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Antecedentes y objetivo: El término microangiopatía trombótica (MAT) incluye un grupo heterogéneo de enfermedades potencialmente mortales o invalidantes, rápidamente evolutivas, caracterizadas por anemia hemolítica microangiopática y trombocitopenia. La actuación en las primeras horas es crucial para mejorar el pronóstico de los pacientes. El objetivo de esta revisión es proporcionar recomendaciones orientadas a optimizar el tratamiento inicial de la MAT y agilizar el diagnóstico etiológico. Pacientes y métodos: Se diseña una guía práctica en la cual se diferencian cuatro apartados en el abordaje inicial de las MAT: sospecha diagnóstica, confirmación sindrómica, tratamiento urgente y estudios complementarios. Resultados: La detección de anemia hemolítica microangiopática (caracterizada por aumento de reticulocitos, LDH y bilirrubina indirecta, Coombs directo negativo y esquistocitos en el frotis de sangre periférica) y trombocitopenia no justificable por otras causas secundarias confirma el diagnóstico sindrómico de anemia hemolítica microangiopática y trombocitopenia (AHMAT). Estos pacientes requieren ingreso en la unidad de cuidados intensivos para iniciar lo antes posible el recambio plasmático, preferiblemente en las primeras 4-8h. Antes de realizar el recambio plasmático deben extraerse las muestras para el estudio de ADAMTS13 y de complemento. Finalmente, es importante solicitar las pruebas complementarias necesarias para obtener un correcto diagnóstico etiológico. Conclusiones: La puesta en práctica de las recomendaciones consensuadas en esta guía permitirá mejorar los resultados terapéuticos al facilitar la cooperación de los distintos especialistas implicados en la atención de las MAT

Background and aim: The term thrombotic microangiopathy (TMA) involves a heterogeneous group of diseases that can be overwhelming or invalidating, with an acute development, characterised by microangiopathic haemolytic anaemia and thrombocytopaenia. Its management during its initial hours is essential to improving the prognostic of these patients. The aim of this review is to give recommendations about the optimisation of TMA initial treatment and to accelerate the aetiological diagnosis. Patients and methods: We provide a practice guideline based on four steps for the initial management of TMA: diagnosis of suspicion, syndromic confirmation, emergent treatment and complementary tests. Results: The detection of microangiopathic haemolytic anaemia (characterised by elevated reticulocytes, LDH and indirect bilirubin, negative direct Coombs test and schistocytes in peripheral blood), and thrombocytopaenia not explained by other secondary aetiologies confirm the syndromic diagnosis of microangiopathic haemolytic anaemia and thrombocytopaenia (MAHAT). These patients require admission to an Intensive Care Unit to initiate plasma exchange therapy as soon as possible, ideally within the first 4-8hours. Prior to this, samples for ADAMTS13 and complement study should be obtained. Finally, it is important to request the complementary tests necessary to have a correct aetiological diagnosis. Conclusions: Adherence to the agreed recommendations in this guideline will improve therapeutic results by facilitating cooperation between different specialists involved in TMA management

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BACKGROUND AND AIM: The term thrombotic microangiopathy (TMA) involves a heterogeneous group of diseases that can be overwhelming or invalidating, with an acute development, characterised by microangiopathic haemolytic anaemia and thrombocytopaenia. Its management during its initial hours is essential to improving the prognostic of these patients. The aim of this review is to give recommendations about the optimisation of TMA initial treatment and to accelerate the aetiological diagnosis. PATIENTS AND METHODS: We provide a practice guideline based on four steps for the initial management of TMA: diagnosis of suspicion, syndromic confirmation, emergent treatment and complementary tests. RESULTS: The detection of microangiopathic haemolytic anaemia (characterised by elevated reticulocytes, LDH and indirect bilirubin, negative direct Coombs test and schistocytes in peripheral blood), and thrombocytopaenia not explained by other secondary aetiologies confirm the syndromic diagnosis of microangiopathic haemolytic anaemia and thrombocytopaenia (MAHAT). These patients require admission to an Intensive Care Unit to initiate plasma exchange therapy as soon as possible, ideally within the first 4-8hours. Prior to this, samples for ADAMTS13 and complement study should be obtained. Finally, it is important to request the complementary tests necessary to have a correct aetiological diagnosis. CONCLUSIONS: Adherence to the agreed recommendations in this guideline will improve therapeutic results by facilitating cooperation between different specialists involved in TMA management.

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Background. Invasive fungal disease (IFD) treatment is challenging in hematologic patients due to drug interactions and toxicities that limit the use of the antifungal agents. Aims. To analyze retrospectively in terms of safety and potential efficacy anidulafungin therapy, alone or in combination. Methods. Our institutional guidelines recommended anidulafungin treatment in hematologic patients with suspected IFD and concomitant renal or liver impairment (to avoid drug interactions and preserve organ function). Results. From 2008 to 2013, 24 episodes of IFD occurring in 21 patients were classified as proven (4 cases), probable (15 cases) and possible (5 cases). Anidulafungin was administered alone (13%) or in combination (88%). Eight (33%) episodes were resolved, using monotherapy (1 out of 3, 33%) or a combined therapy (7 out of 21, 33%). Twelve cases (50%) were registered as failure (death due to IFD progression in 4 patients, and treatment change due to lack of efficacy in 8), and 4 cases (17%) were not evaluable (death unrelated to the IFD). Anidulafungin was not withdrawn in any case due to toxicity. Conclusions. Anidulafungin therapy, alone or in combination, could be considered in hematologic patients with IFD and concomitant liver or renal impairment. Due to the low number of patients, we cannot draw any conclusion about efficacy (AU)

Antecedentes. El tratamiento de una infección fúngica invasiva (IFI) supone un importante desafío en los pacientes hematológicos debido a las interacciones farmacológicas y a la toxicidad de los agentes antifúngicos, que restringen su uso. Objetivos. Analizar de forma retrospectiva el tratamiento con anidulafungina, sola o combinada, en términos de su seguridad y posible eficacia. Métodos. En los pacientes hematológicos con sospecha de IFI e insuficiencia renal o hepática concomitante, las guías clínicas de nuestro entorno recomendaban el tratamiento con anidulafungina (para evitar las interacciones farmacológicas y preservar la función orgánica). Resultados. De 2008 a 2013 se documentaron 24 episodios de IFI en 21 pacientes, que se clasificaron como IFI demostrada (4 casos), IFI probable (15 casos) e IFI posible (5 casos). Se administró anidulafungina como monoterapia (13%) y en combinación (88%). Se resolvieron 8 episodios (33%), 1 caso de 3 tratados con monoterapia (33%) y 7 casos de 21 tratados con terapia combinada, (33%). En 12 casos (50%), el tratamiento fracasó (muerte por progresión de la IFI en 4 pacientes y cambio de tratamiento por falta de eficacia en 8). Por último, 4 casos (17%) no se pudieron evaluar (muerte no relacionada con IFI). En ningún caso se retiró el tratamiento con anidulafungina por toxicidad. Conclusiones. El tratamiento con anidulafungina, sola o combinada, podría considerarse apropiado para pacientes hematológicos con IFI e insuficiencia hepática o renal concomitante. Debido al reducido número de pacientes incluidos, no es posible extraer conclusiones respecto a la eficacia(AU)

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BACKGROUND: Invasive fungal disease (IFD) treatment is challenging in hematologic patients due to drug interactions and toxicities that limit the use of the antifungal agents. AIMS: To analyze retrospectively in terms of safety and potential efficacy anidulafungin therapy, alone or in combination. METHODS: Our institutional guidelines recommended anidulafungin treatment in hematologic patients with suspected IFD and concomitant renal or liver impairment (to avoid drug interactions and preserve organ function). RESULTS: From 2008 to 2013, 24 episodes of IFD occurring in 21 patients were classified as proven (4 cases), probable (15 cases) and possible (5 cases). Anidulafungin was administered alone (13%) or in combination (88%). Eight (33%) episodes were resolved, using monotherapy (1 out of 3, 33%) or a combined therapy (7 out of 21, 33%). Twelve cases (50%) were registered as failure (death due to IFD progression in 4 patients, and treatment change due to lack of efficacy in 8), and 4 cases (17%) were not evaluable (death unrelated to the IFD). Anidulafungin was not withdrawn in any case due to toxicity. CONCLUSIONS: Anidulafungin therapy, alone or in combination, could be considered in hematologic patients with IFD and concomitant liver or renal impairment. Due to the low number of patients, we cannot draw any conclusion about efficacy.

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