IBD-PODCAST Spain: A Close Look at Current Daily Clinical Practice in IBD Management.

BACKGROUND: Crohn's disease (CD) and ulcerative colitis (UC) are inflammatory bowel diseases (IBD) that contributes in part to irreversible bowel damage and long-term complications, reduced quality of life, invalidity, and economic burden. Suboptimal control of IBD is associated with higher healthcare resource utilization (HCRU), impaired quality of life (QoL), and reduced work productivity. AIMS: The IBD-PODCAST study aimed to assess the proportion of IBD patients with suboptimal control and its associated impact. METHODS: IBD-PODCAST is a cross-sectional, multicenter study that aimed to characterize the CD and UC population with optimal or suboptimal control according to the STRIDE-II criteria and patient- and physician-reported measures. Here we present the results of the Spanish cohort (n = 396). RESULTS: A total of 104/196 (53.1%) CD and 83/200 (41.5%) UC patients were found to have suboptimal disease control. Long-term treatment targets according to STRIDE-II were applied in 172 (87.8%) CD and 181 (90.5%) UC patients. 125 of 172 (72.7%) CD and 74 of 181 (40.9%) UC patients were currently treated with targeted immunomodulators. Patients with CD and UC and suboptimal disease control showed impaired QoL, higher HCRU and direct costs, and also loss of work productivity compared to those with optimal control. CONCLUSION: Despite a high rate of targeted immunomodulator therapy, a substantial proportion of IBD patients show suboptimal disease control according to the STRIDE II criteria. Those patients with suboptimal disease control exhibit impaired QoL, less work productivity, and higher HCRU, suggesting that there is considerable need for better treatment approaches in IBD.

Improving the quality of care in the molecular era for children and adolescents with medulloblastoma.

PURPOSE: Elevated mortality and morbidity rates persist in pediatric patients with medulloblastoma. We present a clinical audit of a real-world cohort of patients in search for pragmatic measures to improve their management and outcome. METHODS/PATIENTS: All pediatric patients with medulloblastoma treated between 2003 and 2016 at a Spanish reference center were reviewed. In the absence of internationally accepted quality indicators (QIs) for pediatric CNS tumors, diagnostic, therapeutic, survival, and time QIs were defined and assessed. RESULTS: Fifty-eight patients were included, 24% were younger children (< 3 years), 36% high risk (anaplastic, metastasis, or surgical residue > 1.5 cm2), and 40% standard risk. Five-year OS was 59.2% (95% CI 47-75); 5-year PFS 36.4% (95% CI 25-53). Five main areas of quality assurance were identified: diagnosis, global strategy, frontline treatment modalities, outcomes, and long-term and end-of-life care. A set of 34 QIs was developed and applied. Lack of central pathology review, delay in the incorporation of novel molecular markers, and absence of a neurocognitive and quality-of-life evaluation program were some of the audit findings. CONCLUSIONS: This real-world research study resulted in the development of a pragmatic set of QIs, aimed to improve clinical audits and quality of care given to children and adolescents with medulloblastoma. We hope that our findings will serve as a reference to further develop a quality assurance system with specific QIs for pediatric CNS tumors in the future and that this will ultimately improve the survival and quality of life of these patients.

Outcome of childhood leukaemia survivors and necrosis of the femoral head treated with autologous mesenchymal stem cells

Purpose. Corticoid-induced osteonecrosis (ON) of femoral head can lead to severe hip joint impairment and hip replacement, with negative impact in young survivors of acute lymphoblastic leukaemia (ALL) with long life expectancy. We aim to improve quality of life in these patients with a novel approach. Methods/patients. Based on the regenerative capacities of mesenchymal stem cells (MSCs), we performed locally implanted autologous cell therapy in two adolescents suffering of bilateral femoral ON. This required a simple, minimally invasive surgical procedure. Results. Both patients experienced significant pain relief and restoration of gait kinematic values. Radiographic evaluation showed cessation of hip collapse. No toxicities/complications were observed after a 4-year follow-up. Conclusions. Our preliminary results suggest that autologous MSCs can be considered as a novel treatment for children and young adults with ON after overcoming ALL. It may avoid hip replacement and improve quality of life of leukaemia survivors

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Outcome of childhood leukaemia survivors and necrosis of the femoral head treated with autologous mesenchymal stem cells.

PURPOSE: Corticoid-induced osteonecrosis (ON) of femoral head can lead to severe hip joint impairment and hip replacement, with negative impact in young survivors of acute lymphoblastic leukaemia (ALL) with long life expectancy. We aim to improve quality of life in these patients with a novel approach. METHODS/PATIENTS: Based on the regenerative capacities of mesenchymal stem cells (MSCs), we performed locally implanted autologous cell therapy in two adolescents suffering of bilateral femoral ON. This required a simple, minimally invasive surgical procedure. RESULTS: Both patients experienced significant pain relief and restoration of gait kinematic values. Radiographic evaluation showed cessation of hip collapse. No toxicities/complications were observed after a 4-year follow-up. CONCLUSIONS: Our preliminary results suggest that autologous MSCs can be considered as a novel treatment for children and young adults with ON after overcoming ALL. It may avoid hip replacement and improve quality of life of leukaemia survivors.

Management and outcome of children with neuroendocrine tumors of the appendix in Spain: is there room for improvement?

Purpose. Neuroendocrine tumors (NETs) are, after lymphomas, the most frequent gastrointestinal tumors in children, mainly located in the appendix. Best management remains unclear, given the absence of pediatric guidelines. We present the first Spanish series of pediatric patients with NETs. Patients and methods. Retrospective study of all pediatric patients (<18 years) with NET treated in four oncology reference institutions in Spain between 1994 and 2015. Results. Seventeen patients were included. All patients presented with acute abdomen. TNM stage was T1a (82%) and T1b (12%). Extension study was heterogenous, with only 4 patients undergoing an OctreoScan. Four patients met criteria for second surgery (affected surgical margins or mesoappendix invasion), but it was only performed in two. Despite the diverse management, none of the patients relapsed during follow-up. Conclusions. The disparity in diagnostic tests, second surgery criteria and follow-up shown in this study highlights the need for specific pediatric guidelines (AU)

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Management and outcome of children with neuroendocrine tumors of the appendix in Spain: Is there room for improvement?

PURPOSE: Neuroendocrine tumors (NETs) are, after lymphomas, the most frequent gastrointestinal tumors in children, mainly located in the appendix. Best management remains unclear, given the absence of pediatric guidelines. We present the first Spanish series of pediatric patients with NETs. PATIENTS AND METHODS: Retrospective study of all pediatric patients (<18 years) with NET treated in four oncology reference institutions in Spain between 1994 and 2015. RESULTS: Seventeen patients were included. All patients presented with acute abdomen. TNM stage was T1a (82%) and T1b (12%). Extension study was heterogenous, with only 4 patients undergoing an OctreoScan. Four patients met criteria for second surgery (affected surgical margins or mesoappendix invasion), but it was only performed in two. Despite the diverse management, none of the patients relapsed during follow-up. CONCLUSIONS: The disparity in diagnostic tests, second surgery criteria and follow-up shown in this study highlights the need for specific pediatric guidelines.

Landscape of early clinical trials for childhood and adolescence cancer in Spain

Purpose: Despite numerous advances, survival remains dismal for children and adolescents with poor prognosis cancers or those who relapse or are refractory to first line treatment. There is, therefore, a major unmet need for new drugs. Recent advances in the knowledge of molecular tumor biology open the door to more adapted therapies according to individual alterations. Promising results in the adult anticancer drug development have not yet been translated into clinical practice. We report the activity in early pediatric oncology trials in Spain. Methods: All members of the Spanish Society of Pediatric Hematology Oncology (SEHOP) were contacted to obtain information about early trials open in each center. Results: 22 phase I and II trials were open as of May 2015: 15 for solid tumors (68 %) and 7 for hematological malignancies (32 %). Fourteen (64 %) were industry sponsored. Since 2010, four centers have joined the Innovative Therapies For Children With Cancer, an international consortium whose aim is developing novel therapies for pediatric cancers. A substantial number of studies have opened in these 5 years, improving the portfolio of trials for children. Results of recently closed trials show the contribution of Spanish investigators, the introduction of molecularly targeted agents and their benefits. Conclusions: Clinical trials are the way to evaluate new drugs, avoiding the use of off-label drugs that carry significant risks. The Spanish pediatric oncology community through the SEHOP is committed to develop and participate in collaborative academic trials, to favor the advancement and optimization of existing therapies in pediatric cancer (AU)

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Establecimiento de un modelo preclínico de neuroblastoma en ratones inmunocompetentes / Establishment of a preclinical neuroblastoma model in immunocompetent mice

Objetivo. Desarrollar un modelo animal de neuroblastoma (NB) que posibilite estudios relacionados con la inmunidad tumoral. Material y métodos. Se utilizaron dos tipos de células NB. La línea 36769 procedía del ratón TH-MYCN+ en el que la sobreexpresión del gen MYCN está gobernada por el promotor de la tirosín hidroxilasa de rata. La línea 4040 procedía de ratones TH-MYCN+/ALK+, que además expresan una mutación activadora del gen ALK. De cada tipo celular se implantaron 1x106 neurosferas en ratones 129/SVJ (mismo fondo genetico que los donantes, n=8), mediante inyección ortotópica en glándula suprarrenal izquierda por abordaje intraperitoneal, a través de laparotomía transversa supraumbilical. Se realizó seguimiento clínico diario postquirúrgico de los animales hasta su sacrificio a las 4 semanas. La presencia de tumor se confirmó macroscópicamente. La pieza tumoral se extirpó y se procesó para estudios de inmunidad celular y mediadores moleculares de tolerancia. Se investigó la existencia de metástasis por citometría de flujo en bazo, médula ósea y sangre periférica. Resultados. 1) En todos los ratones trasplantados se generó NB ortotópico. 2) La pieza tumoral se encontró infiltrada por diversas subpoblaciones inmunes, con inmunofenotipo efector, regulador y supresor, similar a la situación descrita en los NB humanos. Además, los mediadores moleculares del microambiente apuntan a un estado de tolerancia protumoral. Conclusiones. La implantación ortotópica de neurosferas NB en ratones singénicos nos ha permitido generar un modelo de NB en el que ha sido posible estudiar la inmunidad tumoral

Aim. To develop a NB animal model which makes possible studies related to tumor immunity. Materials and methods. Two types of NB cells were used. Cell line 36769 was derived from TH-MYCN+ mouse in which overexpression of the MYCN gene is governed by rat tyrosine hydroxylase promotor. Cell line 4040 was derived from TH-MYCN/ALK mice, which in addition express an activating mutation of ALK gene. For each cell type, 1x106 neurospheres were implanted in 129/SVJ mice (with the same genetic background as donors, n=8), via orthotopic injection in the left suprarenal gland by intraperitoneal approach, through a transverse supraumbilical laparotomy. Daily postsurgical clinical follow-up of the animals was done until they were sacrificed at four weeks. The tumor presence was macroscopically confirmed. The tumoral sample was excised and was processed for cellular immunity and molecular tolerance mediator’s studies. The existence of metastasis was investigated by flow cytometry in the spleen, bone marrow and peripheral blood. Results. 1) Orthotopic Neuroblastoma was generated in all the transplanted mice. 2) The tumors were infiltrated by several immune subpopulations, with effector, regulatory and suppressor inmunophenotype. This was similar to the inmunophenotype described in human NB. Furthermore, the molecular mediators of the environment point to a state of protumoral tolerance. Conclusion. The orthotopic implantation of NB neurospheres in syngeneic mice has allowed us to generate a NB model in which it has been possible to study the tumor immunity

[Establishment of a preclinical neuroblastoma model in immunocompetent mice]. / Establecimiento de un modelo preclínico de neuroblastoma en ratones inmunocompetentes.

AIM: To develop a NB animal model which makes possible studies related to tumor immunity. MATERIALS AND METHODS: Two types of NB cells were used. Cell line 36769 was derived from TH-MYCN+ mouse in which overexpression of the MYCN gene is governed by rat tyrosine hydroxylase promotor. Cell line 4040 was derived from TH-MYCN/ALK mice, which in addition express an activating mutation of ALK gene. For each cell type, 1x106 neurospheres were implanted in 129/SVJ mice (with the same genetic background as donors, n=8), via orthotopic injection in the left suprarenal gland by intraperitoneal approach, through a transverse supraumbilical laparotomy. Daily postsurgical clinical follow-up of the animals was done until they were sacrificed at four weeks. The tumor presence was macroscopically confirmed. The tumoral sample was excised and was processed for cellular immunity and molecular tolerance mediator's studies. The existence of metastasis was investigated by flow cytometry in the spleen, bone marrow and peripheral blood. RESULTS: 1) Orthotopic Neuroblastoma was generated in all the transplanted mice. 2) The tumors were infiltrated by several immune subpopulations, with effector, regulatory and suppressor inmunophenotype. This was similar to the inmunophenotype described in human NB. Furthermore, the molecular mediators of the environment point to a state of protumoral tolerance. CONCLUSION: The orthotopic implantation of NB neurospheres in syngeneic mice has allowed us to generate a NB model in which it has been possible to study the tumor immunity.

OBJETTIVO: Desarrollar un modelo animal de neuroblastoma (NB) que posibilite estudios relacionados con la inmunidad tumoral. MATERIAL Y METODOS: Se utilizaron dos tipos de células NB. La línea 36769 procedía del ratón TH-MYCN+ en el que la sobreexpresión del gen MYCN está gobernada por el promotor de la tirosín hidroxilasa de rata. La línea 4040 procedía de ratones TH-MYCN+/ALK+, que además expresan una mutación activadora del gen ALK. De cada tipo celular se implantaron 1x106 neurosferas en ratones 129/SVJ (mismo fondo genetico que los donantes, n=8), mediante inyección ortotópica en glándula suprarrenal izquierda por abordaje intraperitoneal, a través de laparotomía transversa supraumbilical. Se realizó seguimiento clínico diario postquirúrgico de los animales hasta su sacrificio a las 4 semanas. La presencia de tumor se confirmó macroscópicamente. La pieza tumoral se extirpó y se procesó para estudios de inmunidad celular y mediadores moleculares de tolerancia. Se investigó la existencia de metástasis por citometría de flujo en bazo, médula ósea y sangre periférica. RESULTADOS: 1) En todos los ratones trasplantados se generó NB ortotópico. 2) La pieza tumoral se encontró infiltrada por diversas subpoblaciones inmunes, con inmunofenotipo efector, regulador y supresor, similar a la situación descrita en los NB humanos. Además, los mediadores moleculares del microambiente apuntan a un estado de tolerancia protumoral. CONCLUSIONES: La implantación ortotópica de neurosferas NB en ratones singénicos nos ha permitido generar un modelo de NB en el que ha sido posible estudiar la inmunidad tumoral.

Landscape of early clinical trials for childhood and adolescence cancer in Spain.

PURPOSE: Despite numerous advances, survival remains dismal for children and adolescents with poor prognosis cancers or those who relapse or are refractory to first line treatment. There is, therefore, a major unmet need for new drugs. Recent advances in the knowledge of molecular tumor biology open the door to more adapted therapies according to individual alterations. Promising results in the adult anticancer drug development have not yet been translated into clinical practice. We report the activity in early pediatric oncology trials in Spain. METHODS: All members of the Spanish Society of Pediatric Hematology Oncology (SEHOP) were contacted to obtain information about early trials open in each center. RESULTS: 22 phase I and II trials were open as of May 2015: 15 for solid tumors (68 %) and 7 for hematological malignancies (32 %). Fourteen (64 %) were industry sponsored. Since 2010, four centers have joined the Innovative Therapies For Children With Cancer, an international consortium whose aim is developing novel therapies for pediatric cancers. A substantial number of studies have opened in these 5 years, improving the portfolio of trials for children. Results of recently closed trials show the contribution of Spanish investigators, the introduction of molecularly targeted agents and their benefits. CONCLUSIONS: Clinical trials are the way to evaluate new drugs, avoiding the use of off-label drugs that carry significant risks. The Spanish pediatric oncology community through the SEHOP is committed to develop and participate in collaborative academic trials, to favor the advancement and optimization of existing therapies in pediatric cancer.

Gliomas de bajo grado: revisión de 10 años / Low-grade gliomas: Review of 10 years

INTRODUCCIÓN: Los tumores del SNC son los tumores sólidos más frecuentes en la edad pediátrica. Dentro de ellos los gliomas de bajo grado constituyen el tipo más común de tumor del SNC en niños, representando hasta el 30-50% de los mismos. PACIENTES Y MÉTODOS: Análisis retrospectivo de las características epidemiológicas, manifestaciones clínicas, localización del tumor, histología, tipo de tratamiento si lo ha recibido, evolución y secuelas a largo plazo de 111 pacientes diagnosticados de glioma de bajo grado en el Hospital Infantil Universitario Niño Jesús de Madrid entre enero de 2002 y diciembre de 2011. RESULTADOS: De los 111 pacientes 57 eran niños y 54 niñas. La edad media fue de 7,26 años (intervalo 2 meses-19 anos). Los síntomas de presentación más frecuentes fueron la cefalea (27%) y los vómitos (19%). Las localizaciones más frecuentes fueron los hemisferios cerebrales (38%), seguido del tronco cerebral (27,4%) y del cerebelo (18,5%). Se realizó estudio histológico en 89 pacientes (80,18%), siendo el astrocitoma pilocítico el tipo histológico más frecuente. Se realizó biopsia diagnóstica en 20 pacientes (22,5%), resección parcial en 38 pacientes (42,7%) y resección total en 31 pacientes (34,8%). Recibieron quimioterapia 16 pacientes (14%) y radioterapia 18 pacientes (16%). La supervivencia global fue del 88,3%. Un paciente presentó secuelas auditivas, 5 pacientes presentaron secuelas visuales y 4 pacientes secuelas endocrinas. CONCLUSIONES: El tipo histológico más frecuente es el astrocitoma pilocítico. La supervivencia global fue del 88,3%. Solo el 9% de los pacientes presentaron algún tipo de secuela auditiva, visual o endocrinológica

INTRODUCTION: Central nervous system (CNS) tumors are the most common solid tumors in children. Among these, the low-grade gliomas are the most common type, accounting for up to 30-50% of them. PATIENTS AND METHODS: A retrospective analysis was carried out on the epidemiology, clinical characteristics, tumor location, histology, treatment, outcome and long-term sequelae of 111 patients diagnosed with low-grade glioma in the Nino˜ Jesús Children's Hospital of Madrid from January 2002 to December 2011. RESULTS: Of the 111 patients, there were 57 boys and 54 girls. The mean age was 7.26 years (range, 2 months - 19 years). The most common symptoms of presentation were headache (27%) and vomiting (19%). The most common locations were the cerebral hemispheres (38%), followed by the brainstem (27.4%), and cerebellum (18.5%). Histological examination was performed in 89 patients (80.18%). Pilocytic astrocytoma was the most common histological type. Diagnostic biopsy was performed in 20 patients (22.5%), partial resection in 38 patients (42.7%), and total resection in 31 patients (34.8%). Sixteen patients received chemotherapy (14%), and eighteen patients received radiotherapy (16%). Overall survival was 88.3%. Long term hearing, visual and endocrine sequelae were note in 1, 5, and 4 patients, respectively. CONCLUSIONS: The most common histological type is pilocytic astrocytoma. Overall survival was 88.3%. Only 9% of patients had some kind or auditory, visual or endocrine sequelae

Coloración anormal del suero asociado al tratamiento con eltrombopag, en paciente con trombopenia inmune / Abnormal coloring of serum associated to treatment with eltrombopag, in patients with immune thrombocytopenia

Se presenta un caso de trombopenia inmune tratado con eltrombopag en el que a las seis semanas se presentó una coloración rojiza en el suero del paciente que impedía la valoración analítica de su función hepática. Dicha coloración persistió hasta la supresión total del fármaco por falta de respuesta terapéutica Se describen experiencias similares en la literatura (AU)

A case of immune thrombocytopenia treated with eltrombopag is presented. At six weeks, the patient had reddish coloring in the serum that prevented analytic evaluation of the hepatic function. This coloring persisted until the total suppression of the drug due to lack of therapeutic response. Similar experiences have been described in the literature (AU)

[Low-grade gliomas: review of 10 years]. / Gliomas de bajo grado: revisión de 10 años.

INTRODUCTION: Central nervous system (CNS) tumors are the most common solid tumors in children. Among these, the low-grade gliomas are the most common type, accounting for up to 30-50% of them. PATIENTS AND METHODS: A retrospective analysis was carried out on the epidemiology, clinical characteristics, tumor location, histology, treatment, outcome and long-term sequelae of 111 patients diagnosed with low-grade glioma in the Niño Jesús Children's Hospital of Madrid from January 2002 to December 2011. RESULTS: Of the 111 patients, there were 57 boys and 54 girls. The mean age was 7.26 years (range, 2 months - 19 years). The most common symptoms of presentation were headache (27%) and vomiting (19%). The most common locations were the cerebral hemispheres (38%), followed by the brainstem (27.4%), and cerebellum (18.5%). Histological examination was performed in 89 patients (80.18%). Pilocytic astrocytoma was the most common histological type. Diagnostic biopsy was performed in 20 patients (22.5%), partial resection in 38 patients (42.7%), and total resection in 31 patients (34.8%). Sixteen patients received chemotherapy (14%), and eighteen patients received radiotherapy (16%). Overall survival was 88.3%. Long term hearing, visual and endocrine sequelae were note in 1, 5, and 4 patients, respectively. CONCLUSIONS: The most common histological type is pilocytic astrocytoma. Overall survival was 88.3%. Only 9% of patients had some kind or auditory, visual or endocrine sequelae.

Transformación de síndrome mielodisplásico a leucemia linfoblástica aguda: 2 casos nuevos / Transformation of myelodysplastic syndrome to acute lymphoblastic leukemia: 2 new cases

Los síndromes mielodisplásicos (SMD) son desórdenes clonales de las células hematopoyéticas, con riesgo variable de transformación a leucemia mieloblástica aguda. La evolución a leucemia linfoblástica aguda (LLA) es extremadamente rara, con muy pocos casos pediátricos. Este artículo describe 2 nuevos casos de SMD que progresaron a LLA, junto a una revisión de la literatura, observando un pronóstico similar a los adultos (AU)

Myelodysplastic syndromes (MDS) are clonal disorders of hematopoietic stem cells, with a variable risk of transformation to acute myeloid leukemia. Progression into acute lymphoblastic leukemia (ALL) is an extremely rare event, with very few cases published in children. In this report, we describe two cases of myelodysplastic syndromes that progressed to ALL. Moreover, we review previously reported cases of MDS transformation to acute lymphoblastic leukemia in the pediatric population whose prognosis seems to be similar to that for adults (AU)

[Transformation of myelodysplastic syndrome to acute lymphoblastic leukemia: 2 new cases]. / Transformación de síndrome mielodisplásico a leucemia linfoblástica aguda: 2 casos nuevos.

Myelodysplastic syndromes (MDS) are clonal disorders of hematopoietic stem cells, with a variable risk of transformation to acute myeloid leukemia. Progression into acute lymphoblastic leukemia (ALL) is an extremely rare event, with very few cases published in children. In this report, we describe two cases of myelodysplastic syndromes that progressed to ALL. Moreover, we review previously reported cases of MDS transformation to acute lymphoblastic leukemia in the pediatric population whose prognosis seems to be similar to that for adults.

Candidiasis hepatoesplénica en pacientes hemato-oncológicos pediátricos / Hepatosplenic candidiasis in paediatric haematology-oncology patients

Introducción: En las últimas décadas, la supervivencia en las enfermedades hemato-oncológicas infantiles ha aumentado gracias a la mejora de los tratamientos, con regímenes quimioterápicos más agresivos. Esto ha conducido a un aumento en la incidencia de infecciones graves, con la consecuente morbimortalidad. La candidiasis hepatoesplénica es una infección diseminada por Candida que suele afectar más frecuentemente al hígado y bazo, aunque pueden verse involucrados otros órganos. Pacientes y métodos: Se ha realizado un estudio retrospectivo de 13 pacientes pediátricos diagnosticados de candidiasis hepatoesplénica desde enero de 2002 a febrero de 2010 en el Servicio de Hemato-Oncología pediátrica del Hospital Niño Jesús de Madrid siguiendo los criterios propuestos por la EORTC/MSG (European Organization for Research and Treatment of Cancer and Mycoses Study Group) revisados en el 2008. Se analizaron las características clínicas, los métodos diagnósticos, el tratamiento realizado y la evolución posterior. Resultados: El síntoma más frecuente de presentación de la candidiasis hepatoesplénica en nuestra serie fue la fiebre persistente, hasta en un 84,6% de los casos, en casi todos, asociada a algún otro síntoma. Los factores de riesgo para desarrollarla son bastante inespecíficos y estaban presentes hasta en un 92,3% de nuestros pacientes. El diagnóstico de infección probada, que se realiza mediante histología o cultivos, normalmente es difícil de obtener y sólo se logró obtener en nuestro caso en un 23,1%. La mayoría de las veces, como en nuestra serie, sólo se llega a un diagnóstico de sospecha. Las pruebas de laboratorio no tienen utilidad en la población pediátrica y tan sólo en un paciente se apreció aumento de la fosfatasa alcalina al diagnóstico. Este resultado difiere del comportamiento de este marcador biológico en los adultos. Aunque suelen ser necesarios tratamientos prolongados para la resolución de las lesiones, la supervivencia es muy alta, en nuestra serie un 100%, con un tratamiento adecuado. Conclusión: La mortalidad de la candidiasis hepatoesplénica es baja si se realiza un tratamiento precoz y adecuado (AU)

Introduction: In the last few decades, the survival of children with haematology-oncological malignancies has increased due to more aggressive chemotherapy regimens. This has led to an increase of fungal infections causing significant morbidity and mortality in these patients. Hepatosplenic candidiasis is a disseminated candida infection that affects most commonly the liver and spleen, although other organs may be involved. Patients and methods: We performed a retrospective study of 13 paediatric patients diagnosed with hepatosplenic candidiasis from January 2002 to February 2010 in our paediatric haematology-oncology department following the criteria proposed by the EORTC/MSG (European Organization for Research and Treatment of Cancer and Mycoses study group) updated in2008. We analysed the clinical characteristics, diagnostic methods, treatment and outcome. Results: The most common symptom of presentation of hepatosplenic candidiasis in our series was persistent fever, up 84.6% of cases, almost all associated with other symptoms. Risk factors for development are non-specific and were present in 92.3% of our patients. The diagnosis of proven infection, which is made by histology or culture, is usually difficult to obtain and in our series it was obtained only in 23.1% of cases. As in our series, diagnosis is often presumptive. Laboratory tests are not useful in the paediatric population and only in one patient we observed an increase in alkaline phosphatase at diagnosis. This result differs from the behaviour of this biological marker in adults. Although prolonged treatment is often necessary for the resolution of the lesions, survival is high with appropriate treatment, 100% in our series. Conclusions: The mortality of hepatosplenic candidiasis is low with early and adequate treatment (AU)