Sección de Oncología y Hematología Pediátricas del Hospital General Universitario Gregorio Marañón / Pediatric Oncology and Hematology Area of the Hospital General Universitario Gregorio Marañón

La Unidad de Oncología y Hematología Infantil del Hospital Gregorio Marañón comenzó su andadura en los años 70, presentando desde entonces un crecimiento progresivo y una modernización acorde con la evolución de la propia especialidad. En esta monografía se describe la organización de la sección, así como los recursos estructurales, las características del trabajo asistencial, la actividad docente e investigadora y la participación en diversos grupos de trabajo colaborativos o multidisciplinares. Se destaca la capacidad de abordaje integral de este tipo de patologías en todas las fases de las mismas, desde el diagnóstico al tratamiento, sin Olvidar el aspecto psicosocial o la atención paliativa en su fase terminal, si fuera necesario. En conjunto, se dibuja un cuadro que es una obra coral de muchos profesionales sanitarios (personal médico, psicooncología, enfermería, auxiliares...) y no sanitarios, pero cuyo tema principal es proporcionar la mejor asistencia posible al niño y a su familia (AU)

The Pediatric Oncology and Hematology Unit at the Gregorio Marañón Children's Hospital began its activity in the 705, presenting since then a progressive growth and modernization in accordance with the evolution of the specialty itself… In this paper we describe the organization of the section, our structural resources, the characteristics of care work, teaching and research activities and our participation in various collaborative or multidisciplinary work groups. It is remarkable the ability to comprehensively address this type of pathologies in its different phases, from diagnosis to treatment, without forgetting to mention the psychosocial aspect or palliative care in its terminal phase, if necessary. Altogether, a choral picture is drawn with the work of many health professionals (medical, psycho-oncology, nursing, assistants …) and non—health, but the main theme is to provide the best care for the child and his family (AU)

Cystosarcoma phyllodes of the breast: a case report in a 12-year-old girl

Breast tumors in adolescents are very rare and mostly benign. Fibroadenomas are the most frequent, but within the extensive differential diagnosis, the phyllodes tumor must be mentioned, which accounts for about 1% of breast tumors and the diagnosis of which is very rare in patients younger than 20 years. There are no specific symptoms or radiological images to distinguish phyllodes tumor from fibroadenoma; therefore, histological examination is mandatory for diagnosis. Histology also allows the classification of phyllodes tumor into benign, borderline, or malignant types for appropriate surgical treatment: freemargin excision in benign tumors and mastectomy in the other two types. Fortunately, the majority of these tumors are benign, and treatment maximizes breast conservation with free infiltration margins surgery, given that this fact is the most important factor to prevent local recurrence. In this article, we describe a rare case of borderline cystosarcoma phyllodes in a 12-year-old girl (AU)

Anomalías hematológicas asociadas al síndrome de Down: síndrome mieloproliferativo transitorio neonatal y leucemia mieloblástica congénita / Hematological abnormalities associated with Down syndrome: transient myeloproliferative disorder and congenital acute myeloblastic leukemia

La asociación entre síndrome de Down y anomalías hematológicas es bien conocida. En el periodo neonatal son frecuentes las alteraciones inespecíficas (neutrofilia, trombocitopenia y policitemia), pero la entidad más importante clínicamente es el síndrome mieloproliferativo transitorio (SMPT), casi exclusivo de recién nacidos con fenotipo Down. Se presentan dos casos de recién nacidos con este fenotipo con hiperleucocitosis, en uno de ellos asociada además a colestasis y lisis tumoral (AU)

It is known that Down syndrome is frequently associated with hematological abnormalities. In newborn infants, it is not unusual to find nonspecific features (neutrophilia, thrombocytopenia, polycythemia),but the entity of the greatest clinical importance is transient myeloproliferative disorder (TMD), which is observed almost exclusively in neonates with Down syndrome. Two newborns with Down syndrome are presented. They were studied forhyperleukocytosis, in one case, associated with cholestasis and tumor lysis síndrome (AU)

[Thrombocytosis in the oncology-haematology clinic: description, aetiological diagnosis and progression thrombocytosis]. / Trombocitosis en la consulta de oncohematología. Descripción, diagnóstico etiológico y evolución.

INTRODUCTION: Thrombocytosis is a common cause of patient referral to a paediatric haematologist specialist which requires a significant number of laboratory tests and visits to confirm the diagnosis. The aim of our study has been to analyse the characteristics of patients referred to our centre for specialised thrombocytosis assessment. Based on this assessment we established the criteria patients must fulfil to be recommend for further hospital study. PATIENTS AND METHODS: We categorised the 33 patients referred for thrombocytosis assessment according to sex, age, origin, personal and family history, platelet count at diagnosis and the reason why the red and white blood count at diagnosis (Haemoglobin, mean corpuscular volume, mean corpuscular haemoglobin, leukocyte, neutrophils, lymphocyte and monocyte count), maximum platelet count during follow-up and other complementary examinations were done. The final diagnosis itself and number of previous visits before were also considered. The classification used to grade thrombocytosis was: low (500-700 X 10(3)/microl), mild (700-900 x 10(3)/microl), severe (900-1.000 x 10(3)/microl) and extreme (> 1.000 x 10(3)/microl). RESULTS: There was no predominance of males or females. 45 % of patients were under 2 years old and 55 % of them came from their primary care centre. The mean platelet count at the first medical visit was 669,000 (mild thrombocytosis). During follow-up, 24 % of the patients reached extreme platelets levels. In 28 % the initial blood count was performed because of an infection. The most frequently requested laboratory test was iron metabolism (82 % of the cases). All cases correspond to secondary thrombocytosis (48 % were reactive to infections, 24 % secondary to iron deficiency, and 15 % were associated to both causes). The mean number of visits before hospital discharge was 5.12. CONCLUSIONS: The finding of thrombocytosis in the majority of the cases studied was casual or in the context of an infectious process. Most of the thrombocytosis were mild. Due to the extremely low incidence of primary thrombocytosis in childhood and the fact that diagnosis is made by exclusion of other possibilities, the initial study of these patients should be done in primary care centres. The first conditions to be ruled out are infectious, inflammatory or bleeding processes. Once these causes are excluded, the most useful complementary test is to measure iron level given the relation between iron deficiency and thrombocytosis. Once these causes are ruled out and thrombocytosis persists, it would then be indicated to refer the patient to a paediatric oncology-haematology department for a more exhaustive follow-up.

Linfoma de Burkitt: el tumor pediátrico más frecuente en África / Burkitt’s lymphoma. The most common paediatric tumour in Africa

El linfoma de Burkitt supone casi un 40% de los linfomas no hodgkinianos en la infancia. Se reconocen tres variantes clínicas: asociado a inmunodeficiencia, esporádico y endémico, propio del África ecuatorial. Éste es el tumor pediátrico más frecuente en África ecuatorial, en cuya etiopatogenia actúan como cofactores la infección por el virus de Epstein-Barr y la malaria. Presenta diferencias clínicas y moleculares con los casos esporádicos, como la característica afectación de los huesos de la cara (principalmente de la mandíbula, seguida de la órbita), la mayor frecuencia de afectación del sistema nervioso central, de las glándulas salivales y del tiroides, y la menor frecuencia de infiltración de la médula ósea. El tratamiento se basa en quimioterapia combinada, y en el tratamiento inicial es crucial la prevención del síndrome de lisis tumoral. Los resultados del linfoma de Burkitt esporádico y endémico son superponibles, respecto a las tasas de respuesta, recaídas y supervivencia(AU)

Burkitt’s lymphoma accounts for nearly 40% of the non-Hodgkin’s lymphomas in childhood. There are three recognized clinical variants of Burkitt’s lymphoma: Burkitt’s lymphoma associated with immunodeficiency, sporadic Burkitt’s lymphoma and endemic Burkitt’s lymphoma, from Equatorial Africa. This is the paediatric tumour most frequent in Equatorial Africa, in the etiopathogeny of which the infection caused by the Epstein Barr virus and malaria act as co-factors. It shows clinical and molecular differences compared to the sporadic cases, such as: the characteristic involvement of the bones of the face, mainly the jaw followed by the eye socket; the greater frequency of the involvement of the central nervous system, of the salivary glands and of the thyroid glands; and the lesser frequency of the infiltration of the bone marrow. The treatment is based on combined chemotherapy, with the prevention of the tumour lysis syndrome being crucial in the initial treatment. The results of the sporadic and endemic Burkitt’s lymphoma are superimposable with regard to the rates of response, relapse and survival rates(AU)

Trombocitosis en la consulta de oncohematología. Descripción, diagnóstico etiológico y evolución / Thrombocytosis in the oncology-haematology clinic: Description, aetiological diagnosis and outcome

Introducción: La trombocitosis es un motivo frecuente de consulta en oncohematología infantil, y precisa un importante número de visitas y determinaciones analíticas para su diagnóstico o resolución. Nuestro objetivo ha sido evaluar las características de los pacientes derivados a nuestro servicio en los últimos meses para estudio de trombocitosis y establecer cuáles deberían ser los pacientes que precisan un estudio más exhaustivo en el hospital. Pacientes y métodos: Se determina en 33 pacientes derivados por este motivo el sexo, el rango de edad, la procedencia, los antecedentes personales y familiares, el grado de trombocitosis por la que se consulta y la cifra máxima durante el seguimiento, el motivo por el que se realiza la primera analítica, los valores hematimétricos en la primera analítica (hemoglobina [Hb], volumen corpuscular medio [VCM], hemoglobina corpuscular media [HCM], leucocitos [linfocitos y neutrófilos]), las exploraciones complementarias realizadas, el diagnóstico y el número de visitas que precisaron antes del alta. Se clasifica la trombocitosis en leve (500-700 × 103/μl), moderada (700-900 × 103/μl), grave (900-1.000 × 103/μl) y extrema (> 1.000 × 103/μl). Resultados: No hubo predominancia de sexos. El 45 % de los pacientes eran menores de 2 años. Procedían en un 55 % de su centro de salud. La cifra media de plaquetas por la que consultaron fue de 669.000 (trombocitosis leve). En el seguimiento llegaron a cifras extremas el 24 %. En el 28 % la analítica se había realizado por un cuadro infeccioso. La exploración complementaria más solicitada fue el metabolismo del hierro (en el 82 %). Todos se corresponden con trombocitosis secundarias (el 48 % reactivas a infecciones, el 24 % secundarias a ferropenia y el 15 % por ambas causas). El número medio de visitas ha sido de 5,12. Conclusiones: El hallazgo de la trombocitosis es en la mayoría de los casos casual o en el contexto de un cuadro infeccioso y, además, son leves. Dada la baja incidencia de trombocitosis primaria en la infancia y que el diagnóstico es de exclusión, se debería iniciar el estudio de la misma en la consulta de atención primaria, descartando inicialmente una causa infecciosa, inflamatoria o secundaria a sangrado. Una vez descartadas estas causas la prueba complementaria más rentable es el metabolismo del hierro, dada la asociación de ferropenia con trombocitosis. Si también se excluye esta etiología y se comprueba la persistencia de la trombocitosis, estaría indicado derivar al paciente a un servicio de oncohematología infantil para completar estudio (AU)

Introduction: Thrombocytosis is a common cause of patient referral to a paediatric haematologist specialist which requires a significant number of laboratory tests and visits to confirm the diagnosis. The aim of our study has been to analyse the characteristics of patients referred to our centre for specialised thrombocytosis assessment. Based on this assessment we established the criteria patients must fulfil to be recommend for further hospital study. Patients and methods: We categorised the 33 patients referred for thrombocytosis assessment according to sex, age, origin, personal and family history, platelet count at diagnosis and the reason why the red and white blood count at diagnosis (Haemoglobin, mean corpuscular volume, meen corpuscular haemoglobin, leukocyte, neutrophils, lymphocyte and monocyte count), maximum platelet count during follow-up and other complementary examinations were done. The final diagnosis itself and number of previous visits before were also considered. The classification used to grade thrombocytosis was: low (500-700 × 103/μl), mild (700-900 × 103/μl), severe (900-1.000 × 103/μl) and extreme (> 1.000 × 103/μl). Results: There was no predominance of males or females. 45 % of patients were under 2 years old and 55 % of them came from their primary care centre. The mean platelet count at the first medical visit was 669,000 (mild thrombocytosis). During follow-up, 24 % of the patients reached extreme platelets levels. In 28 % the initial blood count was performed because of an infection. The most frequently requested laboratory test was iron metabolism (82 % of the cases). All cases correspond to secondary thrombocytosis (48 % were reactive to infections, 24 % secondary to iron deficiency, and 15 % were associated to both causes). The mean number of visits before hospital discharge was 5.12. Conclusions: The finding of thrombocytosis in the majority of the cases studied was casual or in the context of an infectious process. Most of the thrombocytosis were mild. Due to the extremely low incidence of primary thrombocytosis in childhood and the fact that diagnosis is made by exclusion of other possibilities, the initial study of these patients should be done in primary care centres. The first conditions to be ruled out are infectious, inflammatory or bleeding processes. Once these causes are excluded, the most useful complementary test is to measure iron level given the relation between iron deficiency and thrombocytosis. Once these causes are ruled out and thrombocytosis persists, it would then be indicated to refer the patient to a paediatric oncology-haematology department for a more exhaustive follow-up (AU)

Síndrome de esplenomegalia tropical en el diagnóstico diferencial de esplenomegalia masiva / Tropical splenomegaly syndrome in the differential diagnosis of massive splenomegaly

Presentamos el caso de un niño de 11 años de edad, de origen guineano, enviado a nuestro hospital por presentar esplenomegalia masiva, anemia, eosinofilia y fiebre intermitente. El estudio etiológico inicial no fue concluyente. Finalmente, se estableció el diagnóstico de síndrome de esplenomegalia por hiperreactividad a la malaria, o síndrome de esplenomegalia tropical, para lo cual fueron esenciales la exclusión de otras causas y la respuesta al tratamiento antipalúdico; la biopsia hepática fue característica del cuadro

We report the case of an 11-year-old boy from Equatorial Guinea, referred to our hospital with massive splenomegaly, anemia, eosinophilia and intermittent fever. The preliminary etiological study was not conclusive. Finally, a diagnosis of splenomegaly syndrome due to hyperreactivity to malaria or “tropical splenomegaly syndrome” was established. The exclusion of other causes and the response to antimalarial treatment provided the key to the diagnosis, and liver biopsy revealed the pattern typically associated with this condition

Edema hemorrágico agudo del lactante; una entidad poco conocida / No disponible

El edema hemorrágico agudo del lactante (EHAL) es una rara vasculitis leucocitoclástica que afecta a lactantes y niños hasta los 3 años de edad. Cursa con un cuadro clínico caracterizado por la rápida aparición de una extensa púrpura palpable, pero su pronóstico generalmente es muy bueno. Presentamos el caso de un lactante con EHAL y comentamos las similitudes y las diferencias que guarda este cuadro con la púrpura de Schonlein-Henoch (AU)

The acute hemorrhagic edema of infancy (AHEI) is an unusual variant of leukocitoclastic vasculitis affecting infants and children up to three years of age. Clinically it is characterised by a large palpable purpura with a dramatic appearance but an usually benign course. We present an infant with AHEI and compare this disorder with the Schonlein-Henoch purpura (AU)

Validation of the Spanish version of the Phase III ISAAC questionnaire on asthma.

The prevalence of allergic diseases in childhood has increased significantly over the last decades. This increase seems to be closely associated with the way of life of western societies. The high prevalence differences on different regions may be due to linguistic and cultural reasons and not to real variations in prevalence. This is the reason why several authors felt the need to perform an objective validation of their versions. Our working group has published the results of the Phase I validation and now is publishing the Phase III validation in order to guarantee the reliability of this phase results. The study sample is formed by 366 children aged 3 to 17 years. The following steps were followed in this study: I. Assessment of the "Criterion validity" of the Spanish ISAAC-Bronchial Asthma questionnaire, evaluating the sensitivity, specificity, relative value, and positive and negative predictive values. 2. Determine the questionnaire reliability, analysing its "Inner consistency". 3. Statistical comparison between our ISAAC-Bronchial Asthma results and the ones obtained by other groups (external concordance and consistency), in order to prove the previously evaluated reliability. 4. Comparison between the ISAAC-Bronchial asthma questionnaire diagnostic ability and the standard diagnostic criteria universally used in clinical praxis. We could confirm that there is a high and very significant concordance between the questions aimed to detect children with asthma. In this sense, it is especially useful the question about "ever had wheezing" because of its high sensitivity (93.3%) and specificity (89.9%), that make it able to be used as initial screening test in a general population, and that has shown a high concordance percentage with the questions "ever had asthma" (98%), "wheezing with exercise" (75%), and "cough at night"(80%). The questions that give more information about the evolution and control of the asthmatic disease are "wheezing in the last 12 months", "number of attacks in the last 12 months", "wakening at night", "wheezing with exercise" and "dry cough at night in the last 12 months". The questions more related to asthma severity were "number of attacks in the last 12 months", "wakening at night", "stop speaking in order to breath", and "wheezing with exercise". We conclude that ISAAC-Asthma questionnaire Phase III is a useful tool for the assessment of childhood asthma due to its criterion validity, inner consistency and external concordance.