OBJECTIVES: To investigate the causal relationship between Helicobacter pylori (Hp) infection and immune thrombocytopenia (ITP) using Mendelian randomization (MR), as well as the association between Hp infection and chronic ITP (cITP) through a clinical study. METHODS: The datasets from genome-wide association studies were used to select the single nucleotide polymorphism loci significantly associated with Hp infection as genetic instrumental variables. The MR analysis model was used to investigate the causal relationship between ITP and Hp infection. A retrospective analysis was conducted on the medical data of 316 children with newly diagnosed ITP at the First Affiliated Hospital of Xinjiang Medical University from January 2020 to December 2023. The children were followed up for 1 year, and a multivariate logistic regression analysis was used to investigate the risk factors for cITP. RESULTS: The inverse variance weighted analysis revealed that Hp infection was significantly associated with an increased risk of ITP (OR=1.280, 95%CI: 1.098-1.492, P=0.002). There was no heterogeneity or pleiotropy in this MR study (P>0.05), and the model was stable. The "leave-one-out" sensitivity analysis verified the reliability of the results. The multivariate logistic regression analysis demonstrated that Hp infection was an independent risk factor for progression to cITP (OR=7.916, 95%CI: 3.327-18.832, P<0.001). CONCLUSIONS Hp infection is a risk factor for the onset of ITP and is an independent risk factor for cITP in children.
Humans ; Helicobacter Infections/complications* ; Purpura, Thrombocytopenic, Idiopathic/etiology* ; Child ; Male ; Female ; Helicobacter pylori ; Prognosis ; Child, Preschool ; Logistic Models ; Retrospective Studies ; Risk Factors ; Polymorphism, Single Nucleotide ; Adolescent ; Infant

This report describes two cases of severe immune-mediated thrombocytopenia after allogeneic hematopoietic stem cell transplantation (HSCT) who were treated with umbilical cord mesenchymal stem cells (UC-MSCs). Case 1 was a child with severe aplastic anemia who underwent haploidentical bone marrow and peripheral blood HSCT, with a chimerism rate of 99.8% on day +25 and severe immune-mediated thrombocytopenia on day +60. After intravenous immunoglobulin (IVIG) pulse therapy, platelet count increased temporarily but then decreased, while cyclosporine, methylprednisolone, and rituximab had a poor therapeutic effect. Case 2 was a child with Gaucher's disease who underwent unrelated umbilical cord blood HSCT, with a chimerism rate of 96.35% on day +41 and severe immune-mediated thrombocytopenia on day +153. After three sessions of IVIG pulse therapy, the platelet count increased initially but subsequently decreased. Therapies with dexamethasone, prednisone, cyclosporine, and recombinant human thrombopoietin also yielded a poor response. Both children received three sessions of UC-MSCs infusion, and platelet counts increased and were subsequently maintained within the normal range. Case 1 has been followed up for 10 years and remains in disease-free survival. UC-MSCs infusion may be effective for severe immune-mediated thrombocytopenia that is unresponsive to first- and second-line therapies after HSCT and could potentially improve the quality of life and disease-free survival rate.
Child ; Humans ; Hematopoietic Stem Cell Transplantation/adverse effects* ; Mesenchymal Stem Cell Transplantation ; Purpura, Thrombocytopenic, Idiopathic/etiology* ; Thrombocytopenia/therapy* ; Transplantation, Homologous ; Umbilical Cord/cytology*

OBJECTIVE: To investigate the efficacy and safety of zanubrutinib in the treatment of autoimmune cytopenia (AIC) secondary to indolent B-cell lymphoma (iBCL). METHODS: A total of 23 patients with iBCL complicated with AIC who were admitted to our hospital from December 2019 to September 2023 were selected as the research subjects. All patients were administered zanubrutinib 160 mg, twice daily, and continued oral administration. The objective response rate (ORR) of AIC, the therapeutic effect on lymphoma, and the incidence of adverse reactions were observed. RESULTS: After a median follow-up of 20 (5 to 48) months, the median duration of response was 9 (interquartile range [IQR] 5-24)months. AICA efficacy assessment showed that there were 10 cases of complete remission (CR), 9 cases of partial remission (PR), and 4 cases of no response (NR), and the ORR was 82.6% (19/23) (95%CI : 61.2-95.0). Among them, for the 14 patients with autoimmune hemolytic anemia (AIHA), 7 achieved CR, 5 had PR, and 2 had NR. For the 4 patients with immune thrombocytopenia (ITP), 1 reached CR, 2 had PR, and 1 had NR. Regarding the 5 patients with Evans syndrome (ES), 2 achieved CR, 2 had PR, and 1 had NR. The assessment of lymphoma efficacy showed that there were 10 cases of CR , 7 cases of PR , 6 cases of stable disease (SD), and no progressive cases, with an ORR of 73.9% (17/23) (95%CI : 51.6-89.8). The main adverse reactions during the treatment were infection, hemorrhage, neutropenia, elevated lymphocyte count, rash, and anemia. Most of these adverse reactions were grade 1-2 and tolerable. No arrhythmia and hypertension occurred, and no deaths due to adverse reactions. CONCLUSION Zanubrutinib is effective and safe for AIC secondary to iBCL.
Humans ; Pyrazoles/therapeutic use* ; Lymphoma, B-Cell/complications* ; Pyrimidines/therapeutic use* ; Piperidines/therapeutic use* ; Female ; Male ; Anemia, Hemolytic, Autoimmune/etiology* ; Thrombocytopenia/etiology* ; Middle Aged ; Purpura, Thrombocytopenic, Idiopathic/etiology* ; Treatment Outcome ; Adult ; Aged ; Cytopenia

OBJECTIVETo study the role of Helicobacter pylori (H. pylori) infection in newly diagnosed childhood immune thrombocytopenia (ITP).

METHODSA total of 495 children with newly diagnosed ITP who were hospitalized for the first time between January 2011 and December 2013 were included as the case group. A total of 123 children with common respiratory tract infection (not ITP or other diseases of blood system) were randomly selected as the control group. All patients were divided into four groups by age: <1 year group, 1-3 years group, 3-7 years group, and 7-14 years group. The incidence of H. pylori infection in all age groups and the clinical outcomes of ITP children with or without H. pylori infection were retrospectively analyzed.

RESULTSThe incidence rate of H. pylori infection in the case group increased with increasing age. There was no significant difference in the incidence rate of H. pylori infection between the case and the control groups among subjects of the same age (P>0.05). All the ITP patients were not given anti-H. pylori treatment and only received the treatment (glucocorticoid and/or immunoglobulin) for ITP, and their remission rate declined with increasing age. There was no significant difference in the remission rate between the ITP children with H. pylori infection and those without H. pylori infection in the same age group (P>0.05).

CONCLUSIONSH. pylori infection may not be a major cause of ITP in children, and the clinical outcomes of children with acute ITP are not affected by receiving anti-H. pylori treatment or not.

Adolescent ; Age Factors ; Child ; Child, Preschool ; Female ; Helicobacter Infections ; complications ; epidemiology ; Helicobacter pylori ; Humans ; Incidence ; Infant ; Male ; Purpura, Thrombocytopenic, Idiopathic ; etiology

OBJECTIVETo explore the clinical value of immature platelet fraction (IPF), absolute immature platelet fraction (A- IPF) and thrombelastograph (TEG) on assessment of bleeding risk of immune thrombocytopenia (ITP).

METHODStwo hundred and seventy- one patients with ITP were assessed based on ITP-BAT bleeding grading system. IPF, A-IPF were determined in 271 patients ,TEG in 125 patients. The correlations between bleeding grades and IPF, A-IPF, variables of TEG in subgroups were analyzed by statistical method. The predictive value of IPF, A-IPF, and variables of TEG on bleeding risk of ITP patients was evaluated.

RESULTSThere were no significant differences in bleeding degree in all patients with different gender and disease stage (P>0.05). Mild bleeding rate in children was higher than that in adult (P<0.05). PLT inversely correlated with bleeding grade for the entire cohort (P<0.001). In all subjects, PLT< 30 × 10⁹/L and pediatric cohorts with PLT< 30 × 10⁹/L, PLT were negatively correlated with IPF (P<0.05), positive correlated with A-IPF (P<0.001) and the maximum amplitude (MA (P<0.05). Bleeding grades were significantly correlated with IPF, A-IPF, MA in all subjects and patients with PLT< 30 × 10⁹/L (P<0.001). IPF, A-IPF and MA did not correlate with bleeding grades in children with PLT< 30 × 10⁹/L (P>0.05). ROC curve analysis revealed IPF, A-IPF and MA had better predictive value (AUC 0.745, 0.744, 0.813, P<0.001). Multivariate analysis showed that IPF and MA were independence factors for predicting bleeding risk in ITP patients and comprehensive predictive value was higher (AUC 0.846, P<0.001) than single variable.

CONCLUSIONIPF, A-IPF and MA could accurately evaluate bleeding risk in ITP patients. It may be considered as reference index of the treatment and observation index of curative effect.

Adult ; Blood Platelets ; Child ; Hemorrhage ; etiology ; physiopathology ; Humans ; Multivariate Analysis ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic ; complications ; physiopathology ; ROC Curve

Adult ; Antirheumatic Agents ; therapeutic use ; Castleman Disease ; drug therapy ; etiology ; Humans ; Lupus Erythematosus, Systemic ; complications ; drug therapy ; Male ; Purpura, Thrombocytopenic, Idiopathic ; complications ; drug therapy ; Rituximab ; therapeutic use ; Treatment Outcome

No abstract available.
Carcinoma, Renal Cell/*complications/pathology/surgery ; Danazol/therapeutic use ; Humans ; Kidney Neoplasms/*complications/pathology/surgery ; Male ; Middle Aged ; Nephrectomy ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic/blood/drug therapy/*etiology

OBJECTIVETo explore the clinical significance, reliability and responsiveness of ITPBAT bleeding grading system for patients with immune thrombocytopenia (ITP).

METHODSOne hundred and eighty-three patients with ITP were assessed by using of ITP-BAT bleeding grading system. Test-retest reliability, responsiveness of ITP-BAT bleeding grading system and association between bleeding grades and platelet counts, age, gender, disease stage were analyzed.

RESULTSBleeding degree of ITP patients and the platelet count were negatively correlated (r=- 0.744, P<0.01) and bleeding degree increased significantly with platelet counts below 20×10⁹/L (χ²=82.40，P<0.01). Mild bleeding rate in children was 68.5%, higher than that in adult(χ²=8.839，P<0.01), and severe bleeding rate in the elderly was 14.3%, higher than that in non-elderly(χ²=7.056，P<0.01). There were no significant differences in bleeding degree in patients with different gender and disease stage (χ²=4.922, P>0.05 and χ²=3.411, P>0.05). Bleeding grades before and after treatment had more significant difference(Z=-6.61, P<0.01). Scoring consistency of two doctors was 66.1% (κ=0.561), and scoring consistency of the same doctor was 94.7% (κ=0.874).

CONCLUSIONITP-BAT bleeding grading system in China has good validity and responsiveness, closely related to clinical indicators. It is sensitive to the variation of the hemorrhage in patients. ITP-BAT could be used as a reference index of the treatment, and also be used as an observation index of curative effect.

Hemorrhage ; etiology ; physiopathology ; Humans ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic ; complications ; physiopathology ; Reproducibility of Results

Adolescent ; Child ; Humans ; Leukemia, Myeloid, Acute ; drug therapy ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; drug therapy ; Purpura, Thrombocytopenic, Idiopathic ; drug therapy ; etiology

The study was aimed to explore the relationship between infection of Helicobacter Pylorus (H. Pylorus) and etiology of idiopathic thrombocytopenic purpura (ITP) and evaluate whether H. Pylorus eradication can increase platelet count in patients with ITP. The data-bases of cqvip, Wanfang, TsingHua TongFang, CNKI and PubMed were searched, inclusion and exclusion criteria and heterogeneity test were determined. The studies of H. Pylorus infection and ITP were investigated with fixed effect mode Meta-analysis. Relationship between H. Pylorus infection and etiology of ITP, H. Pylorus eradication and curative effect of ITP were comprehensively and quantitatively evaluated. OR > 1 indicated that factor of exposure was the risk factor of disease; OR < 1 suggested that factor of exposure was the protective factor of disease; OR = 1 revealed that there were no correlation between factor of exposure and diseases. 95%CI was the confidence interval of total OR. The results showed that a total of 211 cases and 210 controls from 5 studies was included to evaluate the exposure of H. Pylorus between ITP patients and controls, the pooled OR was 1.73 (95%CI = 1.12 - 2.67); a total of 458 cases and 305 controls from 13 studies was included to evaluate the relationship between H. Pylorus eradication and curative effect of ITP, the pooled OR was 6.53 (95%CI = 4.44 - 9.61). It is concluded that H. Pylorus infection plays a role in the etiology of ITP. Eradication of H. Pylorus increases platelet count in patients with ITP. H. Pylorus eradication can be used as the first line measure to treat H. Pylorus-positive ITP.
Helicobacter Infections ; therapy ; Helicobacter pylori ; Humans ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic ; etiology ; microbiology ; therapy ; Treatment Outcome