Síndrome de Evans y embarazo: reporte de un caso / Evans syndrome and pregnancy: a case report

Resumen El síndrome de Evans es una enfermedad conformada por la presencia simultánea o secuencial de trombocitopenia inmunitaria y anemia hemolítica autoinmunitaria, que puede ser primaria o secundaria a otra patología. Es una afección poco frecuente, por lo que es necesario tener una alta sospecha, y descartar otras patologías que cursan con dichas alteraciones hematológicas, para hacer el diagnóstico. Su manejo representa un desafío terapéutico dado su curso crónico y recidivante. La presentación durante el embarazo se asocia a morbilidad materna y fetal. A continuación presentamos el caso de una gestante en quien se pesquisó trombocitopenia severa aislada al ingreso al control prenatal, y que en el curso del embarazo desarrolló AHAI conformando un síndrome de Evans, que se consideró secundario a LES incompleto al realizar el estudio reumatológico. Debido a la pobre respuesta al tratamiento médico con corticoides e inmunosupresores, la mayor parte del embarazo se mantuvo hospitalizada para observación, ajuste y cambio de terapia, siendo necesario recurrir a manejo quirúrgico con esplenectomía.

Abstract Evans syndrome is a rare entity formed by the simultaneous or sequential presence of immune thrombocytopenia and autoimmune hemolytic anemia, which can be primary or secondary to another pathology. The presentation of this disease during pregnancy is associated with maternal and fetal morbidity. The syndrome's diagnosis requires a high suspicion and the ruling out of other pathologies that can happen with the same hematological alterations. The management represents a therapeutic challenge because of its chronic and recurrent course. Below we present the case of a pregnant woman in whom isolated severe thrombocytopenia was detected at admission for prenatal control, and who developed AIHA during the pregnancy, forming Evans syndrome, which was considered secondary to incomplete SLE when performing the rheumatological study. Due to the poor response to medical treatment with corticosteroids and immunosuppressants, the patient was hospitalized for most of her pregnancy for observation, adjustment and change of therapy, and even it was necessary resort to surgical management with splenectomy.

Effect of artificial liver with double plasma molecular absorb system model on patients' platelets and corresponding treatment strategy / 北京大学学报(医学版)

OBJECTIVE@#To compare the effects of artificial liver treatment with double plasma molecular adsorption system(DPMAS) mode and traditional plasma exchange (PE) mode on platelets in patients, and to evaluate the clinical efficacy of recombinent human thrombopoietin (rhTPO) in the treatment of thrombocytopenia.@*METHODS@#A total of fifteen patients undergoing artificial liver with DPMAS model admitted to the Fifth Affiliated Hospital of Guangzhou Medical University from January 2018 to November 2020 were selected and included in the DPMAS group, and another 15 patients receiving PE were selected and included in the PE group. The improvement of clinical symptoms, such as fatigue, jaundice, oliguria, edema, etc. before and after artificial liver treatment was compared between the two groups, and the trend of blood routine (especially platelet), coagulation function and other indexes before and after treatment were compared between the two groups. The use of rhTPO and the number of platelets were recorded during treatment.@*RESULTS@#The improvement rate of clinical symptoms in DPMAS group was 86.67%, which was higher than that in PE group, but the difference was not statistically significant (P>0.05). There was no statistical significance in the outcome of the two groups within 90 days (P>0.05). There was no significant difference in white blood cell (WBC) and hemoglobin (HB) between the two groups after treatment (P>0.05). However, the level of platelet(PLT) in DPMAS group was significantly lower than that before treatment (P < 0.05), and was significantly lower than that in PE group (P < 0.05). After treatment, the international normalized ratio (INR) level in PE group was significantly improved (P < 0.05), but there was no significant difference in the INR level in DPMAS group (P>0.05). The patients in the DPMAS group received an average of (8.2±3.1) doses of rhTPO and (1.5±0.3) IU of platelet transfusions during hospitalization. In DMPAS group, platelets increased significantly after infusion of terbium.@*CONCLUSION@#Compared with PE mode, the artificial liver with DPMAS mode can reduce platelet levels in patients, but the application of rhTPO can stimulate platelet regeneration and increase platelet levels in the patients, thereby reducing the risk of bleeding due to platelet hypoplasia.

Trombocitopenia trombótica inducida por vacuna. Primer reporte nacional / Vaccine-associated immune thrombosis and thrombocytopenia. First national report / Trombocitopenia trombótica induzida por vacina (VITT). Primeiro relatório nacional

Resumen: Introducción: la vacunación contra SARS-CoV-2 es una herramienta imprescindible en el combate contra la pandemia de COVID-19. La vacuna desarrollada en colaboración entre la Universidad de Oxford y el laboratorio de productos farmacéuticos AstraZeneca (AZN) ha demostrado buena eficacia, pero ha habido reporte de trombosis venosas. Caso clínico: se presenta el caso de un paciente de 70 años, de sexo masculino, que 7 días después de la administración de la primera dosis de la vacuna AZN desarrolla trombosis venosa profunda de ambos miembros inferiores y tromboembolismo pulmonar. Coincide con trombocitopenia de 15.000/mm3, descenso del fibrinógeno y elevación de los D-dímeros. La situación clínica evoca el planteo de trombocitopenia trombótica inducida por vacuna (VITT). Se realizó tratamiento con inmunoglobulinas intravenosas, metilprednisolona y crioprecipitados. Requirió colocación de un filtro de la vena cava inferior. Una vez mejorado el recuento plaquetario se instaló tratamiento anticoagulante con apixaban. Evolucionó favorablemente. Discusión: se trata del primer reporte nacional de VITT. Las trombosis subsiguientes a la vacuna de AZN pueden verse con las vacunas que comparten la misma plataforma vacunal (adenovirus inactivado). Se han reportado casos fundamentalmente en menores de 60 años y en topografías inhabituales. Este caso tiene la particularidad de que se trata de un paciente mayor de 60 años, que ya había tenido COVID-19 cinco meses antes y que se presenta con una trombosis en sitios habituales. El manejo terapéutico se adecuó a las pautas internacionales. El caso deja un aprendizaje relevante tanto en lo que refiere al diagnóstico precoz como al manejo terapéutico.

Abstract: Introduction: vaccines against SARS-CoV-2 are an essential tool against the COVID-19 pandemic. The vaccine developed in collaboration with the University of Oxford and the AstraZeneca (AZN) laboratory has proved to be effective, although venous thrombosis have been reported. Clinical case: the study presents the case of a 70 year old male patient who, 7 days after receiving the first dose of the AZN vaccination develops deep vein thrombosis (DVT) in the lower extremities and pulmonary embolism. Simultaneously, thrombocytopenia is 15.000/mm3, fibrinogen levels drop D-dimer levels are elevated. The clinical situation leads to the suspicion of vaccine-associated immune thrombosis and thrombocytopenia (VITT). The patient was treated with intravenous immune globulin, methylprednisolone and cryoprecipitates, requiring a filter to be placed in the inferior vena cava. Once platelets count improved, anti-coagulation therapy including apixaban was commenced, evolution being good. Discussion: this is the first national report on VITT. Thrombosis after the AZN vaccination may be seen in other vaccines that use the same vaccine platform (inactive adenovirus). Cases have been reported mainly in patients younger than 60 years old and in unusual topographies. In particular, this case presents a male patient that is older than 60 years old, who had already been infected with COVID-19 five months before and who currently consults with thrombosis in regular sites. Therapeutic handling observed international guidelines. The case contributes relevant data both in terms of early diagnosis and therapeutic handling.

Resumo: Introdução: a vacinação contra a SARS-CoV-2 é uma ferramenta essencial na luta contra a pandemia de COVID-19. A vacina desenvolvida pela colaboração entre a Universidade de Oxford e o laboratório farmacêutico AstraZeneca (AZN) tem demonstrado boa eficácia, mas foram relatados casos de trombose venosa. Caso clínico: apresenta-se o caso de um paciente do sexo masculino, 70 anos, que 7 dias após a administração da primeira dose da vacina AZN desenvolveu trombose venosa profunda de ambos os membros inferiores e tromboembolismo pulmonar. Coincide com trombocitopenia de 15.000 / mm3, diminuição do fibrinogênio e aumento dos D-dímeros. A situação clínica lembra a trombocitopenia trombótica induzida por vacina (VITT). O tratamento foi realizado com imunoglobulinas intravenosas, metilprednisolona e crioprecipitados. Foi necessário colocar um filtro de veia cava inferior. Uma vez que a contagem de plaquetas melhorou, o tratamento anticoagulante com apixaban foi instalado. O paciente favoravelmente. Discussão: este é o primeiro relatório nacional de VITT. As tromboses subseqüentes à vacina AZN podem ser vistas com vacinas que compartilham a mesma plataforma (adenovírus inativado). Os casos foram relatados principalmente em pessoas com menos de 60 anos de idade e em topografias incomuns. Este caso tem a particularidade de se tratar de um paciente com mais de 60 anos, já com COVID-19 há cinco meses e que apresenta trombose em sítios comuns. O manejo terapêutico foi adaptado às diretrizes internacionais. O caso deixa um aprendizado relevante tanto no que diz respeito ao diagnóstico precoce quanto ao manejo terapêutico.

Diagnosis and management of coronavirus disease-associated immune thrombocytopenia: a case series

Abstract Data on health problems and fatal complications associated with coronavirus disease (COVID-19) have consistently been reported. Although immune thrombocytopenia has been associated with multiple viral infections, only few studies have shown its association with COVID-19. Here, we have reported a case series of two cases pertaining to patients diagnosed with COVID-19-associated immune thrombocytopenia, elaborating on the clinical course, management, and response to treatment.

Update on thrombocytopenia in pregnancy / Atualização sobre trombocitopenia na gravidez

Abstract Thrombocytopenia, defined as platelet count < 150,000mm3, is frequently diagnosed by obstetricians since this parameter is included in routine surveillance during pregnancy, with an incidence of between 7 and 12%. Therefore, decisions regarding subsequent examination and management are primordial. While most of the cases are due to physiological changes, as gestational thrombocytopenia, other causes can be related to severe conditions that can lead to fetal or maternal death. Differentiating these conditions might be challenging: they can be pregnancy-specific (pre-eclampsia/ HELLP syndrome [hemolysis, elevated liver enzymes, low platelets]), or not (immune thrombocytopenia purpura, thrombotic thrombocytopenic purpura or hemolytic uremic syndrome). Understanding the mechanisms and recognition of symptoms and signs is essential to decide an adequate line of investigation. The severity of thrombocytopenia, its etiology and gestational age dictates different treatment regimens.

Resumo Trombocitopenia, definida como uma contagem de plaquetária < 150.000mm3, é frequentemente diagnosticada pelos obstetras, uma vez que este parâmetro está incluído na vigilância de rotina durante a gravidez, com uma incidência de entre 7 e 12%. Assim, decisões relativas à avaliação e orientação subsequentes são primordiais. Embora a maioria dos casos ocorra devido a alterações fisiológicas, como a trombocitopenia gestacional, outras causas podem estar relacionadas com condições graves que podem levar à morte fetal ou materna. Distinguir entre estas entidades pode ser desafiante: elas podem ser específicas da gravidez (pré-eclâmpsia/síndrome HELLP [hemolysis, elevated liver enzymes, low platelets]) ou não (púrpura trombocitopênica imune, púrpura trombocitopênica trombótica ou síndrome hemolítico urêmico). Compreender os mecanismos e reconhecer os sinais e sintomas é essencial para decidir uma adequada linha de investigação. A severidade da trombocitopenia, a sua etiologia e a idade gestacional ditam regimes de tratamento diferentes.

Rare inherited kidney diseases: an evolving field in Nephrology / Doenças renais hereditárias raras: um campo em evolução na Nefrologia

Abstract There are more than 150 different rare genetic kidney diseases. They can be classified according to diagnostic findings as (i) disorders of growth and structure, (ii) glomerular diseases, (iii) tubular, and (iv) metabolic diseases. In recent years, there has been a shift of paradigm in this field. Molecular testing has become more accessible, our understanding of the underlying pathophysiologic mechanisms of these diseases has evolved, and new therapeutic strategies have become more available. Therefore, the role of nephrologists has progressively shifted from a mere spectator to an active player, part of a multidisciplinary team in the diagnosis and treatment of these disorders. This article provides an overview of the recent advances in rare hereditary kidney disorders by discussing the genetic aspects, clinical manifestations, diagnostic, and therapeutic approaches of some of these disorders, named familial focal and segmental glomerulosclerosis, tuberous sclerosis complex, Fabry nephropathy, and MYH-9 related disorder.

Resumo As doenças renais genéticas raras compreendem mais de 150 desordens. Elas podem ser classificadas segundo achados diagnósticos como (i) distúrbios do crescimento e estrutura, (ii) doenças glomerulares, (iii) tubulares e (iv) metabólicas. Nos últimos anos, houve uma mudança de paradigma nesse campo. Os testes moleculares tornaram-se mais acessíveis, nossa compreensão sobre os mecanismos fisiopatológicos subjacentes a essas doenças evoluiu e novas estratégias terapêuticas foram propostas. Portanto, o papel do nefrologista mudou progressivamente de mero espectador a participante ativo, parte de uma equipe multidisciplinar, no diagnóstico e tratamento desses distúrbios. O presente artigo oferece um panorama geral dos recentes avanços a respeito dos distúrbios renais hereditários raros, discutindo aspectos genéticos, manifestações clínicas e abordagens diagnósticas e terapêuticas de alguns desses distúrbios, mais especificamente a glomeruloesclerose segmentar e focal familiar, complexo da esclerose tuberosa, nefropatia de Fabry e doença relacionada ao MYH9.

Trombocitopenia inmune primaria e infección por citomegalovirus y Epstein Barr virus: autoinmunidad versus inmunosupresión / Immune thrombocytopenia primary and cytomegalovius, Epstein Barr virus infection: immunosuppression versus autoimmunity

La trombocitopenia inmune primaria está asociada con múltiples factores que pueden conducir a la pérdida de autotolerancia a los antígenos en la superficie de las plaquetas y los megacariocitos. Varios agentes infecciosos han sido implicados. Los virus herpes debido a la alta prevalencia en la población y el tropismo por las células linfoides se han relacionado con el desarrollo de esta entidad. Se realizó una revisión bibliográfica utilizando motores de búsqueda como Ebsco y Pubmed que permitió el acceso a artículos relacionados en revistas arbitradas. Se recolectó y organizó información sobre la implicación de la infección por citomegalovirus y EBV en el desarrollo de trombocitopenia inmune primaria. La mayoría de los artículos indican que en estos virus, la naturaleza no citolítica de la replicación favorece el tiempo de interacción y modulación en las células huésped que son frecuentemente linfocitos B, T y asesinas naturales. El diagnóstico oportuno y la terapia adecuada en estos pacientes contribuyen a la inmunomodulación de la autorreactividad y eliminación viral que, a la luz de los conocimientos actuales, es esencial para el tratamiento clínico integrado. Es necesario considerar el monitoreo del estado serológico y molecular de estos herpes virus, en pacientes en los que la historia natural de la enfermedad sugiere su asociación, especialmente en trombocitopenia inmunitaria primaria o secundaria; por el alto nivel de relación de los mecanismos de producción de la autoinmunidad, la dismielopoyesis y la linfoproliferación, con la patogénesis de la infección por estos virus(AU)

Primary immune thrombocytopenia is associated with multiple factors that may lead to loss of self-tolerance to the antigens on the surface of platelets and megakaryocytes. Several infectious agents have been implicated. Herpes viruses due to the high prevalence in the population and tropism by the lymphoid cells have been related to the development of this entity. A bibliographic review was made using search engines such as Ebsco and Pubmed that allowed access to related articles in magazines arbitrated. Information was collected and organized that involved the role of cytomegalovirus and EBV infection in the development of ITP. Most of the articles indicate that in these viruses, the non-cytolytic nature of replication favors the time of interaction and modulation on host cells that are frequently B, T and natural killer lymphocytes. Timely diagnosis and appropriate therapy in these patients contributes to the immunomodulation of self-reactivity and viral elimination, in the light of current knowledge, is essential for integrated clinical treatment. Consider monitoring the serological and molecular status of these herpes viruses in patients in whom the natural history of the disease suggests their association, especially in primary or secondary immune thrombocytopenia; by the high level of relation of the mechanisms of production of the autoimmunity, the dysmielopoyesis and the lymph proliferation with the pathogenesis of the infection by these viruses(AU)

Evaluation of platelet concentrate prescription in pediatric patients at a tertiary care hospital / Avaliação da prescrição de concentrado de plaquetas em pacientes pediátricos em hospital terciário

ABSTRACT Objective: To verify the adequacy of platelet concentrate prescription by pediatricians in different pediatric sectors of a general hospital. Methods: A cross-sectional study evaluating 218/227 platelet concentrate records in children and adolescents (zero to 13 years old), from January 2007 to April 2015, by the pediatricians of the emergency room, sick bay and intensive care unit. The requisitions were excluded in patients with hematological diseases and those without the number of platelets. Results: Children under 12 months received 98 platelet concentrates (45.2%). Most of the transfusions were prophylactic (165; 79%). Regarding the transfusion site, 39 (18%) were in the emergency room, 27 (12.4%) in the sick bay and 151 (69.6%) in the intensive care unit. The trigger, prescribed volume and platelet concentrate subtype were adequate in 59 (28.2%), 116 (53.5%) and 209 (96.3%) of the transfusions, respectively. Patients with hemorrhage presented adequacy in 42 (95.5%), while children without bleeding presented in 17 (10.3%). The most common inadequacy related to volume was the prescription above recommendation (95; 43.8%). Eight platelet concentrates were prescribed with subtype requests without indication. Conclusion: The results obtained in this study showed that transfusion of platelet concentrate occurred more adequately in children with active bleeding compared to prophylactic transfusion. There was a tendency to prescribe high volumes and platelet subtypes not justified according to current protocols. The teaching of transfusion medicine should be more valued at undergraduate and medical residency.

RESUMO Objetivo: Verificar a adequação na prescrição de concentrado de plaquetas por pediatras em diferentes setores da pediatria de um hospital geral. Métodos: Estudo transversal avaliando 218/227 fichas de requisição de concentrado de plaquetas de crianças e adolescentes (zero a 13 anos), de janeiro de 2007 a abril de 2015 pelos pediatras do pronto-socorro, enfermaria e unidade de terapia intensiva. Excluíram-se as requisições em portadores de doenças hematológicas e aquelas sem o número de plaquetas. Resultados: Crianças com menos de 12 meses receberam 98 prescrições de concentrado de plaquetas (45,2%). A maioria das transfusões foi profiláticas (165; 79%). Em relação ao local da transfusão, 39 (18%) foram no pronto-socorro, 27 (12,4%) na enfermaria e 151 (69,6%) na unidade de terapia intensiva. O gatilho, o volume prescrito e o subtipo de concentrado de plaquetas foram adequados em 59 (28,2%), 116 (53,5%) e 209 (96,3%) das transfusões, respectivamente. Prescrições para pacientes com hemorragia apresentaram adequação em 42 (95,5%) transfusões, enquanto para crianças sem hemorragia houve adequação em 17 (10,3%) vezes. A inadequação mais comum em relação ao volume foi a prescrição acima da recomendação (95; 43,8%). Foram prescritos oito concentrados de plaquetas sem indicação de solicitação de subtipos. Conclusão: Os resultados obtidos nesse estudo mostraram que a prescrição de transfusão de concentrado de plaquetas foi mais adequada em crianças com hemorragia ativa em comparação com a transfusão profilática. Houve tendência à prescrição de volumes elevados e de subtipos de plaquetas, o que não se justifica segundo os protocolos atuais. O ensino da medicina transfusional deve ser mais valorizado na graduação e na residência médica.

Refractory severe thrombocytopenia during pregnancy: how to manage / Trombocitopenia grave refratária na gravidez: como abordar

Abstract Thrombocytopenia is the most common hemostatic change in pregnancy, but severe thrombocytopenia is rare. One of the causes, immune thrombocytopenic purpura (ITP), is characterized by increased platelet destruction by immunoglobulin G (IgG) antibodies, presenting a high risk of hemorrhage for the patient, but also for the fetus, since antibodiesmay cross the placenta.We present the case of a 23-year-old pregnant woman with a history of Langerhans cell histiocytosis of the mandible submitted to surgery and chemotherapy when she was 10 years old, with diagnosis of ITP since then. At 28 weeks of gestation, she presented with petechiae, epistaxis, and gingival bleeding, with a platelet count of 3 x 109/L and positive IgG antiplatelet antibodies test. At a multidisciplinary discussion, it was decided to delay a cesarean section, due to the absence of fetal distress and to the high risk of morbidity for the patient. Many therapies were attempted without success. The IgG produced a slight and transient increase in the platelet count. On the 36th week of gestation, an elective cesarean section was performed. The perioperative period transfusions were guided by rotational thromboelastometry (ROTEM) monitoring. The procedure was performed under general anesthesia and videolaryngoscopy-assisted intubation. The patient was hemodynamically stable, without significant bleeding, and was transferred to the intensive care unit. The platelet count eventually decreased and a splenectomy was performed. Regional anesthesia may be contraindicated, and general anesthesia is associated with an increased risk of airway hemorrhage due to traumatic injury during the tracheal intubation and of hemorrhage associated with the surgical procedure. A multidisciplinary approach is essential in high-risk cases.

Resumo A trombocitopenia é a alteração da hemostase mais comum na gravidez. Contudo, a trombocitopenia grave é rara. Uma das suas causas, a púrpura trombocitopênica imunológica (PTI), é caracterizada pelo aumento da destruição plaquetária por anticorpos de imunoglobulina G (IgG), apresentando alto risco de hemorragia para a paciente e também para o feto, uma vez que os anticorpos podem atravessar a placenta. Apresentamos o caso de uma grávida de 23 anos com histórico de histiocitose de células de Langerhans da mandíbula submetida a cirurgia e quimioterapia aos 10 anos de idade, com diagnóstico de PTI desde então. Na 28a semana de gestação, a paciente apresentou um quadro de petéquias, epistaxe e hemorragia gengival, com contagem plaquetária de 3 x 109/L e teste de anticorpos antiplaquetários IgG positivo. Em uma discussão multidisciplinar, decidiu-se adiar a cesariana devido à ausência de sofrimento fetal e ao alto risco de morbidade para a paciente. Muitas terapêuticas foram tentadas sem sucesso. A IgG produziu apenas um ligeiro e transitório aumento na contagem plaquetária. Na 36ª semana de gestação, foi realizada uma cesariana eletiva. As transfusões no período perioperatório foram guiadas por tromboelastometria rotacional (ROTEM). O procedimento foi realizado sob anestesia geral e intubação assistida por videolaringoscopia. A paciente manteve-se hemodinamicamente estável, sem hemorragia significativa. Ela foi transferida para a unidade de terapia intensiva. A contagem plaquetária continuou a diminuir, e a paciente foi submetida a uma esplenectomia. Nestes casos, a anestesia regional pode ser contraindicada, e a anestesia geral está associada a um risco aumentado de hemorragia das via aéreas devido a lesão traumática durante a intubação traqueal e de hemorragia associada ao procedimento cirúrgico. Uma abordagem multidisciplinar é essencial em casos de alto risco.

Efecto de la Homeopatía en un Paciente con Trombocitopenia Inmunitaria Primaria / Effect of Homeopathy on a Patient with Primary Immune Thrombocytopenia

Se presenta el caso clínico de un paciente masculino de 70 años, que acude en octubre del 2013 a la Unidad Médica Homeopática de la Escuela Nacional de Medicina y Homeopatía, del Instituto Politécnico Nacional (IPN), con el diagnóstico de trombocitopenia inmunitaria primaria, diagnosticada en diciembre del 2010 por el servicio de Hematología de un hospital de alta especialidad de la zona norte de la Ciudad de México, tratado con prednisona y danazol sin resultado, recomendando esplenectomía para la resolución de su problema de salud. El paciente es tratado con Arnica y Phosphorus en potencias bajas y medias respectivamente, medidas generales higiénico-dietéticas y retiro gradual y total de los medicamentos convencionales. Después de 22 meses de tratamiento homeopático las plaquetas se han incrementado a 112,000 por microlitro y no se han presentado zonas equimóticas, ni sangrados activos a otros niveles durante el tratamiento homeopático. (AU)

The clinical case of a male patient aged 70, was admitted in October 2013 at the Homeopathic Medical Unit of the Escuela Nacional de Medicina y Homeopatía, del Instituto Politécnico Nacional (IPN), with the diagnosis of primary immune thrombocytopenia detected in December 2010, for the service of Hematology of a hospital of high specialty of the North zone of Mexico City, that have treated him with prednisone and danazol without result, recommending splenectomy for resolution of your health problem. The patient is treated with Arnica and Phosphorus in low and medium powers respectively, general hygiene and dietary measures and gradual and complete withdrawal of conventional medicines. After 22 months of homeopathic treatment platelets have increased to 112,000 per microliter and there have been no ecchymotic areas, or active bleeding at other levels. (AU)

Trombocitopenia inmune primaria aguda presentación clínica y manejo en pacientes pediátricos, Hospital Mario Catarino Rivas / Acute primary immune thrombocytopenia clinical presentation and management in pediatric patients, Hospital Mario Catarino Rivas

Antecedentes: La trombocitopenia inmune primaria, conocida como púrpura trombocito-pénica idiopática (PTI), es un trastorno autoin-mune adquirido que afecta adultos y niños. Objetivo: Caracterizar los pacientes pediátri-cos con PTI aguda mediante su identi cación y seguimiento en el Hospital Nacional Mario Catarino Rivas (HNMCR) entre 1 febrero del 2016 al 31 enero del 2017. Pacientes y Méto-dos: Estudio cuantitativo, diseño no experi-mental longitudinal ­ prospectivo, alcance descriptivo. La población es todo paciente menor de 18 años de edad, que acudió al HNMCR con PTI aguda. La muestra 47 pacien-tes. Excluidos pacientes previamente diagnos-ticados con PTI, con segundos episodios o recaídas, con patologías sobre agregadas. Información recopilada mediante un instru-mento tipo encuesta: situación demográ ca, área geográ ca y revisión del expediente clíni-co, los datos fueron analizados con el progra-ma EPIINFO y EXCEL. Resultados: En cuanto al grupo etario el 56% fueron menores de cinco años de edad, la distribución por sexos hubo predominio del sexo masculino. 81% de los niños fueron hospitalizados, el resto recibió manejo ambulatorio; sin embargo en el 100% se inició alguna terapia con resolución de la trombocitopenia a los 3 meses. Luego del seguimiento, el diagnóstico nal de los casos captados fue PTI aguda 58%(n=27), evolucio-naron a la cronicidad 20% (n=9), el 22% (n=10) abandonaron seguimiento. Conclusiones: La mayoría de los pacientes con PTI son admitidos al hospital, aunque su conteo de plaquetas no los ponga en riesgo claro de hemorragias espontáneas, por no haber guías de manejo en nuestro hospital...(AU)

Hemangioblastoma cerebeloso y trombocitopenia: reporte de caso / Cerebellar hemangioblastoma and thrombocytopenia: report of one case

The association between vascular tumors and thrombocytopenia is rare. Kasabach-Merritt Syndrome is seen in childhood and is characterized by hemangiomas and thrombocytopenia. A 42 years-old man with a cerebellar hemangioblastoma and thrombocytopenia, admitted with a subarachnoid hemorrhage is reported. The patient was operated and required a splenectomy to manage the thrombocytopenia. After the splenectomy the patient developed a subdural hematoma that was operated. Despite the surgical treatment, the patient died.

Uso de rituximab en trombocitopenia inmune: experiencia en el Hospital de Clínicas / Use of rituximab in immune thrombocytopenia at the University Hospital

Introducción: rituximab es un anticuerpo monoclonal que se une específicamente al antígeno CD20 expresado en los linfocitos B. El uso de rituximab en el tratamiento de la trombocitopenia inmune refractaria no se encuentra aprobado en su ficha técnica. Objetivo: describir las características clínicas, la respuesta terapéutica y los aspectos vinculados a la seguridad con el uso de rituximab en los pacientes con trombocitopenia inmune refractaria asistidos en la Cátedra de Hematología del Hospital de Clínicas y revisar la evidencia sobre el beneficio clínico esperado en este grupo de pacientes. Material y método: se realizó un estudio descriptivo de los pacientes con trombocitopenia inmune refractaria asistidos en la Cátedra de Hematología del Hospital de Clínicas a quienes se les prescribió rituximab. Se realizó una búsqueda bibliográfica en PubMed sobre el uso de rituximab en este tipo de patología. Resultados: se trataron cuatro pacientes con trombocitopenia inmune refractaria con rituximab. Se obtuvo respuesta en tres de cuatro pacientes. La media de tiempo de respuesta fue 9,25 semanas. La respuesta se ha mantenido en los tres pacientes. No se registraron efectos adversos durante la perfusión de rituximab. La evidencia publicada se limita a estudios observacionales, en adultos, con pocos pacientes, habiendo mostrado respuestas favorables. Conclusiones: existen limitaciones en la evidencia sobre el tratamiento de la trombocitopenia inmune refractaria, pero rituximab constituye una alternativa efectiva. Es indispensable la integración clínica para monitorizar la efectividad y seguridad del uso de anticuerpos monoclonales, especialmente en indicaciones no aprobadas...

Cardiac surgery in a patient with severe thrombocytopenia: How low is too low.

Platelets play a very important role in hemostasis, especially after cardiac surgery. Excessive bleeding after such surgery may lead to increased need for transfusion and its incumbent increase in post-operative morbidity and mortality. Although most cardiac surgeons will offer a surgical option to a patient with moderate thrombocytopenia (platelet count around 70 × 10 9 /L), successful cardiac surgery has not been reported in patients with significantly lower platelets counts (less than 40 × 10 9 /L). We report a case of severe thrombocytopenia (19 × 10 9 /L) where coronary artery bypass grafting was performed with minimal blood loss post-operatively, discuss the patient's management and provide insights while dealing with such patients.

Heparin-induced thrombocytopenia: a review of concepts regarding a dangerous adverse drug reaction / Trombocitopenia induzida por heparina: revisão de conceitos de uma importante reação adversa a medicamentos

Heparin is a natural agent with antithrombotic action, commercially available for therapeutic use as unfractionated heparin and low molecular weight heparin. Heparin-induced thrombocytopenia (HIT) is a serious adverse reaction to heparin that promotes antibodymediated platelet activation. HIT is defined as a relative reduction in platelet count of 50% (even when the platelet count at its lowest level is above > 150 x 10(9)/L) occurring within five to 14 days after initiation of the therapy. Thrombocytopenia is the main feature that directs the clinical suspicion of the reaction and the increased risk of thromboembolic complications is the most important and paradoxical consequence. The diagnosis is a delicate issue, and requires a combination of clinical probability and laboratory tests for the detection of platelet activation induced by HIT antibodies. The absolute risk of HIT has been estimated between 1% and 5% under treatment with unfractionated heparin, and less than 1% with low molecular weight heparin. However, high-quality evidence about the risk of HIT from randomized clinical trials is scarce. In addition, information on the frequency of HIT in developing countries is not widely available. This review aims to provide a better understanding of the key features of this reaction and updated information on its frequency to health professionals and other interested parties. Knowledge, familiarity, and access to therapeutic options for the treatment of this adverse reaction are mandatory to minimize the associated risks, improving patient safety.

A heparina é um agente natural com ação antitrombótica, sendo disponibilizadas para uso terapêutico a heparina não fracionadaeaheparina de baixo peso molecular. A trombocitopenia induzida por heparina (TIH) é uma reação adversa grave às heparinas mediada por anticorpos que promovem ativação de plaquetas. A TIH é definida como uma redução rela- tiva na contagem de plaquetas de 50% (mesmo se a contagem de plaquetas no seu nível mais baixo estiver acima 150 x 10(9)/L) que pode ocorrer no período de cinco a 14 dias após o início da terapia com o medicamento. A trombocitopenia é a principal característica que direciona a suspeita clínica da reação, sendo o aumento do risco de complicações tromboembólicas a consequência mais importante e paradoxal. O diagnóstico é uma questão delicada e requer a combinação da probabilidade clínica com testes laboratoriais para detectar a ativação plaquetária induzida pelos anticorpos da TIH. O risco absoluto de TIH tem sido estimado entre 1 e 5% no tratamento com heparina não fracionada e inferior a 1% no uso de heparina de baixo peso molecular. No entanto, evidências de alta qualidade provenientes de ensaios clínicos randomizados sobre a frequência dessa reação são escassas. Além disso, informações sobre a frequência de TIH em países em desenvolvimento não são amplamente disponíveis. Esta revisão teve como objetivo fornecer aos profissionais de saúde e demais interessados um melhor conhecimento sobre a TIH e as principais características dessa reação, bem como apresentar dados atualizados sobre a frequência da mesma. Conhecimento, familiaridade e acesso a opções terapêuticas para o tratamento dessa reação adversa são necessários para minimizar os riscos associados, melhorando a segurança do paciente.

Trombocitopenia inmune primaria refractaria: opciones terapéuticas / Refractory primary immune thrombocytopenia: treatment options

La trombocitopenia inmune primaria (PTI) es un trastorno autoinmune adquirido caracterizado por una disminución transitoria o persistente del recuento de plaquetas con riesgo incrementado de sangramiento. La forma crónica de la enfermedad afecta fundamentalmente a los adultos. Puesto que las remisiones espontáneas son muy poco frecuentes, los pacientes son tratados desde el inicio, usualmente con esteroides. Aproximadamente un tercio de los casos no responden a los regímenes esteroideos y el tratamiento de segunda línea es la esplenectomía, tratamiento con el que se logran las mayores tasas de curación. Sin embargo, entre el 10 y el 30 por ciento de los pacientes no responden a la exéresis quirúrgica del bazo, que constituyen el grupo de pacientes con PTI crónica refractarios al tratamiento. No existen evidencias sobre cuál esquema terapéutico es el más efectivo en estos casos, por lo que el tratamiento continúa siendo empírico. En este trabajo se revisan las diferentes opciones terapéuticas que pueden ser utilizadas en la PTI crónica refractaria, el mecanismo de acción de las drogas, las dosis y los efectos adversos más frecuentes

The primary immune thrombocytopenia (PIT) is an acquired autoimmune disorder characterized by transient or persistent decreased platelet count with increased risk of bleeding. The chronic form of this disease primarily affects adults. Since spontaneous remissions are rare, patients usually are treated with steroids from the start. Approximately one third of the cases does not respond to steroid regimens; the second-line treatment is splenectomy, achieving the highest cure rates. However, between 10 to 30 percent of patients do not respond to spleen surgical removal, which is the group of patients with refractory chronic PIT. There is no evidence on whether this therapeutic regimen is most effective in these cases, so the treatment remains empirical. In this paper, various treatment options that can be used in refractory chronic PIT, the action mechanism of drugs, doses and more frequent adverse effects were reviewed