2.Thrombocytopenia with absent radii syndrome: a case report.
Wen-ying ZHANG ; Chun-xiao FANG ; Tie-zhen YE
Chinese Journal of Pediatrics 2010;48(8):633-634
Humans
;
Infant
;
Male
;
Radius
;
abnormalities
;
Syndrome
;
Thrombocytopenia
;
complications
4.Interpretation of the Guidance for Immune Thrombocytopenia-Review.
Xiao-Yang YANG ; Meng-Jie WAN ; Fang-Ping CHEN
Journal of Experimental Hematology 2018;26(2):621-625
Since the American Medical Association published the 2011 guidelines for immune thrombocytopenia, China has been the first to update the guidelines for immune thrombocytopenia based on evidence-based medicine. Recently, there have been many breakthroughs in clinical research published, especially the Chinese medical workers have made a prominent contribution to the treatment of the immune thrombocytopenia. However, the references of systematic drug introduction for children, adults, aged and pregnant women are still insufficient, and the first or second line treatment for some patients were ineffective. Therefore, we tried to combine the references to interpret the guidelines, to explore the advantages and disadvantages of each treatment, to find out the bottleneck of clinical treatment, so as to facilitate the implementation and understanding of the guidelines, then update the next guideline.
China
;
Female
;
Humans
;
Pregnancy
;
Pregnancy Complications, Hematologic
;
Thrombocytopenia
;
United States
5.The epidemiology and pathogeny investigation of two clusters of severe fever with thrombocytopenia syndrome disease outbreaking in Henan Province, 2022.
Yi LI ; Xiao Yang WANG ; Ya Fei LI ; Dong Xiao LI ; Xiao HU ; Lin ZHU ; Ai Guo YOU ; Hai Feng WANG ; Ying YE ; Wan Shen GUO ; Xue Yong HUANG
Chinese Journal of Preventive Medicine 2023;57(10):1719-1724
To investigate two clusters of severe fever with thrombocytopenia syndrome virus (SFTSV) in Xinyang City, Henan Province, in 2022, and analyze their causes, transmission route, risk factors, and the characteristics of virus genetic variation. Case search and case investigation were carried out according to the case definition. Blood samples from cases, family members and neighbors and samples of biological vectors were collected for RT-PCR to detect SFTSV. The whole genome sequencing and bioinformatics analysis were performed on the collected positive samples. A total of two clustered outbreaks occurred, involving two initial cases and ten secondary cases, all of which were family recurrent cases. Among them, nine secondary cases had close contact with the blood of the initial case, and it was determined that close contact with blood was the main risk factor for the two clustered outbreaks. After genome sequencing analysis, we found that the SFTSV genotype in two cases was type A, which was closely related to previous endemic strains in Xinyang. The nucleotide sequence of the SFTSV in the case was highly homologous, with a total of nine amino acid mutation sites in the coding region. It was not ruled out that its mutation sites might have an impact on the outbreak of the epidemic.
Humans
;
Severe Fever with Thrombocytopenia Syndrome/epidemiology*
;
Bunyaviridae Infections/epidemiology*
;
Thrombocytopenia/complications*
;
Phlebovirus/genetics*
;
Disease Outbreaks
;
China/epidemiology*
6.The epidemiology and pathogeny investigation of two clusters of severe fever with thrombocytopenia syndrome disease outbreaking in Henan Province, 2022.
Yi LI ; Xiao Yang WANG ; Ya Fei LI ; Dong Xiao LI ; Xiao HU ; Lin ZHU ; Ai Guo YOU ; Hai Feng WANG ; Ying YE ; Wan Shen GUO ; Xue Yong HUANG
Chinese Journal of Preventive Medicine 2023;57(10):1719-1724
To investigate two clusters of severe fever with thrombocytopenia syndrome virus (SFTSV) in Xinyang City, Henan Province, in 2022, and analyze their causes, transmission route, risk factors, and the characteristics of virus genetic variation. Case search and case investigation were carried out according to the case definition. Blood samples from cases, family members and neighbors and samples of biological vectors were collected for RT-PCR to detect SFTSV. The whole genome sequencing and bioinformatics analysis were performed on the collected positive samples. A total of two clustered outbreaks occurred, involving two initial cases and ten secondary cases, all of which were family recurrent cases. Among them, nine secondary cases had close contact with the blood of the initial case, and it was determined that close contact with blood was the main risk factor for the two clustered outbreaks. After genome sequencing analysis, we found that the SFTSV genotype in two cases was type A, which was closely related to previous endemic strains in Xinyang. The nucleotide sequence of the SFTSV in the case was highly homologous, with a total of nine amino acid mutation sites in the coding region. It was not ruled out that its mutation sites might have an impact on the outbreak of the epidemic.
Humans
;
Severe Fever with Thrombocytopenia Syndrome/epidemiology*
;
Bunyaviridae Infections/epidemiology*
;
Thrombocytopenia/complications*
;
Phlebovirus/genetics*
;
Disease Outbreaks
;
China/epidemiology*
8.Chinese expert consensus on the clinical management of liver disease-associated thrombocytopenia syndrome.
Chinese Journal of Hepatology 2023;31(11):1137-1148
Liver disease-associated thrombocytopenia syndrome refers to thrombocytopenia caused by liver disease or the treatment of liver disease, and its incidence rate is related to the duration and severity of liver disease. The direct effect of thrombocytopenia on clinical outcomes is an increased risk of bleeding in patients with liver disease, whereas the indirect effect involves delay or termination of treatment due to the potential risk of bleeding. Liver disease-associated thrombocytopenia pathophysiological mechanisms involve decreased platelet production, abnormal distribution, destruction, or increased consumption. Presently, treatment strategies targeting different mechanisms include platelet-stimulating drugs, surgery, immunosuppressive drugs, and platelet transfusion, but the clinical application needs to be standardized further. The National Clinical Research Center for Infectious Diseases organized experts to discuss and formulate consensus with reference to the latest evidence-based medical evidence in the field so as to improve the clinical management level of liver disease-associated thrombocytopenia syndrome in China in terms of diagnosis, typing, and reasonable selection of treatment schemes.
Humans
;
Consensus
;
Thrombocytopenia/complications*
;
Liver Diseases/complications*
;
Hemorrhage/etiology*
;
Blood Platelets
10.Clinical analysis of 5 cases of dermatomyositis complicated with macrophage activation syndrome.
Xiao Yan XING ; Jun Xiao ZHANG ; Fen Yun Zhi ZHU ; Yi Fan WANG ; Xin Yao ZHOU ; Yu Hui LI
Journal of Peking University(Health Sciences) 2022;54(6):1214-1218
To investigate the clinical and immunological features of dermatomyositis (DM) complicated with macrophage activation syndrome (MAS). The demographic and clinical characteristics of five patients diagnosed with DM complicated with MAS hospitalized in the Department of Rheumatology and Immunology, Peking University People ' s Hospital from 2011 to 2021 were collected. The results of clinical manifestations, laboratory tests, immunological features, treatments and prognosis were analyzed and summarized. In this study, five female patients in Peking University People's Hospital with an average age of 63.8 (44.0-83.0) years and an average disease duration of 16.1 (1.5-48.0) months. All the patients had typical DM rash (such as heliotrope sign, V/shawl sign or Gottron's sign/papules). They all had muscle involvement (including myalgia or muscle weakness). Two patients had positive myositis-specific antibodies (MSAs), in which case 1 had anti-TIF1-γ antibody and case 5 had anti-NXP-2 antibody. Four patients had interstitial lung disease except case 3. All of the cases developed MAS in the active stage of DM. Common manifestations of MAS in these five patients included high-grade fever, cytopenia, decreased fibrinogen, elevated ferritin and increased soluble CD25. Case 1 presented with neutropenia (0.6×109 /L), thrombocytopenia (26.0×109 /L), hypofibrinogenemia (0.9 g/L), markedly elevated ferritin (26 331.0 μg/L), decreased NK cell activity. Case 2 had anaemia (hemoglobin 81.0 g/L), thrombocytopenia (55.0×109 /L), hypertriglyceridemia (4.7 mmol/L), hypofibrinogenemia (1.2 g/L), elevated ferritin (>100 000.0 μg/L), hemophagocytosis in bone marrow. Case 3 had anaemia (hemoglobin 88 g/L), decreased fibrinogen (1.9 g/L), increased ferritin (>27 759.0 μg/L), splenomegaly, hemophagocytosis in bone marrow. Case 4 suffered from neutropenia(0.3×109 /L), anaemia(hemoglobin 78 g/L), hypertriglyceridemia (4.2 mmol/L), hypofibrinogenemia (0.9 g/L), increased ferritin (>100 000.0 μg/L), and decreased NK cell activity. Case 5 presented anaemia (hemoglobin 60.0 g/L), thrombocytopenia (67.0×109 /L), hypertriglyceridemia (12.7 mmol/L), decreased fibrinogen (1.1 g/L), and elevated ferritin (>923.0 μg/L). All the patients were treated with methylprednisone pulse therapy (200-500 mg) combined with cyclosporine while case 5 received rituximab after methylprednisone pulses. In addition, case 3 also received the combination of mycophenolate mofetil. Case 1 was given etoposide while case 4 was treated with cyclophosphamide and repeated plasmapheresis at the same time. Moreover, intravenous immunoglobulin was added meantime apart from case 3. The condition of four patients improved significantly, nevertheless case 4 experienced recurred pulmonary symptoms and died of respiratory failure. As for complications about infection, case 2 had bacterial infection with high level procalcitonin (PCT) before MAS treatment and condition was improved after empiric antibacterial therapy. Case 3 had cytomegalovirus DNAemia before diagnosis of MAS and viral titer turned negative after ganciclovir therapy. After treatment of MAS, four patients developed cytomegalovirus DNAemia except case 3, in which case 5 was co-infected with bacteria. To sum, DM complicated with MAS is relatively rare, and its patients are of ten in life-threatening condition. Early detection, treatment and prevention of infection during treatment are critical to improve the prognosis.
Humans
;
Female
;
Middle Aged
;
Dermatomyositis/complications*
;
Macrophage Activation Syndrome/complications*
;
Afibrinogenemia/complications*
;
Autoantibodies
;
Neutropenia
;
Thrombocytopenia/complications*
;
Ferritins/therapeutic use*
;
Hypertriglyceridemia/complications*
;
Fibrinogen/therapeutic use*