Sepsis por celulitis necrotizante en paciente embarazada con sindrome antifosfolípido en tratamiento / Necrotizing sepsis cellulitis pregnant patient with antiphospholipid syndrome treatment

Introducción: El término ''síndrome antifosfolipídico'' (SAF) describe la asociación de los anticuerpos antifosfolipídicos (AAF) con un cuadro clínico de hipercoagulabilidad caracterizado por trombosis a repetición y abortos recurrentes. Objetivo: Presentar un caso de celulitis severa de periné en paciente con SAF y tratamiento con hidroxicloroquina. Caso clínico: Paciente de 39 años con embarazo de término con SAF tratado con hidroxicloroquina y anticoagulación que desarrolló una infección severa de partes blandas del periné que fue tratado con interrupción del embarazo, drenaje agresivo del periné y tratamiento antibiótico extenso con buena evolución. Conclusión: La asociación del tratamiento con hidroxicloroquina, embarazo y una complicación séptica es incierta. El tratamiento con inmunosupresión no es estándar y podría haber favorecido el mal pronóstico del cuadro clínico. (AU)

Objetive: To present a case of severe perineal cellulitis in a pregnant patient with Antiphospholipid syndrome treated wiht hidroxicloroquine. Case report: A 39 years old female pregnant patient with AFS treated with hidroxicloroquine and heparin developed severe perineal infection with systemic impairment. Final treatment included aggressive perineal drainage in multiple sessions, pregnancy delivered and systemic treatment with wide spectrum antibiotics and general measures. Discusion and Conclusion: Treatment with hidroxicloroquine, pregnancy and septic complication is infrequent. This approach is not standard and it could favored worst prognostic of the general syndrome. (AU)

Microabscesos hepáticos en una paciente con síndrome antifosfolipidos. Reporte de caso / Liver microabscesses in a patient with antiphospholipid syndrome. Case report

Los abscesos hepáticos en la actualidad se siguen considerando un reto diagnóstico. Estos pueden dividirse en tres categorías principales según las condiciones subyacen­ tes: infecciosas, malignas e iatrogénicas. Incluyen aquellos secundarios a la extensión directa de una infección local, bacteriemia sistémica e infecciones intraabdominales procedente de la porta, Sin embargo, a lo largo de los años, con los estudios diagnósti­ cos la lista de factores de riesgo aumento, obligando a mas investigaciones para su en­ tendimiento; logrando su pronto reconocimiento y tratamiento eficaz con el fin de obtener buenos resultados. Se presenta un caso de femenino con antecedentes de sín­ drome antifosfolípidos con dolor abdominal asociado a intolerancia a la vía oral. Image­ nología abdominal muestra lesiones compatibles con microabscesos hepáticos siendo imposible la toma de muestra, requiriendo cubrimiento antibiótico de amplio espectro con resolución clínico radiológico completa. Tac de abdomen que muestra lesiones hepáticas múltiples con discreta colestasis intrahepática, lesiones compatibles con mi­ croabscesos múltiples.

Liver abscesses are currently still considered a diagnostic challenge. These can be divi­ ded into three main categories according to the underlying conditions: infectious, malig­ nant and iatrogenic. They include those secondary to the direct extension of a local infection, systemic bacteraemia and intra­abdominal infections from the portal, however, over the years, with diagnostic studies the list of risk factors increased, forcing more re­ search for its understanding; achieving its prompt recognition and effective treatment in order to obtain good results. A case of a female with a history of antiphospholipid syn­ drome with abdominal pain associated with oral intolerance is presented. Abdominal imaging shows lesions compatible with hepatic microabscesses, the sampling being im­ possible, requiring broad­spectrum antibiotic coverage with complete radiological clini­ cal resolution. Abdominal tac showing multiple liver lesions with discrete intrahepatic cholestasis, lesions compatible with multiple microabscesses.

Anti-phospholipid syndrome in seven leprosy patients with thrombotic events on corticosteroid and/or thalidomide regimen: insights on genetic and laboratory profiles

Abstract INTRODUCTION Corticosteroids and/or thalidomides have been associated with thromboembolism events (TBE) in multibacillary (MB) leprosy. This report aimed to determine genetic and laboratory profiles associated with leprosy and TBE. METHODS Antiphospholipid antibodies (aPL), coagulation-related exams, prothrombin and Leiden's factor V mutations, and ß2-glycoprotein-I (ß2GPI) Val247Leu polymorphism were assessed. RESULTS Six out of seven patients with leprosy were treated with prednisone and/or thalidomide during TBE and presented at least one positive aPL. All patients presented ß2GPI polymorphism, and one showed prothrombin mutation. CONCLUSIONS Corticosteroid or thalidomide adverse effects and aPL and ß2GPI polymorphisms may cause TBE in patients with MB leprosy.

Diagnosis and treatment of antiphospholipid antibody-related recurrent spontaneous abortion and analysis of therapeutic drugs and pregnancy outcome in 75 patients with antiphospholipid syndrome / 北京大学学报(医学版)

Recurrent spontaneous abortion is one of the common complications in women of childbearing age during pregnancy. The immune factor accounts for a large proportion of many causes. Antiphospholipid antibody syndrome is the most common type of acquired thrombophilia disease. Autoimmune diseases that cause thrombosis and obstetric complications under the action of antibodies are also the most common type of immune-related recurrent abortion. At present, there is no unified opinion on the treatment of this disease, especially the treatment of immunoglobulins and other drugs like glucocorticoid. Here we reviewed the progress of diagnosis and treatment of antiphospholipid antibody-related recurrent abortions and retrospectively analyzed and summarized the drug regimens and pregnancy outcomes of this disease with pregnancy patients in our hospital. A total of 75 patients were included. According to their clinical manifestations and laboratory results, these patients were basically divided into two categories: classical antiphospholipid syndrome and non-classical antiphospholipid syndrome. The latter was further divided into serum-negative antiphospholipid syndrome and antiphospholipid antibody-related recurrent abortion patients based on their clinical manifestations and antiphospholipid antibody results. The patients were divided into four categories: aspirin + hydroxychloroquine, aspirin + low molecular weight heparin, aspirin + low molecular weight heparin + hydroxychloroquine, aspirin + hydroxychloroquine + low molecular weight heparin + low dose glucocorticoids. Among them, aspirin + hydroxychloroquine + low molecular weight heparin + low dose glucocorticoid treatment regimen was most commonly used. Most of the patients who received the above different treatment regimens achieved full-term infants, and a small number of patients had adverse pregnancy outcomes, such as premature delivery, placental abruption, eclampsia, and fetal malformation. And adverse pregnancy outcomes also occurred in this group. It might be related to the severity of the disease and the potential adverse effects of maternal fetal. However, further statistical analysis is needed for the risk factors affecting the pregnancy outcome of this part of patients.

Nuevas estrategias en el tratamiento del síndrome antifosfolípido / New alternatives for the treatment of antiphospholipid syndrome. A literature review

For years the mainstay of antiphospholipid syndrome treatment has been anticoagulation and antiplatelet therapy, but the autoimmune nature of the disease, and complications of these therapies, created the need to develop new therapeutic strategies. New therapeutic alternatives inhibit at different levels, the cascade of events leading to the pro-thrombotic state characteristic of the antiphospholipid syndrome. We conducted a literature review of these new treatments, focusing on the pathophysiological bases that support them and their possible clinical applications.

Diretrizes para o tratamento da síndrome do anticorpo antifosfolipídeo / Guidelines for the treatment of antiphospholipid syndrome

A síndrome do anticorpo antifosfolipídeo (SAF) é uma doença sistêmica autoimune caracterizada por trombose arterial e venosa, morbidade gestacional e presença de níveis séricos de anticorpos antifosfolipídeos elevados e persistentemente positivos. O tratamento da SAF ainda é sujeito a controvérsias, já que qualquer decisão terapêutica potencialmente irá confrontar-se com o risco de uma cobertura antitrombótica insuficiente ou com o risco excessivo associado à anticoagulação e seus principais efeitos adversos. Esta diretriz foi elaborada a partir de nove questões clínicas relevantes e relacionadas ao tratamento da SAF pela Comissão de Vasculopatias da Sociedade Brasileira de Reumatologia. O objetivo deste trabalho foi criar uma diretriz que incluísse as questões mais relevantes e controversas no tratamento da SAF, com base na melhor evidência científica disponível. As questões foram estruturadas por meio do P.I.C.O. (paciente, intervenção ou indicador, comparação e outcome/desfecho), o que possibilitou a geração de estratégias de busca da evidência nas principais bases primárias de informação científica (MEDLINE/Pubmed, Embase, Lilacs/Scielo, Cochrane Library, Premedline via OVID). Também realizou-se busca manual da evidência e de teses (BDTD e IBICT). A evidência recuperada foi selecionada a partir da avaliação crítica, utilizando instrumentos (escores) discriminatórios de acordo com a categoria da questão terapêutica (JADAD para ensaios clínicos randomizados e New Castle Ottawa Scale para estudos não randomizados). Após definir os estudos potenciais para sustento das recomendações, eles foram selecionados pela força da evidência e pelo grau de recomendação, segundo a classificação de Oxford.

The antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by arterial and venous thrombosis, gestational morbidity and presence of elevated and persistently positive serum titers of antiphospholipid antibodies. The treatment of APS is still controversial, because any therapeutic decision potentially faces the risk of an insufficient or excessive antithrombotic coverage associated with anticoagulation and its major adverse effects. This guideline was elaborated from nine relevant clinical questions related to the treatment of APS by the Committee of Vasculopathies of the Brazilian Society of Rheumatology. Thus, this study aimed at establishing a guideline that included the most relevant and controversial questions in APS treatment, based on the best scientific evidence available. The questions were structured by use of the PICO (patient, intervention or indicator, comparison and outcome) process, enabling the generation of search strategies for evidence in the major primary scientific databases (MEDLINE/PubMed, Embase, Lilacs, Scielo, Cochrane Library, Premedline via OVID). A manual search for evidence and theses was also conducted (BDTD and IBICT). The evidence retrieved was selected based on critical assessment by using discriminatory instruments (scores) according to the category of the therapeutic question (JADAD scale for randomized clinical trials and Newcastle-Ottawa scale for non-randomized studies). After defining the potential studies to support the recommendations, they were selected according to level of evidence and grade of recommendation, according to the Oxford classification.

Management of acquired thrombophilic disorders in 2011: focus on heparin-induced thrombocytopenia, antiphospholipid syndrome, myeloproliferative neoplasms and paroxysmal nocturnal hemoglobinuria

Arterial and venous thrombosis are interrelated disorders at the interplay of platelets and fibrin. Arterial thrombi are platelet-rich and occur at sites vulnerable to atherosclerotic plaque rupture where blood shear rates are high; on the contrary, venous thrombi occur in association with slow blood flow and shear rates. These differences may underlie why anti-platelet agents are more effective in prevention of arterial thrombosis, while anticoagulants are preferred for venous thrombosis. Although some common thrombophilic disorders [e.g., Factor V Leiden, prothrombin gene mutation, etc.] are almost exclusively associated with venous thromboembolism, there are several disorders that are important to consider when caring for patients with both arterial and venous thromboembolism. This article will review the evidence-based management of heparin induced thrombocytopenia with thrombosis, anti-phospholipid antibody syndrome and catastrophic anti-phospho-lipid antibody syndrome, thrombohemorrhagic manifestations of Philadelphia chromosome-negative chronic myeloproliferative neoplasms including polycythemia vera, essential thrombocythemia and primary myelofibrosis, as well as paroxysmal nocturnal hemoglobinuria

Pregnancy outcome in women with antiphospholipid syndrome on low-dose aspirin and heparin: a retrospective study

This retrospective review of hospital records analysed pregnancy outcome with 2 different treatments for women with recurrent miscarriage diagnosed with antiphospholipid syndrome in the index pregnancy. Of 64 women, 29 had received aspirin and 35 aspirin plus heparin. Pregnancy-induced hypertension, prematurity, intrauterine growth restriction and neonatal death were considered as maternal and fetal complications. There were no significant differences in antenatal and maternal complications between the groups. However, there were significant differences in mean anticardiolipin IgG antibody levels. Aspirin alone or in combination with heparin was equally efficacious in women with antiphospholipid syndrome and recurrent miscarriage

Anticuerpos antifosfolípidos. Valoración clínica actual / Antiphospholipid antibodies. Current clinical assessment

El síndrome anticuerpo antifosfolipido (SAF) constituye una enfermedad autoinmune que se caracteriza por presentar trombosis vascular y abortos recurrentes, asociada a la elevación persistente de anticuerpos antifosfolípidos séricos. Los eventos trombóticos pueden ocurrir tanto en las arterias y venas como en la microcirculación. Las manifestaciones clínicas dermatológicas más frecuentes son livedo reticularis, vasculitis livedoide y úlceras, entre otras. En este trabajo se actualizan los conceptos de su etiología y los criterios diagnósticos corregidos desde el año 2004. Además se plantea un algoritmo diagnóstico y las pautas de tratamiento actuales.

Síndrome do anticorpo antifosfolipídio e gravidez / Antiphospholipid syndrome and pregnancy

A Síndrome do Anticorpo Antifosfolipídio (SAF) é uma doença sistêmica adquirida, com manifestações clínicas predominantes de trombose venosa ou arterial, abortos recorrentes, trombocitopenia, restrição do crescimento intra-uterino, oligodrâmnio inexplicável, pré-eclampsia, óbito fetal, descolamento prematuro de placenta e a presença no soro dos anticorpos antifosfolipídios (AAF), expressos pelo anticopo anticardiolipina e o anticoagulante lúpico. Esses anticorpos são detectáveis em mais de 5 porcento das pacientes saudáveis e em mais de 50 porcento das pacientes lúpicas. A fisiopatogenia da SAF está relacionada à inibição das reações na cascata de coagulação, interação com as plaquetas e com células endoteliais, pelos AAF. O diagnóstico primário é clínico, com a paciente apresentando eventos trombóticos e/ou perdas fetais recorrentes. Devido à associação relativamente comum entre SAF e gestação, objetivou-se, com a presente revisão, apresentar de forma atualizada, aspectos referentes à fisiopatologia, diagnóstico e tratamento dessa intercorrência clínica, associados à gravidez

Maternal and fetal complications of antiphospholipid syndrome: a case report with long-term follow-up

We report a case of a 26- year old woman having antiphospholipid syndrome in pregnancy with long-term follow up. She presented with recurrent miscarriages, venous thrombosis, avascular necrosis of femoral head, mid-cerebral artery infarction and skin ulcers. Antiphospholipid syndrome is a recognized disorder of pregnancy. Diagnosis requires a high index of suspicion when evaluating women with recurrent pregnancy losses and vascular thrombosis. A low dose aspirin combined with heparin can reduce morbidity and improves the pregnancy outcome

Trombofilias e aborto habitual / Recurrent miscarriage and thrombophilias

O aborto habitual é evento relativamente freqüente na população obstétrica. Sua etiologia permanece desconhecida em cerca de 40 porcento dos casos. Entre as causas imunológicas, a prevalência da síndrome antifosfolipídeo é alta e, atualmente, outras trombofilias têm sido associadas a casos de etiologia desconhecida. O tipo e a magnitude desta associação varia conforme a população estudada e tipo de perda fetal, fatores importantes para racionalizar a solicitação de exames diagnósticos de custo elevado. O tratamento atualmente preconizado consiste no uso de heparina associada ou não a aspirina; porém, estudos adicionais são necessários para se comprovar a real eficácia e a segurança desta terapêutica

Enoxaparina y aspirina a bajas dosis en el tratamiento de los síndromes antifosfolípicos del embarazo. Seguridad y utilidad / Low dose enoxaparine and aspirin in the therapy of antiphospholipid syndromes in pregnancy. Safety and usefulness

La pérdida recurrente de embarazo constituye una complicación de los síndromes antifosfolipídicos, con graves consecuencias para las parejas, su entorno familiar y la sociedad. El objetivo de este estudio fue: determinar la efectividad de la heparina de bajo peso molecular (enoxaparina) asociada a bajas dosis de aspirinas, en mujeres con pérdidas recurrentes de embarazo (PRE) vinculada a la presencia de anticuerpos antifosfolípidos (aPL). Determinar la seguridad de estos tratamientos en la madre y en el feto vigilando la aparición de sangrado o plaquetopenia vinculada a la heparina. Para ello se identificó a una población de 76 mujeres con síndrome antifosfolipídico y PRE. Todas cursaron por lo menos un nuevo embarazo tratado con las drogas mencionadas. De 76 nuevos embarazos, 69 llegaron a término con por lo menos un hijo vivo. En la historia previa de las pacientes se registra un 3,8 por ciento de embarazos con hijos vivos mientras que cuando son tratadas 89,5 por ciento de los embarazos resultan exitosos (IC al 95 por ciento para la probabilidad de éxito del tratamiento indica que la misma está comprometida entre el 82,6 por ciento y el 92,4 por ciento). La comparación de proporciones indica que la diferencia es altamente significativa (p-valor del orden 10 a la -8). En relación a los efectos secundarios no se observaron sangrados fetales ni maternos ni plaquetopenias inducidas por heparina. Concluimos que el tratamiento con Enoxaparina y aspirina a bajas dosis es altamente efectivo y seguro para tratar los síndromes antifosfolipícos del embarazo y por lo tanto no debería esperarse PRE sino que las deberían estudiarse siempre que se sospeche el diagnóstico para poder tratarlas en forma adecuada.