Nuevos paradigmas en la hemofilia / New paradigms in hemophilia

Resumen La hemofilia es una diátesis hemorrágica producida por la deficiencia hereditaria de un factor (proteína) de la coagulación sanguínea que afecta principalmente a los varones. Su grado de severidad puede variar desde casos con poco sangrado, hasta condiciones muy graves que en muchas ocasiones llevan a la muerte a los enfermos. Existen dos tipos de hemofilia: la A por carencia del factor VIII y la B por falta del factor IX. En este editorial se resume de manera global la situación actual de los avances de la hemofilia desde el punto de vista clínico y del laboratorio.

Abstract Hemophilia is a hemorrhagic diathesis that is caused by the hereditary deficiency of a factor (protein) of blood clotting and that affects mainly men. Its degree of severity can vary from cases with little bleeding, to very serious conditions that often lead to death. There are two types of hemophilia, A for lack of factor VIII, and B for lack of factor IX. This editorial summarizes the current state of progress of hemophilia from the clinical and laboratory point of view.

Anticoagulantes, Antiplaquetarios y sus implicaciones en cirugía: una revisión de la literatura / Anticoagulants, Anti-platelets, and their implications in Surgery: A Literature review

All health care providers should be aware of the impact of bleeding disorders on their patients during any surgical procedures. The knowledge of the mechanisms of hemostasis and optimized management are very important. Initial recognition of a bleeding disorder, in such patients with a systemic pathologic process, may occur in surgical practice. The surgical treatment of those patients might be complicated during the surgery due to the use of anticoagulant and/or antiplatelet medications raises a challenge in the daily practice of surgical professionals. Adequate hemostasis is critical for the success of any surgical procedure because bleeding problems can give rise to complications associated with important morbidity-mortality. Besides, prophylactic, restorative, and surgical care of patients with any bleeding disorders is handled skillfully by practitioners who are well educated regarding the pathology, complications which could arise, and surgical options associated with these conditions. The purpose of this paper is to review common bleeding disorders and their effects on the surgical aspect. Many authors consider that patient medication indicated for the treatment of background disease should not be altered or suspended unless so indicated by the prescribing physician. Local hemostatic measures have been shown to suffice for controlling possible bleeding problems resulting from surgery.

Diagnóstico de enfermagem risco de sangramento como indicador de qualidade assistencial à segurança de pacientes / Diagnóstico de enfermería riesgo de sangrado como indicador de calidad asistencial de la seguridad de pacientes / Nursing diagnosis risk for bleeding as an indicator of quality of care for patient safety

Resumo OBJETIVO Descrever a implantação de um indicador de qualidade assistencial associado ao diagnóstico de enfermagem de pacientes com alto risco de sangramento, com base nos resultados alarmantes de tempo de protombina (TP), tempo de tromboplastina parcialmente ativada (TTPa) e plaquetas. MÉTODOS Relato de experiência retrospectivo de ações multidisciplinares desenvolvidas em um hospital universitário. As etapas do estudo envolveram reuniões de equipes, busca de estratégias de comunicação efetiva e criação de um novo indicador de qualidade assistencial. RESULTADOS O indicador foi denominado "Conformidade do Diagnóstico de Enfermagem Risco de Sangramento", monitorado mensalmente desde junho de 2016. A ficha técnica contempla as características e atributos do indicador. Com base nas suas análises são estabelecidos planos de ações para sua qualificação. CONCLUSÃO A implantação do indicador de qualidade assistencial associado ao diagnóstico de enfermagem aprimorou o processo de comunicação, monitoramento e cuidado de enfermagem a pacientes com risco de sangramento.

Resumen OBJETIVO Describir la implementación de un indicador de calidad asistencial asociado al diagnóstico de enfermería de pacientes con alto riesgo de sangrado, con base en los resultados alarmantes de tiempo de protombina (Tp), tiempo de tromboplastina parcialmente activada (TTPa) y plaquetas. MÉTODO Relato de experiencia retrospectiva de acciones multidisciplinares desarrolladas en un hospital universitario. Las etapas del estudio involucraron reuniones de equipos, búsqueda de estrategias de comunicación efectiva y creación de un nuevo indicador de calidad asistencial. RESULTADOS El indicador se denominó "Conformidad del Diagnóstico de Enfermería Riesgo de Sangrado", y se monitoreó mensualmente desde junio de 2016. La ficha técnica contempla las características y atributos del indicador. Con base en los análisis del indicador se establecen planes de acción para su cualificación. CONCLUSIÓN La implementación del indicador de calidad asistencial asociado al diagnóstico de enfermería mejoró el proceso de comunicación, el monitoreo y el cuidado de enfermería a pacientes con riesgo de sangrado.

Abstract OBJECTIVE To describe the implantation of a care quality indicator associated to the nursing diagnosis of patients at high risk of bleeding, based on the alarming results of prothrombin time (PT), partially activated thromboplastin time (aPTT) and platelets. METHODS Retrospective experience report of multidisciplinary actions developed in a university hospital. The stages of the study involved team meetings, search for effective communication strategies and creation of a new indicator of quality of care. RESULTS The indicator was called "Compliance of Nursing Diagnosis Risk for bleeding", monitored monthly since June 2016. The technical file includes the characteristics and attributes of the indicator. Based on the analyzes of the indicator, action plans are established for its qualification. CONCLUSION The implementation of the quality of care indicator associated to the nursing diagnosis improved the communication process, the monitoring and the nursing care to patients at risk of bleeding.

Hemofilia A adquirida. A propósito de un caso / Acquired hemophilia A. A case report

La hemofilia A adquirida es un trastorno hemorrágico poco frecuente caracterizado por la presencia de autoanticuerpos contra el factor VIII (FVIII) circulante. Se ha observado en un grupo heterogéneo de entidades que incluyen, entre otros, enfermedades malignas; de ellas el 32 por ciento asociada a procesos urológicos, donde el cáncer de próstata tiene la mayor prevalencia. Se presenta un paciente que fue atendido en el servicio de Oncología del Hospital Universitario Celestino Hernández Robau con el diagnóstico de hemofilia A adquirida en la evolución de un adenocarcinoma prostático. Se realizó estudio de coagulación en el Instituto de Hematología e Inmunología donde se comprobó la presencia de inhibidor del factor VIII, lo que confirmó el diagnóstico. Se puso tratamiento inmunosupresor con prednisona 1 mg/kg de peso, con una evolución favorable(AU)

Acquired hemophilia A is a rare bleeding disorder characterized by the presence of autoantibodies against circulating factor VIII (FVIII). It has been observed in a heterogeneous group of entities that include, among others, malignant diseases; 32 percent associated with urological processes, where prostate cancer has the highest prevalence. We present a patient who was treated at the Oncology Service of the Celestino Hernández Robau University Hospital with the diagnosis of acquired hemophilia A in the course of a prostatic adenocarcinoma. A coagulation study was carried out at the Institute of Hematology and Immunology where the presence of factor VIII inhibitor was confirmed, confirming the diagnosis. Immunosuppressive treatment was given with prednisone 1 mg/kg of weight, with a favorable evolution(AU)

Perfil clínico y epidemiológico de pacientes con hemofilia registrados en la Liga de Hemofílicos de Antioquia (Colombia) / Clinical and epidemiological profile of patients with hemophilia in Liga de Hemofilicos de Antioquia (Colombia)

Introducción: La hemofilia es una enfermedad hemorrágica hereditaria caracterizada por la ausencia o deficiencia de factores VIII y IX de la coagulación. Objetivo. Determinar el perfil clínico y epidemiológico de los pacientes con hemofilia registrados en la liga de hemofílicos de Antioquia (LIHEA) durante 2005-2011. Metodología. Estudio descriptivo transversal, la población fueron pacientes con hemofilia de la LIHEA que cumplieron con criterios de selección. Se diseñó un instrumento de recolección de información y se aplicó el instrumento Modelo de actitud en pacientes con hemofilia (MAPACHE). Las variables cuantitativas se analizaron con medidas de tendencia central y dispersión, a las variables cualitativas se les estimó frecuencias absolutas, proporciones y razones. Resultados. Se incluyeron 36 hombres con hemofilia, con una mediana de 22 años de edad. En cuanto a los antecedentes personales los más frecuentes fueron HTA (8.3%) y tromboflebitis (5.6%). El sedentarismo fue el factor de riesgo de estilo de vida más común (8.3%). Las manifestaciones clínicas más frecuentes fueron hemartrosis (94.4%), hematomas musculares (80.6%) y equimosis (77.8%). En la mitad de los pacientes la enfermedad generó algún tipo de discapacidad, siendo la más frecuente la física (44.4%). El tratamiento farmacológico más frecuente fue profilaxis con factor VIII con un 88.9%, acompañado de la natación como principal tratamiento no farmacológico con un 77.8%. En cuanto al MAPACHE el 30.6% manifestó una actitud negativa hacia la enfermedad, un 5.6% fue neutra y para el 63.9% restante fue positiva. Conclusión. La hemofilia tuvo grandes repercusiones físicas y emocionales para los pacientes estudiados que la padecen

Introduction: Hemophilia is a hereditary hemorrhagic disorder characterized by the absence or deficiency of factors VIII and IX of the coagulation. Objective. To determine the clinical and epidemiological profile of patients with hemophilia recorded in the Liga de Hemofilicos de Antioquia (LIHEA) during 2005-2011. Methodology. Descriptive transversal study, population were patients with hemophilia from LIHEA which met selection criteria. We designed a data collection instrument and applied the attitude instrument in patients with hemophilia (MAPACHE). The quantitative variables were analyzed using measures of central tendency and dispersion, the qualitative variables were estimated absolute frequencies, proportions and reasons. This research had the respective ethical guarantee. Results. We included 36 men with hemophilia, with a median of 22 years of age. In terms of the personal history, the most frequent were hypertension (8.3%) and thrombophlebitis (5.6%). Sedentariness was the most common lifestyle risk factor (8.3%). The most frequent clinical manifestations were hemarthrosis (94.4%), muscle hematomas (80.6%) and ecchymosis (77.8%). In half of the patients, disease generated some kind of disability, being the most common the physics (44.4%). The most common drug treatment was prophylactic factor VIII with 88.9 %, accompanied by swimming as the main non-pharmacological treatment with 77.8 %. Respect MAPACHE, the 30.6% expressed a negative attitude towards the disease, a 5.6% was neutral and the 63.9% was positive. Conclusion. Hemophilia is still a disease of great physical and emotional implications for studied patients who suffer it

Acquired Hemophilia A Combined with Systemic Lupus Erythematosus: A Case Report and Literature Review

Acquired hemophilia A (AHA) is a rare hemorrhagic disorder caused by autoantibodies against factor VIII (FVIII). An 80-year-old woman presented multiple bruises on her upper and lower extremities, along with gross hematuria. Extensive ecchymosis and swelling were observed on the buttocks. She had anemia and normal platelet count. The initial coagulation results showed prolonged activated partial thromboplastin time (aPTT, 68.5 seconds) and normal prothrombin time. According to the mixing test, we observed a decreased FVIII activity (2%), increased factor VIII inhibitor (FVIII-I) titer (74.4 BU), and negative lupus anticoagulant. AHA was diagnosed based on late onset bleeding and increased FVIII-I titer. Additionally, she met the criteria for systemic lupus erythematosus (oral ulcer, photosensitivity, renal disorder, and positivity for antinuclear and anti-β2-glycoprotein-I antibodies). She was started on oral prednisolone for FVIII-I eradication. Post-treatment, her bleeding tendency, aPTT (47.3 seconds), and FVIII-I titer decreased (1.24 BU), and FVIII activity increased (10%).

Differential diagnosis of childhood hemorrhagic disorders

Bruising and bleeding are common events in children. The pediatrician must be able to determine whether a child's symptoms are normal or perhaps indicative of hemorrhagic disorders. A thorough medical history and physical examination should enable the pediatricians to identify those patients warranting further evaluation. This review describes the characteristics of the medical history, physical examination, and clinical laboratory testing that are important in recognizing clinically significant bleeding disorders. This review will help the pediatrician to perform the initial laboratory evaluation, differentiate for patients with bleeding tendency and make the correct diagnosis for a variety of cases.

A Case of Successful Endoscopic Submucosal Dissection of Gastric Adenoma in a Patient with Congenital Factor VII Deficiency

Congenital factor VII deficiency is a rare hemorrhagic disorder, and invasive procedures are likely to cause excessive bleeding in these patients. Endoscopic submucosal dissection (ESD) has been accepted as a curative treatment modality for gastric adenoma, early gastric cancer (EGC) and any other mucosal and submucosal tumors. The most important complications of ESD are bleeding and perforation. The use of antiplatelet agents or coagulopathies are risk factors for these complications. There are only few reports of successful ESD with coagulation disorders. We report a case of a 70-year-old female patient who was diagnosed with a gastric adenoma and factor VII deficiency. The patient was successfully treated with ESD. Before ESD, recombinant Coagulation factor VIIa was injected, and the procedure was performed successfully without any complications. In conclusion, ESD can be performed successfully in patients with factor VII deficiency, when recombinant human factor VIIa is administered properly.

Analysis of factors affecting hemorrhagic diathesis and overall survival in patients with acute promyelocytic leukemia

BACKGROUND/AIMS: This study investigated whether patients with acute promyelocytic leukemia (APL) truly fulfill the diagnostic criteria of overt disseminated intravascular coagulation (DIC), as proposed by the International Society on Thrombosis and Haemostasis (ISTH) and the Korean Society on Thrombosis and Hemostasis (KSTH), and analyzed which component of the criteria most contributes to bleeding diathesis. METHODS: A single-center retrospective analysis was conducted on newly diagnosed APL patients between January 1995 and May 2012. RESULTS: A total of 46 newly diagnosed APL patients were analyzed. Of these, 27 patients (58.7%) showed initial bleeding. The median number of points per patient fulfilling the diagnostic criteria of overt DIC by the ISTH and the KSTH was 5 (range, 1 to 7) and 3 (range, 1 to 4), respectively. At diagnosis of APL, 22 patients (47.8%) fulfilled the overt DIC diagnostic criteria by either the ISTH or KSTH. In multivariate analysis of the ISTH or KSTH diagnostic criteria for overt DIC, the initial fibrinogen level was the only statistically significant factor associated with initial bleeding (p = 0.035), but it was not associated with overall survival (OS). CONCLUSIONS: Initial fibrinogen level is associated with initial presentation of bleeding of APL patients, but does not affect OS.

Clinical and laboratory characteristics of patients with dengue hemorrhagic fever manifestations and their transfusion profile

Background: Dengue is an infectious disease with a recurring incidence, especially in developing countries. Despite recent economic growth, success in disease control has not been achieved, and dengue has evolved from cyclic epidemic outbreaks to a lack of seasonality. The lack of scientific basis for the proper management of cases with hemorrhagic manifestations, especially regarding transfusion procedures, might contribute to the high death rate in potentially avoidable cases. Objective: The aim of the study was to identify the clinical and laboratory manifestations in hemorrhagic dengue fever treated at the emergency services in Rio Branco, AC, Brazil, as well as to describe transfusion characteristics of patients and identify possible prognostic factors. Methods: A retrospective descriptive study was performed to analyze the distribution of relative frequencies of clinical and laboratory variables. The study was carried out in Rio Branco with confirmed dengue fever cases. Secondary data were obtained by Acre Epidemiological Surveillance teams of cases with bleeding or platelet counts under 100.0 × 109/L. The patients' clinical, laboratory and transfusion data were obtained from hospital records. Results: A total of 90,553 dengue cases were reported of which 7,447 had serologic confirmation; 267 cases had hemorrhagic manifestations and 193 patients were located. Nearly half of the patients had anemia and the mean of the lowest platelet count of these patients was 26.4 × 109/L. Platelet concentrate was transfused in 22.3% of cases with a mean of 7.5 IU/patient, fresh frozen plasma in 21.2% with a mean of 5.2 IU/patient and just 2.6% of patients received concentrated red blood cells with a mean of 3.2 IU/patient. Bleeding led to transfusions. Signs of plasma leakage and cardiopulmonary dysfunction were correlated to unfavorable outcomes...

Hemorrhagic Diathesis as the Presenting Symptom of Neonatal Cholestasis / 대한소아소화기영양학회지

A 4-week-old infant presented with a coagulation disorder resulting from a vitamin K deficiency. The vitamin K deficiency was caused by neonatal cholestasis due to biliary atresia. Jaundice, hepatomegaly and pale stools are the predominant presenting symptoms of biliary atresia, none of which were recognized in our patient before admission. However, the patient presented with bleeding caused by vitamin K deficiency. She was fully breastfed and had received adequate doses of vitamin K at birth and from the age of 1 week. In case of a hemorrhagic diathesis due to neonatal cholestasis, timely identification of treatable underlying disorders, in particular biliary atresia, is important because an early surgical intervention results in a better prognosis. Meticulous history taking and a thorough physical exam can be decisive for an early diagnosis and subsequent intervention.

Haematological manifestations of arthrogryposis-renal dysfunction-cholestasis [ARC] syndrome: a case report

Arthrogryposis multiplex congenita, renal dysfunction, and cholestasis [ARC] syndrome is a rare, fatal, multisystem disorder. Bleeding problems, which occur spontaneously and post organ biopsies, have been reported in these patients. We report the case of an infant who had life-threatening spontaneous nasal bleeding. A detailed assessment of her platelet function and morphology is presented

Intervalos de referencia para agregación plaquetaria en individuos sanos / Platelet aggregation reference interval for healthy individuals / Intervalos de referência para agregação plaquetária

La agregación por transmisión de luz (ATL) es el método más usado por laboratorios clínicos y de investigación para evaluar la función plaquetaria y es considerado actualmente el estándar de oro; no obstante, la ATL aún no es un método estandarizado, pese a los esfuerzos de organismos internacionales como la International Society of Thrombosis and Hemostasis (ISTH). Organismos regulatorios recomiendan que cada laboratorio clínico determine e informe sus intervalos de referencia (IR) para cada agonista que utiliza. Se presenta los IR de este laboratorio en la determinación de agregación plaquetaria mediante ALT utilizando adenosin difosfato (ADP), colágeno y adrenalina como agonistas. Para ello se diseñó un estudio de corte transversal sobre una muestra a conveniencia de voluntarios(as) aparentemente sanos; se usó un agregómetro óptico y se emplearon los siguientes agonistas y concentraciones finales: ADP 5 µM, colágeno 2 µM y adrenalina 10 µM. Se definió IR como los percentiles 2,5 y 97,5 (P2,5 y P97,5) del Porcentaje de Agregación Plaquetaria Máxima APM%. Participaron 63 individuos, rango de edad 18 a 66 años, 79,4% sexo femenino. Los valores de APM% fueron: ADP P2,5=49% y P97,5=87%; colágeno P2,5=43% y P97,5=86%; adrenalina P2,5=42% y P97,5=85%. Atendiendo a recomendaciones internacionalmente aceptadas, se presentan los IR de APM (%) por el método ATL en este laboratorio (ASCARDIO, Barquisimeto, Venezuela), lo que permite al clínico basar sus decisiones en evidencia válida y pertinente.

Platelet aggregation tests by means of light transmission (LTA), the current gold standard, are the most commonly used methods used to evaluate platelet function at clinical and research laboratories. However, LTA has not been determinastandardized despite the work from international organizations such as the International Society of Thrombosis and Homeostasis (ISTH). Regulatory agencies recommend that each clinical laboratory establishes and informs its own Reference Internal (RI) for all agonists they use. RI are presented for our laboratory using the following agonists: diphosphate (ADP), collagen y adrenaline and the pertaining methodology. To assess our RI for platelet aggregation tests by LTA, a cross-sectional study was designed with a convenience sample of healthy volunteer men and women using an optical aggregometer with the following agonist and final concentration: adenosine diphosphate (ADP) 5 µM, collagen 2 µM and adrenaline 10 µM. The RIs were defined as Percentiles 2.5 (P2,5) and 97.5 (P97.5) of the percentage of maximal aggregation (%MA). 63 subjects participate, age range 18 to 66,79.4% were female. The IR for %MA were: ADP P2,5=49% and P97.5=87%; collagen P2,5=43% and P97.5=86%; adrenaline P2,5=42% and P97.5=85%. In agreement with international accepted recommendation guidelines, RIs for the %MA values were presented by LTA done in our clinical laboratory (ASCARDIO, Barquisimeto, Lara State, Venezuela), that allows physicians to base their clinical decision process on valid and pertinent information.

A agregometria por transmissão de luz (ATL) é o método mais utilizado pelos laboratórios clínicos e de pesquisa para avaliar a função plaquetária e é atualmente considerada o padrão-ouro; no entanto, a ATL ainda não é um método padronizado, apesar dos esforços das agências internacionais como a International Society of Thrombosis and Hemostasis (ISTH). Agências reguladoras recomendam que cada laboratório clínico determine e comunique seus intervalos de referência (IR) para cada agonista utilizado. São apresentados o IR de nosso laboratório para determinar a agregação plaquetária através de ALT usando Difosfato de Adenosina (ADP), Colágeno e Adrenalina como agonistas. Para isso foi elaborado um estudo de corte transversal em uma amostra de conveniência de voluntários(as) aparentemente saudáveis , foi usado agregômetro ótico e foram utilizados os seguintes agonistas e concentrações finais: 5µ M ADP, Colágeno 2µM e Adrenalina 10µM. Definiu-se o IR com os percentis 2,5 e 97.5 (P2,5 e P97.5) do Percentual de Agregação Plaquetária Máxima APM%. Participaram 63 indivíduos, na faixa etária 18-66 anos, 79,4% do sexo feminino. Os valores de APM% foram: ADPP2,5=49% e P97.5=87%; Colágeno P2,5=43% e P99,5=86%, Adrenalina P2,5=42% e P97.5=85%. Atendendo às recomendações aceitas internacionalmente, apresentam-se os IR de APM% pelo método ATL em nosso laboratório (ASCARDIO, Barquisimeto, Venezuela), o que permite ao médico basear as suas decisões em evidências válidas e pertinentes.

Pruebas del coagulograma y componentes de la hemostasia. Utilidad para diagnosticar las diátesis hemorrágicas / Tests of coagulation and hemostasis components. Usefulness for diagnosing hemorrhagic diathesis

El coagulograma comprende un conjunto de pruebas que exploran la participación de todos los componentes de la hemostasia: endotelio vascular, actividad plaquetaria, factores plasmáticos y fibrinolíticos. Con frecuencia, la ejecución de estas pruebas resulta compleja para el personal técnico, por lo que la profundización en el conocimiento e interpretación de los resultados de cada una de estas, debe redundar en el fortalecimiento y preparación de los profesionales de la salud. En el presente trabajo se describen las principales pruebas del coagulograma convencional, el principio y los valores de referencia de cada una, así como las posibles enfermedades de acuerdo con la alteración del sistema hemostático que corresponde a la alteración del coagulograma, con el objetivo de brindarle al médico una información básica para la correcta ejecución y adecuada interpretación de los resultados

Coagulogram comprises a set of tests, which explore the participation of all components of hemostasia: vascular endothelium, platelet activity, plasma and fibrinolytic factors. Often, the technical staff finds complex to do these tests, so deepening knowledge, understanding, and interpreting the results of each of these tests should result in strengthening and training of health professionals. This paper describes the main conventional coagulation tests, the beginning and the reference values of each of them, and the possible diseases according to the alteration of the hemostatic system corresponding to the alteration of coagulation, with the aim of providing medical background information for the proper performance and proper interpretation of results

Flexible ureteroscopy and Holmium:YAG laser lithotripsy for stone disease in patients with bleeding diathesis: a systematic review of the literature

INTRODUCTION AND OBJECTIVES: The management of urolithiasis in patients on anticoagulants presents a challenge to the endourologist. Due to multiple comorbidities, it may be impossible to safely discontinue the anticoagulant treatment. Other modalities such as shock wave lithotripsy and PCNL are contraindicated in these patients, so ureteroscopic treatment may be the only option. We conducted a systematic review of the literature to look at the safety and efficacy of ureteroscopic management in these patients. METHODS: Systematic review and quantitative meta-analysis was performed using studies identified by a systematic electronic literature search from January 1990 to August 2011. All articles reporting on treatment for stones in patients with a bleeding diathesis using ureteroscopy and a Holmium:YAG laser were included. Two reviewers independently extracted the data from each study. The data was included into a meta-analysis and discussed. RESULTS: Three studies were identified reporting on 70 patients (73 procedures). All patients had stone fragmentation using Holmium laser. The mean stone size was 13.2mm with a range of 5-35mm. The quality of the included studies was modest. Stone free status was achieved in sixty-four patients (87.7%). There were no major complications and only 11% of the patients developed minor complications with only 4% rate of minor bleeding. CONCLUSIONS: Retrograde stone treatment using ureteroscopy and holmium laser lithotripsy can be safely performed in patients with bleeding diathesis with a low complication rate.

Deficiencia adquirida del factor VIII. Un ejemplo cuando la cirugía puede ser perjudicial y no para salvar vidas / Deficiência adquirida do fator VIII. Um exemplo de quando a cirurgia pode ser prejudicial e nâo vinculada a salvar vidas / Acquired factor VIII deficiency. One instance when surgery may be harmful and not life-saving

La hemofilia adquirida A es una condición extremadamente rara que ocurre en una persona en un millón por año. Puede causar riesgo para la vida por causar episodios de sangrado durante la edad adulta, debido a la producción de auto-anticuerpos que inactivan el factor VIII. Su tratamiento suele incluir la inmunosupresión y la cirugía se debe evitar en lo posible. Presentamos dos casos que nos tocó tratar, y realizamos una recopilación somera de la bibliografia, a fin de enfatizar la necesidad de no realizar tratamiento quirúrgico en estos casos.

A hemofilia adquirida A é uma condição extremamente rara que pode ocorrer com a probabilidade de um caso em um milhão de pessoas por ano. Pode causar risco para a vida por causar episódios de sangramento durante a idade adulta, devido à produção de autoanticorpos que inativam o fator VIII. Seu tratamento geralmente inclui a imunosupressão, e a cirurgia deve, dentro do possível, ser evitada. Apresentamos dois casos por nós tratados, e realizamos um breve resumo da bibliografia, com a finalidade de enfatizar o propósito de não realizar tratamento cirúrgico nestes casos.

Acquired hemophilia A is an extremely rare disease with an incidence of 1 in 1,000,000 per year. It may be life threatening as it produces bleeding episodes in adult life, due to the production of antibodies which inactivate factor VIII. Treatment may include immunosuppression and surgery must be avoided as much as possible. We shall present two of our cases and then make a brief review of the literature, in order to underscore the need of not operating these cases.

Bronchiectasis combined with primary hyperfibrinolysis: report of two cases / 南方医科大学学报

T Hemoptysis is a common complication of bronchiectasis, but concurrent primary hyperfibrinolysis is very rare and easily gives rise to missed diagnosis. Patients with bronchiectasis complicated by primary hyperfibrinolysis do not respond to regular treatment for hemoptysis, which may potential cause fatal consequences. We report two cases of bronchiectasis combined with primary hyperfibrinolysis. Both of the patients had a history of bronchiectasis and were admitted for intermittent hemoptysis. Conventional treatment such as vasopressin failed to produce a favorable response. The eventual definite diagnosis of primary hyperfibrinolysis was established based on laboratory examinations. After diagnosis, the patients received fibrinogen and cryoglobulin to maintain a fibrinogen level over 1.5 g/L. Both of the patients subsequently showed improved conditions and were discharged.

Severe thrombocytopenia and hemorrhagic diathesis due to brucellosis

Background: We aimed to examine cases of brucellosis that presented with severe thrombocytopenia and hemorrhagic diathesis

Methods: A total of 10 brucellosis cases with severe thrombocytopenia were included in this case-series study. Patients' files were reviewed for their clinical and laboratory findings, as well as clinical outcomes and complications. Platelet counts of<20000/mm[3] were diagnosed as severe thrombocytopenia

Results: The lowest thrombocyte count was 3000/mm[3] while the highest was 19000/mm[3] [mean: 12000/mm[3]]. Patients had the following symptoms: epistaxis [7 cases], petechia with epistaxis [4 cases], bleeding gums [3 cases], ecchymosis with epistaxis [2 cases], melena and renal failure [2 cases], and hematuria [1 case]. Patients were given rifampicin and doxycycline along with supportive hematological therapy. All were treated successfully with no evidence of recurrence at follow-up visits

Conclusion: Since brucellosis is endemic in developing countries, it must be considered in the differential diagnosis of cases that present with severe thrombocytopenia and hemorrhagic diathesis