Púrpura fulminante postinfecciosa: a propósito de un caso / Postinfectious purpura fulminans: A case report

La púrpura fulminante adquirida postinfecciosa es una entidad aguda y grave, poco frecuente, caracterizada por necrosis cutánea asociada a coagulopatía intravascular diseminada (CID), en ausencia de infección activa o alteraciones previas de la coagulación. Afecta fundamentalmente a la población pediátrica y, en el 90 % de los casos, está precedida por un proceso infeccioso. El mecanismo fisiopatológico es un déficit transitorio de proteína S mediado por autoanticuerpos que favorece un estado de hipercoagulabilidad. Se presenta el caso de un varón de 8 años previamente sano, con lesiones cutáneas purpúricas características de púrpura fulminante asociada a CID en ausencia de sepsis. Se constató deficiencia plasmática transitoria de proteína S. Requirió tratamiento sustitutivo con plasma fresco congelado y anticoagulación; la evolución fue favorable. La actividad de la proteína S permaneció disminuida durante 2 meses.

Acquired postinfectious purpura fulminans is a rare, acute, and severe disease characterized by skin necrosis associated with disseminated intravascular coagulation (DIC) in the absence of active infection or previous coagulation disorders. It mainly affects the pediatric population and, in 90% of cases, it is preceded by an infectious process. The pathophysiological mechanism is a transient autoantibodymediated protein S deficiency that favors a hypercoagulable state. Here we describe the case of a previously healthy 8-year-old boy with purpuric skin lesions typical of purpura fulminans associated with DIC in the absence of sepsis. A transient plasma protein S deficiency was confirmed. He required replacement therapy with fresh frozen plasma and anticoagulation; he had a favorable course. Protein S activity remained decreased for 2 months.

Research progress of Hp infection and blood system related diseases / 重庆医学

Helicobacter Pylori(Hp)is a spiral bacterium that colonized on the surface of gastric muco-sal epithelium.It is the main cause of gastrointestinal diseases because human is the only natural host and can survive in gastric acid.In recent years,relevant clinical studies have shown that Hp infection is closely related to hematological diseases such as allergic purpura(HSP),immune thrombocytopenic purpura(ITP),iron de-ficiency anemia(IDA),megaloblastic anemia(MA),lymphoma,leukemia and so on.Therefore,for Hp infec-tion,early diagnosis and treatment are of great significance for improving the efficacy of hematological diseases.

Evaluation of ultrasonography combined with serum Gastrin-17 in differential diagnosis of abdominal IgA vasculitis in children / 国际检验医学杂志

Objective To study the value of ultrasonography combined with serum Gastrin-17 in differenti-al diagnosis of abdominal IgA vasculitis in children.Methods A total of 80 children with IgA vasculitis admit-ted to the hospital from June 2020 to December 2022 were selected,including 45 cases of abdominal IgA vascu-litis(observation group)and 35 cases of other types of IgA vasculitis(without gastrointestinal symptoms,control group).The ultrasonographic characteristics and Gastrin-17 level of abdominal IgA vasculitis were an-alyzed,and the relationship between Gastrin-17 level and purpura symptom score was analyzed.Receiver oper-ating characteristic(ROC)curve was used to analyze the diagnostic value of ultrasonography and Gastrin-17 for abdominal IgA vasculitis in children.Results The symptom score of purpura in the observation group was significantly higher than that in the control group(P<0.001),while the serum Gastrin-17 level in the obser-vation group was significantly lower than that in the control group.Pearson correlation analysis showed that serum Gastrin-17 level was negatively correlated with purpura symptom score(r=-0.758,P<0.001).Ul-trasound images showed"doughnut"-like changes in the intestinal wall,with different degrees of central-thick-ness thickening and reduced echo,mainly submucosal thickening.ROC curve analysis showed that the cut-off value of serum Gastrin-17 in the diagnosis of abdominal IgA vasculitis in children was 2.91 pmol/L,the area under the curve was 0.787(95%CI:0.685-0.888),the sensitivity and the specificity were 75.56%(34/45)and 74.29%(26/35),respectively.The sensitivity of ultrasound combined with Gastrin-17 in the diagnosis of abdominal IgA vasculitis in children was 97.78%(44/45),the negative prediction rate was 95.65%(22/23),and the accuracy rate was 82.50%(66/80),which was significantly higher than those of single diagnosis(P<0.05).Conclusion Serum Gastrin-17 level is low in children with abdominal IgA vasculitis,and ultrasound imaging shows"doughnut"-like changes in the intestinal wall and thickening of the submucosa.Combined de-tection of the two could effectively differentially diagnose abdominal IgA vasculitis in children.

Analysis of Vitamin D in Children with Henoch-Schonlein Purpura / 昆明医科大学学报

Objective To analyze the changes of vitamin D in children with Henoch-Schonlein purpura(HSP).Methods 130 children with HSP from Kunming Children's Hospital between July 2022-July 2023 were selected as the study subjects and 100 healthy children were selected during the same period as the control group.The blood samples were collected from the children with HSP and the healthy children.The content of vitamin D was measured by Kunming Kingmed Institute for Clinical Laboratory.Results The content of 25(OH)D in children with HSP was lower than that in healthy children,and the difference was statistically significant(P<0.01).The proportion of vitamin D insufficiency in children with HSP was higher than that in healthy children,and the difference was statistically significant(P<0.01).Conclusion The children with HSP are prone to vitamin D insufficiency.Vitamin D supplementation may provide a new method for the treatment of HSP.

Single-center controlled study for application of hemoperfusion and versus gammaglobulin for the treatment of Henoch-Sch?nlein purpura combined gastrointestinal bleeding in children / 中华实用儿科临床杂志

Objective:To compare the clinical efficacy and safety of hemoperfusion (HP) and gammaglobulin on the treatment of Henoch-Sch?nlein purpura (HSP) with gastrointestinal bleeding in children.Methods:Case-control study.A total of 39 HSP children combined with gastrointestinal bleeding diagnosed in the Department of Pediatric Nephrology, Rheumatology and Immunology, Shengjing Hospital of China Medical University from January 2015 to December 2019 were retrospectively recruited.They were divided into the HP group and the gammaglobulin group according to the therapeutic strategy.Clinical data were collected, and a 6-month follow-up survey was conducted for monitoring the relapse of gastrointestinal bleeding and the occurrence of kidney injury.The differences between groups were compared by Fisher′s exact test, two independent samples t-test, Mann-Whitney U-test, Kruskal-Wallis H-test, and One-Way ANOVA. Results:(1) There were 20 cases in the HP group and 19 cases were included in the gammaglobulin group.The gammaglobulin group was younger than the HP treatment group.(2) In addition to gastrointestinal bleeding, children in both groups had other clinical symptoms, such as abdominal pain, angioneurotic edema, and hematuria.(3)Comparison of laboratory indexes: Inflammatory indexes: white blood cell count (WBC), C-creative protein (CRP) and coagulation function indexes: fibrin degradation products (FDP), D-dimer (DD) were significantly elevated before treatment in the 2 groups, and there was no difference between the 2 groups ( P>0.05); WBC, CRP and FDP, DD declined in the 2 groups after treatment compared with the former, and there was no difference between the 2 groups ( P>0.05); (4) Comparison of clinical manifestations: when HP was applied with gammaglobulin in the treatment window within 3 d, the difference in the time of abdominal pain relief in the HP group was shorter than that of the gammaglobulin group [1.00(1.00, 1.00) d vs.2.00(1.75, 6.50) d, P=0.011]; comparing the time of gastrointestinal bleeding stopping when HP was applied with gammaglobulin comparison, the difference in gastrointestinal bleeding cessation time was not statistically significant ( P>0.05); (5) Comparison of hospitalization time: within 3 d application of HP compared with other window period hospitalization time were significantly reduced [(16.89±4.99) d than (19.20±2.39) d than (34.83±8.40) d, both P<0.05]; (6) Comparison of hospitalization costs: within 3 d application of HP compared with other window period hospitalization costs were significantly reduced [25 554.03 (22 168.61, 28 527.30) yuan than 33 619.48 (32 661.18, 36 971.47) yuan than 51 290.34 (34 163.04, 64 772.66) yuan, both P<0.05]; There were no statistically significant difference in the hospitalization time and hospitalization cost between and within the gammaglobulin group (all P>0.05); (7) Comparison of hormone dosages: the difference in the results of the initial dose of hormone use, pre-treatment dose of gammaglobulin/HP, and post-treatment dose of gammaglobulin/HP between the two groups of children was not statistically significant(all P>0.05). Safety profile was comparable between groups.The difference in hormone dosage before and after treatment within the gammaglobulin and HP treatment group was statistically different ( P<0.001). Conclusions:For children with severe HSP accompanied by gastrointestinal bleeding, early treatment with blood purification can rapidly relieve clinical symptoms and reduce the number of hospital days and hospitalization costs.For cases where blood purification is not available or suitable, gammaglobulin treatment is another option.

Literature Case Analysis of 44 Patients with Thrombocytopenia Induced by Clopidogrel / 医药导报

Objective To investigate the characteristics of clopidogrel-associated thrombocytopenia to provide references for clinically safe drug use.Methods The case reports of thrombocytopenia induced by clopidogrel published in PubMed,Embase,CNKI,Wanfang and VIP were searched from the establishment of each database to November 2022,and their occurrence was collated and analysed.Results A total of 44 cases from 43 articles were identified and included in the analysis.There were 30 males(68.2％)and 14 females(31.8％).Ages ranged from 37 to 88(65.0±11.4)years,of which 30(68.2％)were ≥60 years old.Thrombocytopenia was found from 8 h to 9 months after medication,of which 29 cases(65.9％)appeared within two weeks.There were 31 cases(70.5％)with severe thrombocytopenia and 38 cases(86.4％)with complications,of which 24 cases(63.2％)with bleeding and 19 cases(50.0％)with thrombotic thrombocytopenic purpura(TTP).The platelet countof41 cases(93.2％)returned to normal after drug withdrawal and symptomatic treatment,and 3 cases(6.8％)died finally.Conclusion Clopidogrel related thrombocytopenia is mainly severe thrombocytopenia,and often accompanied by bleeding or thrombotic thrombocytopenic purpura(TTP),but the overall outcome is good.Platelet count should be regularly monitored within the first two weeks after medication.Clopidogrel should be stopped and symptomatic treatment should be given in case of any abnormality.

Efecto de las vacunas inactivada y de ARNm contra la COVID-19 sobre la trombocitopenia en pacientes con trombocitopenia inmunitaria / Effect of inactivated and mRNA COVID-19 vaccines on thrombocytopenia in immune thrombocytopenia patients

Este estudio observacional retrospectivo tuvo como objetivo abordar los posibles efectos de las vacunas inactivada y de ARNm en pacientes con trombocitopenia inmunitaria relacionados con la exacerbación. Para definir exacerbación, se consideró una disminución de más del 30 por ciento en el recuento de plaquetas con respecto al valor basal o un recuento de plaquetas disminuido a menos de 30×109/L o el desarrollo de una nueva hemorragia. Cincuenta y nueve (hombres 30,5 por ciento, mujeres 69,5 por ciento) de 208 pacientes con trombocitopenia inmunitaria, se inscribieron en el estudio. La mediana de edad fue de 47 años (rango 18-86). Se realizó un total de 171 vacunaciones en 59 pacientes. El 38 por ciento y el 62 por ciento de los pacientes fueron vacunados con Sinovac® y BioNTech®, respectivamente. En total, 10 (16,9 por ciento) pacientes experimentaron una disminución del recuento de plaquetas por debajo de 30×109/L tras la vacunación. Durante el último año antes de la pandemia, 19 de la misma cohorte (32,2 por ciento) experimentaron dicha disminución. Después de la primera, segunda y la dosis de refuerzo de la vacunación, el 12,7 por ciento, 13,8 por ciento y 15 por ciento de los pacientes experimentaron exacerbaciones, respectivamente; las exacerbaciones con hemorragias leves fueron del 2,3 por ciento y todos los episodios hemorrágicos se trataron con éxito comenzando con esteroides o aumentando la dosis de esteroides. No se registró ninguna hemorragia grave o potencialmente mortal. Se documentó una diferencia estadística en la exacerbación en los pacientes vacunados con la vacuna de ARNm (p =0,041) sólo después de la primera dosis y los pacientes más jóvenes experimentaron una mayor tasa de exacerbación sin significación estadística (p=0,06) después de la primera dosis. En conclusión, tanto la vacuna de ARNm como la inactivada parecen ser seguras para los pacientes con trombocitopenia inmunitaria con complicaciones hemorrágicas poco frecuentes. Especialmente los pacientes más jóvenes y los vacunados con vacunas de ARNm deben ser objeto de un seguimiento estrecho durante 1-2 meses después de la vacunación para detectar trombocitopenia(AU)

This retrospective observational study was aimed to address the possible effects of inactivated and mRNA vaccines in immune thrombocytopenia patients related to exacerbation. To define exacerbation, more than 30percent decrease in platelet counts from baseline or platelet counts decreased to less than 30×109/L and/or development of new bleeding were considered. Fifty-nine (male 30.5percent, female 69.5percent) out of 208 immune thrombocytopenia patients, were enrolled in the study. The median age was 47 (range18-86). A total of 171 vaccinations were performed in 59 patients. Thirty-eight and 62percent of patients were vaccinated with Sinovac® and BioNTech®, respectively. Overall, 10 (16.9percent) patients experienced decrease in platelet count below 30×109/L after vaccination. During the last year before pandemic, 19 of the same cohort (32.2percent) experienced such decrease. After first, second and booster dose vaccinations, 12.7percent, 13.8percent and 15percent of patients experienced exacerbation respectively; exacerbation with minor bleeding was 2.3percent and all bleeding episodes were successfully treated by starting with steroid or increasing the steroid dose. We did not report any severe and life-threatening bleeding. A statistical difference in exacerbation was documented in patients vaccinated with mRNA vaccine (p =0.041) only after the first dose and younger patients experienced a higher rate of exacerbation without statistical significance (p=0.06) after the first dose. In conclusion, both mRNA and inactivated vaccines seem to be safe for immune thrombocytopenia patients with rare bleeding complications. Especially younger patients and those vaccinated with mRNA vaccines should be followed up closely for 1-2 months post vaccination for thrombocytopenia(AU)

Púrpura fulminante aguda concomitante con proceso infeccioso pulmonar

La púrpura fulminante o purpura fulminans es un síndrome de trombosis microvascular cutánea y necrosis hemorrágica de rápida evolución. Se presenta el caso de un paciente masculino, internado por patología infecciosa y evento cardiovascular agudo, que desarrolla púrpura fulminante por déficit de proteína C, relacionado a cuadro infeccioso concomitante.

Purpura fulminans is a rapidly evolving syndrome of cutaneous microvascular thrombosis and hemorrhagic necrosis. We present the case of a male patient, hospitalized for an infectious pathology and an acute cardiovascular event, who developed purpura fulminans due to protein C deficiency, related to a concomitant infectious condition.

Vasculitis por IgA del adulto. Caso clínico

La vasculitis por IgA, es la vasculitis más frecuente en pediatría. Puede presentarse en adultos, con una clínica y evolución diferente y un pronóstico más grave que en los niños, incluida la progresión a enfermedad renal terminal. La historia natural de la enfermedad y de la nefritis, ha sido poco estudiada en adultos; no se dispone de criterios diagnósticos universalmente aceptados y el tratamiento es controvertido, dada la ausencia de estudios controlados, randomizados que lo avalen. Se reporta el caso de un paciente que presentó un síndrome purpúrico petequial, microhematuria, proteinuria y una evolución rápida a la insuficiencia renal, de cuyo estudio etiológico surge el diagnóstico de vasculitis por IgA del adulto.

The IgA vasculitis is the most common vasculitis in Pediatrics. It can also present in adults but with a different clinical course and a worse prognosis, including the possibility of progression to end stage renal disease. The natural history of the disease and its nephritis have been scarcely studied in adults. There is no universal agreement in diagnostic criteria and the treatment is controversial given the absence of controlled randomized trials. We report the case of a patient who presented clinically with a petechial purpuric rash, microhematuria, proteinuria and rapid progression to renal failure that was diagnosed with IgA vasculitis in adult.

A vasculite por IgA é a vasculite mais comum em pediatria. Pode ocorrer em adultos, com apresentação e evolução clínica diferentes e prognóstico mais grave do que em crianças, incluindo progressão para doença renal terminal. A história natural da doença e da nefrite tem sido pouco estudada em adultos; Não existem critérios diagnósticos universalmente aceitos e o tratamento é controverso, dada a ausência de estudos controlados e randomizados que o apoiem. É relatado o caso de um paciente que apresentou síndrome purpúrica petequial, microhematúria, proteinúria e rápida evolução para insuficiência renal, de cujo estudo etiológico surge o diagnóstico de vasculite por IgA do adulto.

Síndrome de la bolsa de orina púrpura

Introducción: El síndrome de la bolsa orina púrpura es una condición llamativa, que rara vez se presenta en la práctica clínica. Aparece en el contexto de infecciones urinarias en ancianos, pluripatológicos, con sonda vesical y factores de riesgo asociados. Se produce por una reacción química entre la orina, el material plástico de la bolsa colectora y enzimas sulfatasas/fosfatasas de bacterias que generan el color violáceo característico. Objetivo: Reportar un caso con síndrome de la bolsa orina púrpura, como forma de presentación inusual de infección urinaria. Caso Clínico: Paciente femenina, de 76 años de edad, con antecedentes de constipación habitual, acudió a urgencias por pérdida del conocimiento y hemiparesia derecha. Se realizó tomografía axial computarizada de cráneo y se diagnosticó una enfermedad cerebrovascular. Como parte de la conducta se indicó sonda vesical y 14 días después apareció orina de color violeta en la bolsa colectora. Se diagnosticó infección urinaria por Escherichia coli y se trató con ceftriaxona. Se normalizó el color de la orina al tercer día de tratamiento, la paciente evolucionó de forma favorable. Conclusiones: Aunque se puede identificar con facilidad, sin requerir exámenes costosos, constituye un reto para los médicos que atienden a pacientes geriátricos. Conocer este trastorno es fundamental, porque, aunque es infrecuente, puede ser la única manifestación de infección urinaria en pacientes con cateterismo uretral.

Introduction: Purple urine bag syndrome is a striking condition that rarely occurs in clinical practice. It appears in the context of urinary infections in the elderly, with multiple pathologies, with a bladder catheter and associated risk factors. It is produced by a chemical reaction between urine, the plastic material of the collection bag and sulfatase/phosphatase enzymes from bacteria that generate the characteristic purple color. Objective: To report a case with purple urine bag syndrome as an unusual presentation of urinary tract infection. Clinical Case: Female patient, 76 years old, health history, usual constipation, attended at Emergency due to loss of consciousness and right hemiparesis. Computed axial tomography of the skull was performed and a cerebrovascular disease was diagnosed. As part of the conduct, a bladder catheter was indicated and after 14 days, purple urine appeared in the collection bag. Urinary infection due to Escherichia coli was diagnosed and treated with ceftriaxone, normalizing the color of the urine on the third day of treatment, with patient favorable evolution. Conclusions: Although it can be easily identified and without requiring costly tests, it is a challenge for physicians who care for geriatric patients. Knowing this disorder is essential because, although rare, it may be the only manifestation of urinary infection in patients with urethral catheterization.

Schõnlein-Henoch purpura in adults: Report of four cases

Schoenlein-Henoch purpura is a systemic small vessel vasculitis mediated by IgA-1 deposition in organs such as the skin, kidney, and gastrointestinal tract; it has been mainly described in children where it has a favourable prognosis. Although much rarer in adulthood it is associated with an increased risk of severe kidney involvement, gastrointestinal com-plications, and prolonged hospital stay. The therapeutic options are wide and vary according to the degree of involvement of the patient and the organ mainly affected.

La púrpura de Schönlein-Henoch es una vasculitis sistêmica de pequeno vaso mediada por depósito de IgA en órganos como la piel, el riñón y el tracto gastrointestinal. Se ha descrito principalmente en niños, grupo de población en el que tiene un pronóstico favorable. Si bien en la edad adulta es mucho menos frecuente, se asocia con un mayor riesgo de compromiso renal severo, complicaciones gastrointestinales y estancia hospitalaria prolongada. Las opciones terapêuticas son amplias y varían según el grado de compromiso del paciente y el órgano más afectado.

Safety and efficacy of romiplostim in children and adolescents with Immune thrombocytopenia: A systematic review

Abstract Objective To evaluate the efficacy and safety of romiplostim (thrombopoietin-receptor agonist) in the treatment of pediatric immune thrombocytopenia (ITP). Methods Searches were conducted in MEDLINE, EMBASE, LILACS, Cochrane Central Register of Controlled Trials and ClinicalTrials.gov (from January 2011 to August 2021). Randomized controlled trials (RCTs), double-blind, comparing romiplostim with a placebo in pediatric persistent or chronic ITP were included. The primary outcome was the overall response rate (platelets ≥ 50 × 109/L) in the absence of rescue therapy for at least two consecutive weeks. The secondary endpoints were the minimization of clinically significant bleeding and the necessity for rescue treatments and the maximization of safety (incidence of overall adverse events) and durable response (maintaining platelet counts for at least twelve weeks). Results Two double-blind randomized placebo-controlled trials (84 participants) were included in this systematic review. Our data showed that, compared to the placebo group, the proportion of patients achieving durable platelet response was significantly higher in the romiplostim group (p= 0.003, RR = 6.34, 95%CI = 1.89 - 21.23), as was the overall response in the romiplostim group (p= 0.002, RR = 3.62, 95%CI = 1.63 - 8.03). Significant bleeding incidents (p= 0.49), overall adverse events (p= 0.71) and the need for rescue treatment (p= 0.13) were not statistically different between the romiplostim and placebo groups. Conclusions Romiplostim might improve both durable and overall platelet response in children and adolescents with ITP, compared to a placebo. More clinical trials are needed to evaluate the efficacy and safety of romiplostim and to compare it with other second-line treatments that are being used in pediatric ITP.

Factores asociados a dermatoporosis en una muestra de pacientes geriátricos en México / Factors associated with dermatoporosis in a sample of geriatric patients in Mexico

Resumen Introducción: La dermatoporosis es un síndrome crónico de fragilidad cutánea, caracterizado por atrofia, púrpura y pseudocicatrices en piel. Objetivo: Determinar los factores asociados a dermatoporosis en una muestra de sujetos ≥ 60 años. Métodos: Estudio observacional, transversal, descriptivo y analítico de sujetos ≥ 60 años a quienes se realizó historia clínica, exploración física y aplicación de un autocuestionario diagnóstico de dermatoporosis. Para determinar los factores asociados se realizó análisis de regresión logística multivariado. Resultados: En 315 sujetos, la prevalencia de dermatoporosis fue de 29 %; 70 % fue del sexo femenino. Los factores asociados fueron edad > 75 años (p = 0.001), exposición solar prolongada (p = 0.002), ingesta de anticoagulantes/antiplaquetarios (p = 0.004), esteroides orales (p = 0.03) y enfermedad renal crónica (p = 0.03); así como, edad materna > 40 años en el último parto (p = 0.02), lactancia > 7 meses por embarazo y lactancia acumulada > 18 meses (p = 0.01). Se relacionaron con su ausencia, edad < 20 años en el primer embarazo y menopausia después de los 45 años. La correlación entre la autovaloración y el diagnóstico clínico fue muy alta (0.95, p < 0.001). Conclusiones: Los factores de riesgo asociados a dermatoporosis fueron similares a los previamente reportados.

Abstract Introduction: Dermatoporosis is a chronic cutaneous fragility syndrome, characterized by skin atrophy, purpura and pseudo-cicatrices. Objective: To determine factors associated with dermatoporosis in a sample of subjects aged ≥ 60 years. Methods: Observational, cross-sectional, descriptive, analytical study of subjects aged ≥ 60 years who underwent history taking, physical examination and application of a self-administered dermatoporosis diagnostic questionnaire. To determine the associated factors, a multivariate logistic regression analysis was used. Results: In 315 evaluated subjects, the prevalence of dermatoporosis was 29%; 70% were females. Associated risk factors were age > 75 years (p = 0.001), prolonged sun exposure (p = 0.002), use of anticoagulants/antiplatelet medications (p = 0.004), oral steroids (p = 0.03) and chronic kidney disease (p = 0.03); as well maternal age > 40 years at last pregnancy (p = 0.02), breastfeeding for > 7 months per pregnancy and > 18 cumulative months (p = 0.01). Age < 20 years at first pregnancy and menopause after 45 years were related to dermatoporosis absence. The correlation between self-assessment and clinical diagnosis was considerably high (0.95, p < 0.001). Conclusions: The risk factors associated with dermatoporosis were similar to those previously reported.

Cytomegalovirus Infection-related Immune Thrombocytopenia: A Case Report / Púrpura Trombocitopênica Imune Associada à Infecção por Citomegalovírus: Relato de Caso

Immune thrombocytopenia (ITP) is an acquired cause of thrombocytopenia characterized by the presence of autoantibodies against platelets. It may be primary or secondary to several conditions. We present the case of a 63-year-old woman with a diagnosis of immune thrombocytopenia refractory to conventional therapy. After she was tested for secondary causes of ITP, a diagnosis of acute cytomegalovirus (CMV) infection was made. She was treated with ganciclovir and presented normalization of platelet count. CMV-related Immune Thrombocytopenia should always be considered in certain cases of refractory ITP. If the diagnosis of ITP secondary to acute CMV infection is made, specific antiviral therapy with ganciclovir should be considered. In these cases, immunosuppressive agents, such as steroids, may worsen the ITP and should be tapered or withdrawn as rapidly as feasible.

A Púrpura Trombocitopênica Imune (PTI) é uma causa de trombocitopenia adquirida caracterizada pela presença de autoanticorpos contra plaquetas. A doença pode ser primária ou secundária a diversas condições. Apresentamos o caso de uma mulher de 63 anos com diagnóstico de PTI refratária à terapêutica convencional. A investigação de causas secundárias evidenciou infecção aguda por citomegalovírus (CMV). A paciente foi tratada com ganciclovir e evoluiu com normalização no nível de plaquetas. A PTI relacionada ao CMV deve sempre ser investigada em pacientes com PTI refratária, sendo a terapia antiviral específica com ganciclovir o tratamento de escolha. Nestes casos, os agentes imunossupressores, como os corticosteroides, podem piorar a PTI e devem ser reduzidos gradualmente ou retirados o mais rapidamente possível.

iTRAQ-based Proteomic Analysis of Serum Differential Proteins from Henoch-Schönlein Purpura Patients Before and After Qishun Baolier Treatment / 中国实验方剂学杂志

ObjectiveHenoch-Schönlein purpura（HSP） is one of the dominant diseases in Mongolian medicine. Qishun Baolier（QSBLE）， as the main prescription for the treatment of HSP， has significant clinical effect， but its mechanism is not yet clear. Baed on this， this study is intended to screen the differentially expressed proteins before and after treatment， and preliminarily explore the molecular mechanism of QSBLE in the treatment of HSP. MethodTaking oneself as the control， 30 HSP patients aged 6-45 years were collected， and QSBLE was taken orally at 12：00 and 24：00， respectively. The dose was adjusted according to age and the course of treatment was one week. The distribution of proteinuria， hematuria and skin purpura of all patients were determined before and after treatment. The serum samples of 10 patients with clinically significant remission after QSBLE treatment were randomly selected for proteomics. Isobaric tags for relative and absolute quantification（iTRAQ） combined with liquid chromatography tandem mass spectrometry（LC-MS/MS） was used to analyze the proteins in serum of HSP patients before and after treatment， and differential proteins were analyzed bioinformatically and the protein-protein interaction（PPI） networks were constructed. ResultA total of 378 proteins were identified from serum， including 18 differentially expressed proteins， of which 15 proteins were up-regulated and 3 proteins were down regulated. Bioinformatics showed that the differential proteins were mainly involved in biological processes such as immune response， immunoglobulin production， phagocytosis， adaptive immune response before and after treatment. Biological processes， pathways and proteins were used to construct the PPI network， the proteins represented by immunoglobulin heavy constant γ1（IGHG1）， immunoglobulin λ-chain 7-43（IGLV7-43）， gelsolin（GSN） and 60 kDa heat shock protein（HSPD1） were involved in biological processes and related pathways such as adaptive immune response， immunoglobulin production， leukocyte-mediated immunity， regulation of stress response， regulation of immune system processes， regulation of trauma response， and these proteins were at the center of the PPI network. ConclusionQSBLE may play a role in the treatment of HSP by regulating the expression of IGHG1， IGLV7-43， GSN， HSPD1 and other key proteins to affect immune-related biological processes.

Clinical Efficacy of Niaoxue No.1 Prescription in Treatment of Henoch-Schönlein Purpura Nephritis with Blood Heat and Stasis Syndrome in Children / 中国实验方剂学杂志

ObjectiveTo investigate the clinical efficacy of Niaoxue No.1 Prescription in treating Henoch-Schönlein purpura （HSP） nephritis with blood heat and stasis syndrome and its effect on urine erythrocyte， urine protein， blood neutrophils， and blood routine-derived indicators. MethodA multicenter， randomized controlled trial （RCT） was conducted involving 108 HSP nephritis patients from three hospitals. The patients were randomly divided into a control group （54 cases） and a treatment group （54 cases）. The treatment group received Niaoxue No.1 prescription once daily， while the control group was treated with captopril and ferulic acid tablets. Both groups underwent a 4-week course of treatment. The urine erythrocyte， urine microalbumin （mAlb）， urine sediment red blood cell count， traditional Chinese medicine （TCM） syndrome score， 24-hour urine protein， blood neutrophil count， neutrophil to lymphocyte ratio （NLR）， platelet to lymphocyte ratio （PLR）， lymphocyte to monocyte ratio （LMR）， D-dimer， and immunoglobulin A were detected. The recurrence rate of HSP nephritis was followed up for 6 months. ResultThe total effective rates were 88.9% （48/54） in the treatment group and 70.4% （38/54） in the control group， and the treatment group was superior to the control group （χ2=5.708， P<0.05）. Compared with the results before treatment， after 14 days of treatment， the TCM syndrome total score， urine erythrocyte， urine mAlb， and 24-hour urine protein in both groups significantly decreased （P<0.05，P<0.01）， and the improvement was more significant in the treatment group than the control group （P<0.05）. After 28 days of treatment， compared with the results before treatment， the TCM syndrome total score， urine erythrocyte， urine mAlb， urine sediment red blood cell count， D-dimer， and 24-hour urine protein in both groups significantly decreased （P<0.05，P<0.01）， with the treatment group showing a more significant reduction in urine mAlb than the control group （P<0.05）. On the 14th and 28th days of treatment， the neutrophil percentage and NLR were lower in the treatment group than in the control group （P<0.05）， while there was no statistically significant difference in PLR and LMR. The recurrence rate of nephritis in both groups showed no statistically significant difference after a 6-month follow-up. ConclusionNiaoxue No.1 Prescription in the treatment of HSP nephritis with blood heat and stasis syndrome can significantly improve clinical symptoms， shorten the course of the disease， and reduce urine erythrocyte， urine mAlb， 24-hour urine protein， blood neutrophils， and NLR， thereby effectively alleviating the inflammatory state and reducing kidney damage in children with HSP nephritis.

A combined regimen based on bortezomib and glucocorticoids for 6 patients with recurrent/refractory immune thrombotic thrombocytopenic purpura / 中华血液学杂志

Objective: To observe the efficacy and adverse reactions of a combination therapy regimen based on bortezomib and glucocorticoids in recurrent/refractory immune thrombocytopenic purpura (iTTP) . Methods: Six patients with recurrent/refractory TTP were included and treated with a glucocorticoid and two courses of bortezomib-based regimen. The clinical remission status of patients, changes in ADAMTS13 activity/ADAMTS13 inhibitor, and the occurrence of treatment-related adverse reactions were observed. Results: Of the 6 patients, 2 were males and 4 were females, with a median age of 21.5 (18-68) years. Refractory TTP was found in 1 case and recurrent TTP in 5 cases. Glucocorticoids were administered with reference to prednisone at 1 mg·kg(-1)·d(-1), and gradually reduced in dosage after achieving clinical remission. Bortezomib is subcutaneously administered at 1.3 mg/m(2) on days 1, 4, 8, and 11 with a 28-day treatment course consisting of 2 courses. Six patients achieved clinical remission after receiving bortezomib as the main treatment. ADMATS13 activity returned to normal in all patients with TTP after treatment, and the ADAMTS13 inhibitor turned negative. Thrombocytopenia is the most common adverse reaction after treatment, with other adverse reactions, including peripheral neuritis and abdominal pain, but ultimately all patients returned to normal. In a median follow-up of 26 (9-41) months, 5 patients maintained sustained remission, and 1 patient relapsed after 16 months of bortezomib treatment. Conclusion: Combination therapy of bortezomib and glucocorticoids has a satisfactory therapeutic effect and controllable adverse reactions for recurrent/refractory iTTP.

Progress on diagnosis and treatment of kidney transplantation-associated thrombotic microangiopathy / 器官移植

Thrombotic microangiopathy (TMA) is a severe complication after kidney transplantation, mainly characterized by thrombocytopenia, microvascular hemolytic anemia and acute kidney injury, which may lead to kidney allograft failure or even death of the recipients. With the increasing quantity of solid organ transplantation in China and deeper understanding of TMA, relevant in-depth studies have been gradually carried out. Kidney transplantation-associated TMA is characterized with different causes and clinical manifestations. Non-invasive specific detection approach is still lacking. The diagnosis of TMA mainly depends on renal biopsy. However, most TMA patients are complicated with significant thrombocytopenia. Hence, renal puncture is a risky procedure. It is difficult to make a definite diagnosis. For kidney transplantation-associated TMA, plasma exchange, intravenous immunoglobulin and withdrawal of potential risk drugs are commonly employed. Nevertheless, the overall prognosis is poor. In this article, the classification of TMA after kidney transplantation, diagnosis and treatment of kidney transplantation-associated TMA were reviewed, aiming to provide reference for clinical diagnosis and treatment of kidney transplantation-associated TMA.

The Osseous Pathology of Purpura Fulminans in a TwoYear-Old Child: A Case Report

@#Purpura fulminans (PF) is a severe clinical manifestation of Neisseria meningitides infection that is associated with high mortality rates in children. Survivors are frequently left with debilitating musculoskeletal sequelae. There is a paucity of reports on the musculoskeletal pathology of purpura fulminans. We report on a 2-year-old boy with purpura fulminans due to meningococcemia. The child developed distal gangrene in both the upper and lower limbs. Amputations were done for both lower limbs. Histological examination of the amputated specimens showed an inflammatory process and features of osteonecrosis. The latest follow-up at the age of 6 years showed a right knee valgus due to asymmetrical growth arrest of the proximal tibia. PF and its complications are challenging to treat and may require a multidisciplinary approach to improve patient’s functional ability.

Potential components and mechanism of Liangxue Tuezi Mixture in treating Henoch-Schönlein purpura based on network pharmacology and metabolomics / 中国中药杂志

Ultra-performance liquid chromatography-quadrupole time of fight/mass spectrometry(UPLC-Q-TOF-MS) and UNIFI were employed to rapidly determine the content of the components in Liangxue Tuizi Mixture. The targets of the active components and Henoch-Schönlein purpura(HSP) were obtained from SwissTargetPrediction, Online Mendelian Inheritance in Man(OMIM), and GeneCards. A "component-target-disease" network and a protein-protein interaction(PPI) network were constructed. Gene Ontology(GO) functional annotation and Kyoto Encyclopedia of Genes and Genomes(KEGG) pathway enrichment analysis were performed for the targets by Omishare. The interactions between the potential active components and the core targets were verified by molecular docking. Furthermore, rats were randomly assigned into a normal group, a model group, and low-, medium-, and high-dose Liangxue Tuizi Mixture groups. Non-targeted metabolomics was employed to screen the differential metabolites in the serum, analyze possible metabolic pathways, and construct the "component-target-differential metabolite" network. A total of 45 components of Liangxue Tuizi Mixture were identified, and 145 potential targets for the treatment of HSP were predicted. The main signaling pathways enriched included resistance to epidermal growth factor receptor tyrosine kinase inhibitors, phosphatidylinositol 3-kinase/protein kinase B(PI3K-AKT), and T cell receptor. The results of molecular docking showed that the active components in Liangxue Tuizi Mixture had strong binding ability with the key target proteins. A total of 13 differential metabolites in the serum were screened out, which shared 27 common targets with active components. The progression of HSP was related to metabolic abnormalities of glycerophospholipid and sphingolipid. The results indicate that the components in Liangxue Tuizi Mixture mainly treats HSP by regulating inflammation and immunity, providing a scientific basis for rational drug use in clinical practice.