Objective:To explore the effect of low-dose rituximab in primary immune thrombocytopenia.Methods:From January 2022 to January 2023,60 patients with primary immune thrombocytopenia were randomly divided into two groups...Objective:To explore the effect of low-dose rituximab in primary immune thrombocytopenia.Methods:From January 2022 to January 2023,60 patients with primary immune thrombocytopenia were randomly divided into two groups.The control group was treated with standard doses of rituximab,and the observation group was treated with low doses of rituximab.Rituximab was used for treatment,and the clinical curative effect of the two groups was observed.Results:Before treatment,there was no statistically significant difference in platelet count(PLT),anti-GPⅡb/Ⅲa antibody,and anti-GPⅠb/Ⅸantibody between the two groups(P>0.05).After treatment,the PLT of the two groups increased significantly.Antibodies were all decreased,and there was no significant difference between the two groups(P>0.05).The incidence of adverse reactions in the observation group was 13.33%,and that in the control group was 40.00%.The adverse reactions in the observation group were significantly lower than the control group(P<0.05).Conclusion:In the clinical treatment of primary immune thrombocytopenia,low-dose rituximab can control the progression of the disease,improve blood routine indicators,and have fewer adverse reactions.展开更多
Hyperthyroidism and thrombocytopenia have long been associated with each other. We present a case of 38 years old female presenting with complaints of bleeding from gums, bleeding per rectum, hematuria and easy bruisi...Hyperthyroidism and thrombocytopenia have long been associated with each other. We present a case of 38 years old female presenting with complaints of bleeding from gums, bleeding per rectum, hematuria and easy bruising since 7 days ago. She also had a diffuse, non-tender neck swelling moving with deglutition and positive bruit on auscultation. Her laboratory results indicated thrombocytopenia (22 × 10^9/L) and her thyroid function test revealed TSH of 0.01 mIU/ml (normal: 0.17 - 4.05), free T3 of 19.19 pg/ml (normal: 1.6 - 3.7), free T4 of 4.09 ng/dl (normal: 0.89 - 1.79). Thyroid scan showed diffuse goiter with increased tracer uptake. Furthermore, her serum anti-thyroglobulin and anti-thyroid peroxide were elevated to 205 IU/ml and 713 IU/ml respectively. She was started on carbimazole and methylprednisolone immediately. On the 3rd day of admission, she was tested to be positive for Plasmodium Vivax after a fever spike and was started on Chloroquine (CQ) and later shifted to Primaquine (PQ). During 2 weeks of admission, her platelet count kept fluctuating between <10 × 10^9/L and <100 × 10^9/L with frequent transfusions of mega units of platelets. During 3rd week, her platelets improved and she was discharged with a platelet count of 370 × 10^9/L. This case, therefore, supports the association between autoimmune thyroid diseases such as Graves’ disease and idiopathic thrombocytopenic purpura (ITP) strongly suggesting the need for evaluating thyroid disease in cases of severe thrombocytopenia especially those refractory to treatment. Also, the effectiveness of treatment of thyroid disease on thrombocytopenia is also highlighted. In addition, it showed the possible added exacerbating effects of malarial infection on thrombocytopenia.展开更多
Primary immune thrombocytopenia(ITP) is an immunemediated disorder affecting both adults and children, characterised by bleeding complications and low platelet counts. Corticosteroids are the first-line therapy for IT...Primary immune thrombocytopenia(ITP) is an immunemediated disorder affecting both adults and children, characterised by bleeding complications and low platelet counts. Corticosteroids are the first-line therapy for ITP, but only 20%-40% of cases achieve a stable response. Splenectomy is the main therapy for patients failing to respond to corticosteroids for decades, and about two-thirds of patients achieve a long-lasting response. Although some new drugs are developed to treat ITP as second-line therapies in recent years, splenectomy is still the better choice with less cost and more efficiency. Laparoscopic splenectomy(LS) for ITP proves to be a safe technique associated with lower morbidity and faster recovery and similar hematological response when compared to traditional open splenectomy. Based on the unified hematological outcome criteria by current international consensus, the response rate of splenectomy should be reassessed. So far, there are not widely accepted preoperative clinical indicators predicting favorable response to LS. Since the patients undergoing surgery take the risk of complications and poor hematological outcome, the great challenge facing the doctors is to identify a reliable biomarker for predicting longterm outcome of splenectomy which can help make the decision of operation.展开更多
BACKGROUND Wilson disease (WD) is a genetic disorder of hepatic copper excretion,leading to copper accumulation in various tissues.The manifestations are quite variable,and hemolytic anemia is the most common hematolo...BACKGROUND Wilson disease (WD) is a genetic disorder of hepatic copper excretion,leading to copper accumulation in various tissues.The manifestations are quite variable,and hemolytic anemia is the most common hematological presentation.WD associated with thrombocytopenia is very rare.CASE SUMMARY We report the case of an 11-year-old Chinese girl with WD that was associated with immune thrombocytopenia (ITP).Thrombocytopenia was the initial chief complaint for her to visit a hematologist,and ITP was diagnosed based on the results of a bone marrow biopsy and positive antiplatelet autoantibodies.About two weeks before the thrombocytopenia was found,the patient developed drooling.Tremors developed in her right hand about one week after being diagnosed with ITP,after which she was admitted to our hospital.Further evaluations were performed.Ceruloplasmin was decreased,with an increased level of copper in her 24-h urine excretion.Kayser Fleischer's ring (K-F ring) was positive.The ultrasound showed liver cirrhosis,and brain magnetic resonance imaging showed that the lenticular nucleus,caudate nucleus,and brainstem presented a low signal intensity in T1-weighted images and high signal intensity in T2-weighted images.WD was diagnosed and a genetic analysis was performed.A compound heterozygous mutation in ATP7B was detected;c.2333G>T (p.Arg778Leu) in exon 8 and c.3809A>G (p.Asn1270Ser) in exon 18.The former was inherited from her father and the latter from her mother.However,her parents showed normal liver function and negative K-F rings.Such a compound mutation in a case of WD associated with ITP in children has not been published previously.CONCLUSION WD can associate with thrombocytopenia but the mechanism is still unclear.We recommend that antiplatelet autoantibodies should be tested in WD patients with thrombocytopenia in future to verify the association.展开更多
BACKGROUND Primary immune thrombocytopenia(ITP) is a rare autoimmune disease associated with a high bleeding risk. For those patients with gastric cancer, surgical treatment may be the only option for therapy. Here, w...BACKGROUND Primary immune thrombocytopenia(ITP) is a rare autoimmune disease associated with a high bleeding risk. For those patients with gastric cancer, surgical treatment may be the only option for therapy. Here, we present the first case of gastric cancer with severe and medically refractory ITP treated by radical resection of the gastric cancer and splenectomy. CASE SUMMARY A 54-year-old female patient was admitted to our surgical department with a 2 mo history of decreased appetite, nausea, vomiting, and weight loss, which progressed to difficulty in feeding 3 d prior to her visit. According to her medical history, she was diagnosed with refractory ITP [platelets(PLT), 3000-8000/μL] 10 years ago. After admission, the patient underwent a splenectomy and a distal subtotal gastrectomy(D2 radical resection) with Roux-en-Y reconstruction simultaneously. She had an uneventful postoperative course with a slight increase in her PLT count. This case is unique in terms of the patient's complication of severe and medically refractory ITP.CONCLUSION Simultaneous splenectomy, preoperative PLT transfusion, and early enteral nutrition were important treatment methods for helping this patient recover.展开更多
Background: IL-17 is an inflammatory cytokine that plays a crucial role in many autoimmune diseases. Aim: To investigate the association of IL-17A rs2275913 and IL-17F rs763780 gene polymorphisms with acute immune thr...Background: IL-17 is an inflammatory cytokine that plays a crucial role in many autoimmune diseases. Aim: To investigate the association of IL-17A rs2275913 and IL-17F rs763780 gene polymorphisms with acute immune thrombocytopenic purpura (ITP) in Egyptian children. Patients and methods: We examined 80 patients (male/female, 33/47;median age, 7 years old) diagnosed with acute ITP and 55 healthy controls (male/female, 28/27;median age, 7 years old). Genotyping was determined using the polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) technique. Results: In the acute ITP group compared to control, statistical analysis of the genotype frequencies (GG, AG, AA) of the IL-17A rs2275913 polymorphism and its alleles (A, G) showed no significant difference between the two groups (p > 0.05). Interestingly, the IL17A rs2275913 GG genotype was associated with early recovery (p = 0.04). As regard the genotype frequencies of the IL-17F rs763780 polymorphism, there was statistical significant difference in the TT and TC genotype frequencies between the case and control groups (p = 0.001 and p = 0.003, respectively). The number of IL-17F rs763780 T alleles was significantly higher in acute ITP patients as compared with children in the control group (p Conclusion: The present findings indicate that the IL-17 polymorphism IL-17F rs763780, but not IL-17A rs2275913 may be associated with a higher risk of acute ITP in Egyptian children.展开更多
Immune thrombocytopenia(ITP)is an autoimmune disease mediated by anti-platelet autoantibodies.There is growing evidence that the eradication of Helicobacter pylori(H.pylori)effectively increases platelet count in a co...Immune thrombocytopenia(ITP)is an autoimmune disease mediated by anti-platelet autoantibodies.There is growing evidence that the eradication of Helicobacter pylori(H.pylori)effectively increases platelet count in a considerable proportion of ITP patients infected with this bacterium.In the majority of ITP patients responding to H.pylori eradication therapy,the anti-platelet autoantibody response is completely resolved with no relapse for more than 7 years,indicating that the disease is cured.Therefore,adult patients with suspected ITP should be examined for H.pylori infection,and eradication therapy is recommended if the infection is present.Notably,however,the efficacy of H.pylori eradication therapy in ITP patients varies widely among countries,with a higher response rate in Japan compared with the United States and European countries other than Italy.The pathogenesis of H.pylori-associated ITP is still uncertain,although the mechanisms are known to involve multiple factors.H.pylori may modulate the Fcγ-receptor balance of monocytes/macrophages in favor of activating Fcγreceptors,and H.pylori components may mimic the molecular makeup of platelet antigens.Further studies of the pathogenic process of H.pyloriassociated ITP may be useful for the development of new therapeutic strategies for ITP.展开更多
<b>Background:</b> Management of immune thrombocytopenia (ITP) during pre- gnancy can be challenging, particularly by identifying a threshold for safe administration of neuraxial/general anesthesia and min...<b>Background:</b> Management of immune thrombocytopenia (ITP) during pre- gnancy can be challenging, particularly by identifying a threshold for safe administration of neuraxial/general anesthesia and minimizing postpartum hemorrhage. There is controversy over the safety of cesarean section (CS) in ITP patients. In this case report, we discuss general anesthesia management in a patient with ITP with severe thrombocytopenia. <b>Case Presentation:</b> A 28-year-old female with relapsed/refractory ITP and severe thrombocytopenia underwent general anesthesia and emergent cesarean section with successful outcomes and minimal bleeding. Platelet counts before CS were 5000 × 10<sup>9</sup> L, the patient received 1 unit of platelets before the procedure and 1 unit of platelet and tranexamic acid 500 mg was injected slowly during the procedure. No evidence of bleeding and no complications were observed in the patient or newborn. <b>Conclusions:</b> In an emergent circumstance, general anesthesia and cesarean section procedure were performed safely in a patient with severe thrombocytopenia, no hemorrhagic complications were seen for this patient or neonate. <b>Objective of This Manuscript:</b> To share our experience of a safe emergent CS procedure and general anesthesia in a patient with severe thrombocytopenia. Our experience may guide the management of ITP patients in emergent delivery circumstances.展开更多
Objective: To investigate the biological basis of qi, blood and vessel in immune thrombocytopenia(ITP) patients with syndrome of qi failing to govern blood(SQFGB) based on traditional Chinese medicine.Methods: A total...Objective: To investigate the biological basis of qi, blood and vessel in immune thrombocytopenia(ITP) patients with syndrome of qi failing to govern blood(SQFGB) based on traditional Chinese medicine.Methods: A total of 52 ITP patients with SQFCB were enrolled and divided into bleeding group(38 cases) and non-bleeding group(14 cases).Bleeding group was further divided into mild qi deficiency group(25 cases) and moderate/severe qi deficiency group(13 cases) based on Chinese Medicine syndrome score.20 healthy volunteer were recruited as control group.The count of platelet(PLT) was taken as the blood related indicator.The expressions of cytokines including IL-1β, IL-17 A, TNF-α, CD40 L, and TGF-β, detected by Aim Plex Multiple Immunoassays for Flow, were taken as the qi related indicators.The expressions of VEGF-A, detected by Aim Plex Multiple Immunoassays for Flow and NO, NOS, and ET-1 detected by ELISA, were taken as the vessel related indicators.Results: As compared to the control group, the count of PLT, taken as the blood related indicator, was significantly lower in ITP group patients with SQFCB(P<0.05).The expression levels of IL-17 A and TNF-α, taken as the qi related indicators, were significantly higher, while those of CD40 L, IL-1β, and TGF-β, also taken as the qi related indicators, were significantly lower in ITP patients with SQFCB, respectively(P<0.05).The expression levels of NO and ET-1, taken as the vessel related indicators, were significantly higher, while the expression levels of NOS and VEGF-A also taken as the vessel related indicators, were significantly lower in ITP patients with SQFCB, respectively(P<0.05).The count of PLT, taken as the blood related indicator, was significantly lower in moderate/severe group than those in mild group(P<0.05).The expression levels of CD40 L and TGF-β, taken as the qi related indicators, were also significantly lower in moderate/severe group than those in mild group, respectively(P<0.05).Conclusion: The count of PLT might be the biological basis of blood.The expressions of NO, NOS, ET-1 and VEGF-A might be the biological basis of vessel.The expressions of IL-1β, IL-17 A, TNF-α, TGF-β, and CD40 L may be the biological basis of qi.The expressions of CD40 L and TGF-β could reflect the degree of qi deficiency in ITP patients based on the theory of qi and blood.展开更多
Prednisone is the most common first-line treatment for adult primary immune thrombocytopenia (ITP). However, the best initial therapeutic approach is still a matter of debate. Prior studies have shown that high-dose d...Prednisone is the most common first-line treatment for adult primary immune thrombocytopenia (ITP). However, the best initial therapeutic approach is still a matter of debate. Prior studies have shown that high-dose dexamethasone (HD-DXM) produces a high sustained efficacy not achieved by conventional prednisone therapy. However, the definition of response widely differs between individual reports, and this heterogeneity makes comparison of the efficacy difficult. The aim of our study was to compare the therapeutic outcomes of a conventional dose of prednisone with HD-DXM for adult ITP patients as initial therapy. Thirty patients treated with prednisone and 22 patients treated HD-DXM were retrospectively analyzed. No significant differences between the HD-DXM and prednisone groups were observed for the rates of complete response (68% vs. 70%) and response (18% vs. 17%). However, 1 year probability of sustained response was significantly greater in the HD-DXM group than in the prednisone group (78% vs. 38%;P = 0.008). No adverse events necessitating discontinuation of treatment were observed in either group. Our retrospective analysis showed that initial treatment with HD-DXM produced longer response duration compared to a conventional dose of prednisone. Randomized clinical trials are warranted to establish the optimal initial steroid therapy for adult ITP.展开更多
BACKGROUND Vaccine-induced immune thrombotic thrombocytopenia(VITT)is a rare and potentially life-threatening condition after receiving coronavirus disease vaccines.It is characterized by symptom onset at 5 to 30 d po...BACKGROUND Vaccine-induced immune thrombotic thrombocytopenia(VITT)is a rare and potentially life-threatening condition after receiving coronavirus disease vaccines.It is characterized by symptom onset at 5 to 30 d postvaccination,thrombocytopenia,thrombosis,high D-dimer level,and antiplatelet factor 4(anti-PF4)antibody positivity.VITT can progress very fast,requiring urgent management.Only few studies have described its detailed clinical course and imaging changes.We report a typical VITT case in a patient who underwent regular repeated brain imaging examinations.CASE SUMMARY A young woman presented with headaches at 7 d after the ChAdOx1 nCoV-19vaccine(AZD1222)injection.She then showed progressive symptoms of left upper limb clumsiness.Brain computed tomography revealed venous infarction at the right parietal lobe with a hyperacute thrombus in the cortical vein.Two hours later,brain magnetic resonance imaging revealed hemorrhage at the same area.Magnetic resonance venography showed an irregular contour of the right transverse sinus.Laboratory examination revealed a high D-dimer level,thrombocytopenia,and a high titer for anti-PF4 antibodies.She was treated with anticoagulants,intravenous immunoglobulin,and steroids and analgesic agents were administered for pain control.She had a marked improvement on headaches and clumsiness after treatment along with radiological thrombus resolution.During follow-up at the outpatient department,her modified Rankin scale at 90 d was 1.CONCLUSION Clinicians should be alerted whenever patients present with persistent and progressive headaches or focal motor/sensory deficits postvaccination.展开更多
Immune Thrombocytopenic purpura(ITP)is a haematologicimmune-mediated disorder in which the amount of platelet in the blood decreases abnormally.Single-agent therapies for ITP have not proven successful in achieving lo...Immune Thrombocytopenic purpura(ITP)is a haematologicimmune-mediated disorder in which the amount of platelet in the blood decreases abnormally.Single-agent therapies for ITP have not proven successful in achieving long-term remission,with relapse occurring in about half of the patients(p/t).Treatment options which include Rituximab with Dexamethasone as frontline therapy,have durable response rates ranging from 58%to 76%.In this study,we have used‘Total therapy’as treatment which includes low-dose Rituximab in combination with Thrombopoietin receptor agonist(TPO-RA)(Romiplostim)and high-dose Dexamethasone.In this case series study,each patient received romiplostim(250 mcg weekly s/c,4 doses)in combination with low-dose rituximab(100 mg weekly IV,4 doses)and high-dose dexamethasone(40 mgIV on days 1-4and days 15-18).This treatment combination demonstrated rapid response rates and a low rate of side effects,making it a good alternative for individuals with ITP.展开更多
Objective:To analyze the hot spot and frontier of traditional Chinese medicine treatment for primary immune thrombocytopenia with CiteSpace softwar for visualized analysis.Methods:The relevant literature about treatme...Objective:To analyze the hot spot and frontier of traditional Chinese medicine treatment for primary immune thrombocytopenia with CiteSpace softwar for visualized analysis.Methods:The relevant literature about treatment for ITP through traditional Chinese medicine was retrieved from CNKI.The CiteSpace software was used to create visualization map for authors,institutions and keywords.Results:Through visualization analysis,121 authors and 36 institutions were included in the map.The main research hot spot is the clinical effect of traditional Chinese medicine and integrated traditional Chinese and Western medicine in the treatment for ITP.Conclusion:Ratio of research about traditional Chinese medicine treatment on ITP was relatively few.In addition,the cooperation and communication were also insufficient between teams and institutions.The research hot spot mostly focuses on clinical treatment,while theoretical research was very few.There is no deep investigation on treatment mechanism.展开更多
Immune thrombocytopenia is an autoimmune disease resulting in the destruction of platelets.It is classified as acute,thrombocytopenia occurring for < 6 mo and usually resolving spontaneously,and chronic,lasting >...Immune thrombocytopenia is an autoimmune disease resulting in the destruction of platelets.It is classified as acute,thrombocytopenia occurring for < 6 mo and usually resolving spontaneously,and chronic,lasting >6 mo and requiring therapy to improve the thrombocytopenia.The underlying defects leading to autoantibody production are unknown.Molecular mimicry appears to play a role in the development of self-reactive platelet antibodies after vaccination and certain viral infections.Platelet life span is reduced as a consequence of antibody-mediated clearance by tissue macrophages in essentially all patients.Diagnosis is based on the exclusion of the other causes of thrombocytopenia.Steroid is the first choice of the treatment,often followed by splenectomy in unresponsive cases.Intravenous immunoglobulin,anti-Rho(D) immune globulin,azathioprine,cyclosporine A,cyclophosphamide,danazol,dapsone,mycophenolate mofetil,rituximab,thrombopoietin receptor agonists and vinca alkaloids are other choices of treatment.展开更多
文摘Objective:To explore the effect of low-dose rituximab in primary immune thrombocytopenia.Methods:From January 2022 to January 2023,60 patients with primary immune thrombocytopenia were randomly divided into two groups.The control group was treated with standard doses of rituximab,and the observation group was treated with low doses of rituximab.Rituximab was used for treatment,and the clinical curative effect of the two groups was observed.Results:Before treatment,there was no statistically significant difference in platelet count(PLT),anti-GPⅡb/Ⅲa antibody,and anti-GPⅠb/Ⅸantibody between the two groups(P>0.05).After treatment,the PLT of the two groups increased significantly.Antibodies were all decreased,and there was no significant difference between the two groups(P>0.05).The incidence of adverse reactions in the observation group was 13.33%,and that in the control group was 40.00%.The adverse reactions in the observation group were significantly lower than the control group(P<0.05).Conclusion:In the clinical treatment of primary immune thrombocytopenia,low-dose rituximab can control the progression of the disease,improve blood routine indicators,and have fewer adverse reactions.
文摘Hyperthyroidism and thrombocytopenia have long been associated with each other. We present a case of 38 years old female presenting with complaints of bleeding from gums, bleeding per rectum, hematuria and easy bruising since 7 days ago. She also had a diffuse, non-tender neck swelling moving with deglutition and positive bruit on auscultation. Her laboratory results indicated thrombocytopenia (22 × 10^9/L) and her thyroid function test revealed TSH of 0.01 mIU/ml (normal: 0.17 - 4.05), free T3 of 19.19 pg/ml (normal: 1.6 - 3.7), free T4 of 4.09 ng/dl (normal: 0.89 - 1.79). Thyroid scan showed diffuse goiter with increased tracer uptake. Furthermore, her serum anti-thyroglobulin and anti-thyroid peroxide were elevated to 205 IU/ml and 713 IU/ml respectively. She was started on carbimazole and methylprednisolone immediately. On the 3rd day of admission, she was tested to be positive for Plasmodium Vivax after a fever spike and was started on Chloroquine (CQ) and later shifted to Primaquine (PQ). During 2 weeks of admission, her platelet count kept fluctuating between <10 × 10^9/L and <100 × 10^9/L with frequent transfusions of mega units of platelets. During 3rd week, her platelets improved and she was discharged with a platelet count of 370 × 10^9/L. This case, therefore, supports the association between autoimmune thyroid diseases such as Graves’ disease and idiopathic thrombocytopenic purpura (ITP) strongly suggesting the need for evaluating thyroid disease in cases of severe thrombocytopenia especially those refractory to treatment. Also, the effectiveness of treatment of thyroid disease on thrombocytopenia is also highlighted. In addition, it showed the possible added exacerbating effects of malarial infection on thrombocytopenia.
基金Supported by Science and Technique Project of Guangdong Province,No.2012B031800284
文摘Primary immune thrombocytopenia(ITP) is an immunemediated disorder affecting both adults and children, characterised by bleeding complications and low platelet counts. Corticosteroids are the first-line therapy for ITP, but only 20%-40% of cases achieve a stable response. Splenectomy is the main therapy for patients failing to respond to corticosteroids for decades, and about two-thirds of patients achieve a long-lasting response. Although some new drugs are developed to treat ITP as second-line therapies in recent years, splenectomy is still the better choice with less cost and more efficiency. Laparoscopic splenectomy(LS) for ITP proves to be a safe technique associated with lower morbidity and faster recovery and similar hematological response when compared to traditional open splenectomy. Based on the unified hematological outcome criteria by current international consensus, the response rate of splenectomy should be reassessed. So far, there are not widely accepted preoperative clinical indicators predicting favorable response to LS. Since the patients undergoing surgery take the risk of complications and poor hematological outcome, the great challenge facing the doctors is to identify a reliable biomarker for predicting longterm outcome of splenectomy which can help make the decision of operation.
文摘BACKGROUND Wilson disease (WD) is a genetic disorder of hepatic copper excretion,leading to copper accumulation in various tissues.The manifestations are quite variable,and hemolytic anemia is the most common hematological presentation.WD associated with thrombocytopenia is very rare.CASE SUMMARY We report the case of an 11-year-old Chinese girl with WD that was associated with immune thrombocytopenia (ITP).Thrombocytopenia was the initial chief complaint for her to visit a hematologist,and ITP was diagnosed based on the results of a bone marrow biopsy and positive antiplatelet autoantibodies.About two weeks before the thrombocytopenia was found,the patient developed drooling.Tremors developed in her right hand about one week after being diagnosed with ITP,after which she was admitted to our hospital.Further evaluations were performed.Ceruloplasmin was decreased,with an increased level of copper in her 24-h urine excretion.Kayser Fleischer's ring (K-F ring) was positive.The ultrasound showed liver cirrhosis,and brain magnetic resonance imaging showed that the lenticular nucleus,caudate nucleus,and brainstem presented a low signal intensity in T1-weighted images and high signal intensity in T2-weighted images.WD was diagnosed and a genetic analysis was performed.A compound heterozygous mutation in ATP7B was detected;c.2333G>T (p.Arg778Leu) in exon 8 and c.3809A>G (p.Asn1270Ser) in exon 18.The former was inherited from her father and the latter from her mother.However,her parents showed normal liver function and negative K-F rings.Such a compound mutation in a case of WD associated with ITP in children has not been published previously.CONCLUSION WD can associate with thrombocytopenia but the mechanism is still unclear.We recommend that antiplatelet autoantibodies should be tested in WD patients with thrombocytopenia in future to verify the association.
文摘BACKGROUND Primary immune thrombocytopenia(ITP) is a rare autoimmune disease associated with a high bleeding risk. For those patients with gastric cancer, surgical treatment may be the only option for therapy. Here, we present the first case of gastric cancer with severe and medically refractory ITP treated by radical resection of the gastric cancer and splenectomy. CASE SUMMARY A 54-year-old female patient was admitted to our surgical department with a 2 mo history of decreased appetite, nausea, vomiting, and weight loss, which progressed to difficulty in feeding 3 d prior to her visit. According to her medical history, she was diagnosed with refractory ITP [platelets(PLT), 3000-8000/μL] 10 years ago. After admission, the patient underwent a splenectomy and a distal subtotal gastrectomy(D2 radical resection) with Roux-en-Y reconstruction simultaneously. She had an uneventful postoperative course with a slight increase in her PLT count. This case is unique in terms of the patient's complication of severe and medically refractory ITP.CONCLUSION Simultaneous splenectomy, preoperative PLT transfusion, and early enteral nutrition were important treatment methods for helping this patient recover.
文摘Background: IL-17 is an inflammatory cytokine that plays a crucial role in many autoimmune diseases. Aim: To investigate the association of IL-17A rs2275913 and IL-17F rs763780 gene polymorphisms with acute immune thrombocytopenic purpura (ITP) in Egyptian children. Patients and methods: We examined 80 patients (male/female, 33/47;median age, 7 years old) diagnosed with acute ITP and 55 healthy controls (male/female, 28/27;median age, 7 years old). Genotyping was determined using the polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) technique. Results: In the acute ITP group compared to control, statistical analysis of the genotype frequencies (GG, AG, AA) of the IL-17A rs2275913 polymorphism and its alleles (A, G) showed no significant difference between the two groups (p > 0.05). Interestingly, the IL17A rs2275913 GG genotype was associated with early recovery (p = 0.04). As regard the genotype frequencies of the IL-17F rs763780 polymorphism, there was statistical significant difference in the TT and TC genotype frequencies between the case and control groups (p = 0.001 and p = 0.003, respectively). The number of IL-17F rs763780 T alleles was significantly higher in acute ITP patients as compared with children in the control group (p Conclusion: The present findings indicate that the IL-17 polymorphism IL-17F rs763780, but not IL-17A rs2275913 may be associated with a higher risk of acute ITP in Egyptian children.
基金Supported by A research grant for Research on Intractable Diseases from the Japanese Ministry of Health,Labor,and Welfare,No.H23-Nanchi-Ippan-002
文摘Immune thrombocytopenia(ITP)is an autoimmune disease mediated by anti-platelet autoantibodies.There is growing evidence that the eradication of Helicobacter pylori(H.pylori)effectively increases platelet count in a considerable proportion of ITP patients infected with this bacterium.In the majority of ITP patients responding to H.pylori eradication therapy,the anti-platelet autoantibody response is completely resolved with no relapse for more than 7 years,indicating that the disease is cured.Therefore,adult patients with suspected ITP should be examined for H.pylori infection,and eradication therapy is recommended if the infection is present.Notably,however,the efficacy of H.pylori eradication therapy in ITP patients varies widely among countries,with a higher response rate in Japan compared with the United States and European countries other than Italy.The pathogenesis of H.pylori-associated ITP is still uncertain,although the mechanisms are known to involve multiple factors.H.pylori may modulate the Fcγ-receptor balance of monocytes/macrophages in favor of activating Fcγreceptors,and H.pylori components may mimic the molecular makeup of platelet antigens.Further studies of the pathogenic process of H.pyloriassociated ITP may be useful for the development of new therapeutic strategies for ITP.
文摘<b>Background:</b> Management of immune thrombocytopenia (ITP) during pre- gnancy can be challenging, particularly by identifying a threshold for safe administration of neuraxial/general anesthesia and minimizing postpartum hemorrhage. There is controversy over the safety of cesarean section (CS) in ITP patients. In this case report, we discuss general anesthesia management in a patient with ITP with severe thrombocytopenia. <b>Case Presentation:</b> A 28-year-old female with relapsed/refractory ITP and severe thrombocytopenia underwent general anesthesia and emergent cesarean section with successful outcomes and minimal bleeding. Platelet counts before CS were 5000 × 10<sup>9</sup> L, the patient received 1 unit of platelets before the procedure and 1 unit of platelet and tranexamic acid 500 mg was injected slowly during the procedure. No evidence of bleeding and no complications were observed in the patient or newborn. <b>Conclusions:</b> In an emergent circumstance, general anesthesia and cesarean section procedure were performed safely in a patient with severe thrombocytopenia, no hemorrhagic complications were seen for this patient or neonate. <b>Objective of This Manuscript:</b> To share our experience of a safe emergent CS procedure and general anesthesia in a patient with severe thrombocytopenia. Our experience may guide the management of ITP patients in emergent delivery circumstances.
基金the National Program on Key Basic Research Project(2015CB554403)
文摘Objective: To investigate the biological basis of qi, blood and vessel in immune thrombocytopenia(ITP) patients with syndrome of qi failing to govern blood(SQFGB) based on traditional Chinese medicine.Methods: A total of 52 ITP patients with SQFCB were enrolled and divided into bleeding group(38 cases) and non-bleeding group(14 cases).Bleeding group was further divided into mild qi deficiency group(25 cases) and moderate/severe qi deficiency group(13 cases) based on Chinese Medicine syndrome score.20 healthy volunteer were recruited as control group.The count of platelet(PLT) was taken as the blood related indicator.The expressions of cytokines including IL-1β, IL-17 A, TNF-α, CD40 L, and TGF-β, detected by Aim Plex Multiple Immunoassays for Flow, were taken as the qi related indicators.The expressions of VEGF-A, detected by Aim Plex Multiple Immunoassays for Flow and NO, NOS, and ET-1 detected by ELISA, were taken as the vessel related indicators.Results: As compared to the control group, the count of PLT, taken as the blood related indicator, was significantly lower in ITP group patients with SQFCB(P<0.05).The expression levels of IL-17 A and TNF-α, taken as the qi related indicators, were significantly higher, while those of CD40 L, IL-1β, and TGF-β, also taken as the qi related indicators, were significantly lower in ITP patients with SQFCB, respectively(P<0.05).The expression levels of NO and ET-1, taken as the vessel related indicators, were significantly higher, while the expression levels of NOS and VEGF-A also taken as the vessel related indicators, were significantly lower in ITP patients with SQFCB, respectively(P<0.05).The count of PLT, taken as the blood related indicator, was significantly lower in moderate/severe group than those in mild group(P<0.05).The expression levels of CD40 L and TGF-β, taken as the qi related indicators, were also significantly lower in moderate/severe group than those in mild group, respectively(P<0.05).Conclusion: The count of PLT might be the biological basis of blood.The expressions of NO, NOS, ET-1 and VEGF-A might be the biological basis of vessel.The expressions of IL-1β, IL-17 A, TNF-α, TGF-β, and CD40 L may be the biological basis of qi.The expressions of CD40 L and TGF-β could reflect the degree of qi deficiency in ITP patients based on the theory of qi and blood.
文摘Prednisone is the most common first-line treatment for adult primary immune thrombocytopenia (ITP). However, the best initial therapeutic approach is still a matter of debate. Prior studies have shown that high-dose dexamethasone (HD-DXM) produces a high sustained efficacy not achieved by conventional prednisone therapy. However, the definition of response widely differs between individual reports, and this heterogeneity makes comparison of the efficacy difficult. The aim of our study was to compare the therapeutic outcomes of a conventional dose of prednisone with HD-DXM for adult ITP patients as initial therapy. Thirty patients treated with prednisone and 22 patients treated HD-DXM were retrospectively analyzed. No significant differences between the HD-DXM and prednisone groups were observed for the rates of complete response (68% vs. 70%) and response (18% vs. 17%). However, 1 year probability of sustained response was significantly greater in the HD-DXM group than in the prednisone group (78% vs. 38%;P = 0.008). No adverse events necessitating discontinuation of treatment were observed in either group. Our retrospective analysis showed that initial treatment with HD-DXM produced longer response duration compared to a conventional dose of prednisone. Randomized clinical trials are warranted to establish the optimal initial steroid therapy for adult ITP.
文摘BACKGROUND Vaccine-induced immune thrombotic thrombocytopenia(VITT)is a rare and potentially life-threatening condition after receiving coronavirus disease vaccines.It is characterized by symptom onset at 5 to 30 d postvaccination,thrombocytopenia,thrombosis,high D-dimer level,and antiplatelet factor 4(anti-PF4)antibody positivity.VITT can progress very fast,requiring urgent management.Only few studies have described its detailed clinical course and imaging changes.We report a typical VITT case in a patient who underwent regular repeated brain imaging examinations.CASE SUMMARY A young woman presented with headaches at 7 d after the ChAdOx1 nCoV-19vaccine(AZD1222)injection.She then showed progressive symptoms of left upper limb clumsiness.Brain computed tomography revealed venous infarction at the right parietal lobe with a hyperacute thrombus in the cortical vein.Two hours later,brain magnetic resonance imaging revealed hemorrhage at the same area.Magnetic resonance venography showed an irregular contour of the right transverse sinus.Laboratory examination revealed a high D-dimer level,thrombocytopenia,and a high titer for anti-PF4 antibodies.She was treated with anticoagulants,intravenous immunoglobulin,and steroids and analgesic agents were administered for pain control.She had a marked improvement on headaches and clumsiness after treatment along with radiological thrombus resolution.During follow-up at the outpatient department,her modified Rankin scale at 90 d was 1.CONCLUSION Clinicians should be alerted whenever patients present with persistent and progressive headaches or focal motor/sensory deficits postvaccination.
文摘Immune Thrombocytopenic purpura(ITP)is a haematologicimmune-mediated disorder in which the amount of platelet in the blood decreases abnormally.Single-agent therapies for ITP have not proven successful in achieving long-term remission,with relapse occurring in about half of the patients(p/t).Treatment options which include Rituximab with Dexamethasone as frontline therapy,have durable response rates ranging from 58%to 76%.In this study,we have used‘Total therapy’as treatment which includes low-dose Rituximab in combination with Thrombopoietin receptor agonist(TPO-RA)(Romiplostim)and high-dose Dexamethasone.In this case series study,each patient received romiplostim(250 mcg weekly s/c,4 doses)in combination with low-dose rituximab(100 mg weekly IV,4 doses)and high-dose dexamethasone(40 mgIV on days 1-4and days 15-18).This treatment combination demonstrated rapid response rates and a low rate of side effects,making it a good alternative for individuals with ITP.
文摘Objective:To analyze the hot spot and frontier of traditional Chinese medicine treatment for primary immune thrombocytopenia with CiteSpace softwar for visualized analysis.Methods:The relevant literature about treatment for ITP through traditional Chinese medicine was retrieved from CNKI.The CiteSpace software was used to create visualization map for authors,institutions and keywords.Results:Through visualization analysis,121 authors and 36 institutions were included in the map.The main research hot spot is the clinical effect of traditional Chinese medicine and integrated traditional Chinese and Western medicine in the treatment for ITP.Conclusion:Ratio of research about traditional Chinese medicine treatment on ITP was relatively few.In addition,the cooperation and communication were also insufficient between teams and institutions.The research hot spot mostly focuses on clinical treatment,while theoretical research was very few.There is no deep investigation on treatment mechanism.
文摘Immune thrombocytopenia is an autoimmune disease resulting in the destruction of platelets.It is classified as acute,thrombocytopenia occurring for < 6 mo and usually resolving spontaneously,and chronic,lasting >6 mo and requiring therapy to improve the thrombocytopenia.The underlying defects leading to autoantibody production are unknown.Molecular mimicry appears to play a role in the development of self-reactive platelet antibodies after vaccination and certain viral infections.Platelet life span is reduced as a consequence of antibody-mediated clearance by tissue macrophages in essentially all patients.Diagnosis is based on the exclusion of the other causes of thrombocytopenia.Steroid is the first choice of the treatment,often followed by splenectomy in unresponsive cases.Intravenous immunoglobulin,anti-Rho(D) immune globulin,azathioprine,cyclosporine A,cyclophosphamide,danazol,dapsone,mycophenolate mofetil,rituximab,thrombopoietin receptor agonists and vinca alkaloids are other choices of treatment.
