agalactiae sepsis in neonatal infants.

The analysis of JAK2 and MPL mutations and JAK2 single nucleotide polymorphisms in MPN patients by MassARRAY assay.

Recent studies have shown that JAK2 V617F, MPL W515L/K and JAK2 exon 12 mutations underlie the major molecular pathogenesis of myeloproliferative disorders (MPN). Allele-Specific Polymerase Chain Reaction (AS-PCR), direct sequencing and MassARRAY assay were used to ascertain the real prevalence of these mutations and the influence of genetic susceptibility in Chinese MPN patients. The positive rate of JAK2 V617F in polycythaemia vera (PV), essential thrombocythaemia (ET) and primary myelofibrosis (PMF) was 82.0%, 36.6% and 51.1% respectively. One ET patient and two PMF patients harboured the MPL W515L mutation and three PV patients harboured JAK2 exon 12 mutations. All of these patients were confirmed as JAK2 V617F negative. Clinical data demonstrated that PV patients with JAK2 exon 12 mutations were younger, had higher haemoglobin levels and white blood cell counts than PV patients with JAK2 V617F. In addition, through analysis of 4 polymorphic loci of JAK2 gene, no significant difference of distribution frequency was found among PV, ET and PMF patients. Distribution frequency of haplotype also was not significantly different among PV, ET and PMF patients. We conclude that JAK2 V617F is a major molecular pathogenesis in Chinese MPN patients. MPL W515L mutation and JAK2 exon 12 mutations can also be found in JAK2 V617F negative MPN patients.

Molecular cytogenetic aberrations in 21 Chinese patients with plasma cell leukemia.

Plasma cell leukemia (PCL) is a rare malignant plasma cell disorder. Cytogenetic studies performed on plasma cell disorders are scarce and difficult because of the low proliferation rate of plasma cells (PCs). Fluorescence in situ hybridization (FISH) analysis is an attractive alternative for evaluation of chromosomal changes in PCL. To explore the molecular cytogenetic abnormalities in Chinese patients with PCL, interphase FISH studies with three probes for the regions containing 13q14.3 (D13S319), 14q32 (IGHC/IGHV) and 1q12(CEP1) were retrospectively performed in 21 PCL patients. FISH with LSI IGH/CCND1 and LSI IGH/FGFR3 probes were used to detect t(11;14)(q13;q32) and t(4;14)(p16;q32) in patients with 14q32 rearrangement. Among 21 PCL patients, molecular cytogenetic aberrations were found in 18 (81.8%) patients, four (19.0%) patients simultaneously had 13q14 deletion, illegitimate IgH translocation and 1q abnormality. 13q14 deletion was detected in 13 (61.9%) cases and illegitimate 14q32 rearrangement in 16 (76.2%) including six with t(11;14) and three with t(4;14). Chromosome 1 abnormality was found in seven (33.3%) patients, one with deletion of 1q, six with at least three copies amplifications of 1q12 (Amp1q12). 14q32 rearrangement and 13q14 deletion were found concurrently in 11 (52.4%) cases. It was showed that most PCL had chromosomal abnormalities, 14q32 rearrangement, 13q14 deletion and chromosome 1 abnormality are the frequent abnormalities, and over half of the 14q32 rearrangement were t(11;14) or t(4;14). t(4;14) and 13q14 deletion were correlated in PCL. FISH is a highly sensitive technique at detecting molecular cytogenetic aberrations in PCL and should be used in the routine evaluation of PCL.

Study on alteration of white blood cell deformability of NIDDM patients and the influencing factors.

Microfiltration technique was used to measure white blood cell deformability (WCD) in one hundred and thirty patients with non-insulin-dependent diabetes mellitus (NIDDM or type II). It was found that WCD of the diabetics was significantly decreased compared with fifty controls (P < 0.01) and further reduced with the rise of levels of plasma fibrinogen (Fg), plasma lipid, hemoglobin Alc (HbAlc) and fasting blood glucose (FBG), and also with the increase of age, the duration of diabetes, and the reduction of plasma magnesium concentration. It was shown that the decrease of WCD in diabetes mellitus was closely related to the degree of metabolic disturbance of the failure of diabetic control.