ABSTRACT
Classically, several variables have been related to the disease course of chronic primary immune thrombocytopenia (cITP), though to date, there is no consensus on their clinical relevance. In a recent systematic review, a meta-analysis was made and confirmed the existence of certain cITP-related variables that may be related to prognosis in pediatric patients. We retrospectively analyzed a cohort of patients diagnosed with ITP, identified prognostic variables, and compared our results to the variables described by the authors. A multivariate study revealed that older age at diagnosis and higher platelet count were the only independent variables related to cITP. Children up to age 4 years and those with lower platelet counts (below 20 × 109/L) were at lower risk for cITP.Conclusion: We therefore concluded that only age and platelet count at diagnosis are independent variables that should be considered when evaluating the risk of developing cITP. What is Known: ⢠Around 20% of patients with immune thrombocytopenia progress to chronic disease as determined by a sustained platelet count below 100×109/L for more than 12 months. ⢠A number of variables potentially related to the development of cITP are being studied, such as age, sex, cell count, and previous treatment. What is New: ⢠This is a new group of patients diagnosed with ITP in which the platelet count and age at diagnosis are the only independent variables closely related to cITP. ⢠In this new series, we could not confirm other variables previously related to cITP such as total leukocyte count or the absence of treatment at diagnosis.
Subject(s)
Purpura, Thrombocytopenic, Idiopathic , Thrombocytopenia , Aged , Child , Child, Preschool , Chronic Disease , Humans , Platelet Count , Purpura, Thrombocytopenic, Idiopathic/diagnosis , Purpura, Thrombocytopenic, Idiopathic/epidemiology , Retrospective StudiesABSTRACT
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Subject(s)
Humans , Hematopoietic Cell Growth Factors/therapeutic use , Myelodysplastic Syndromes/drug therapy , Risk FactorsABSTRACT
Several genetic diseases, generally considered as congenital diseases, are characterized by bone marrow failure during early childhood. Hematopoietic stem cell transplantation is the only curative treatment for syndromes involving bone marrow failure and thalassemia. In this slate-of-the-art review, we wish to focus on the results of hematopoietic transplantation in treating some of these diseases, with a special emphasis on congenital bone marrow failure and thalassemia. The results of this procedure have improved over the previous years, mainly when performed by experienced teams. New conditioning regimes based on fludarabine and the use of HLA-identical donors have been related with better survivals. In the previous years, donors other than HLA-identical siblings have been increasingly used in patients not responding to conventional measures, but this approach needs to be evaluated in larger studies.
Subject(s)
Bone Marrow Diseases/therapy , Hematopoietic Stem Cell Transplantation , Thalassemia/therapy , Bone Marrow Diseases/congenital , Child , Child, Preschool , Female , Histocompatibility Testing/methods , Humans , Male , Thalassemia/genetics , Tissue Donors , Transplantation Conditioning/methods , Transplantation, HomologousABSTRACT
BACKGROUND: Children with Down syndrome (DS) have a higher risk of acute leukemia than the remaining pediatric population. A favorable outcome of acute myeloid leukemia (AML) has recently been described in these patients whereas the prognosis of acute lymphoblastic leukemias (ALL) is similar to that in other children. The main cause of morbidity and mortality in children with Down syndrome are complications related to chemotherapy, leading to numerous modifications in treatment protocols. OBJECTIVES: To characterize acute leukemias in children with Down syndrome in our center and determine their clinical outcome. METHODS AND RESULTS: Between 1990 and 2002, 214 children were diagnosed with acute leukemia at the Niño Jesus Hospital (40 with AML and 174 with ALL). Of these, eight children (3.8 %) had Down syndrome. AML (2/40) represented 5 % of myeloid leukemias and ALL (6/174) represented 3.4 % of lymphoblastic leukemias. The most frequent complication was hematologic toxicity due to chemotherapy, causing a high incidence of infections: pneumonia (5/8) and bacteriemia (5/8). In all patients, these complications led to treatment interruption or dose reduction. Two children died from treatment-related toxicity. Of these, one with AML developed fulminant sepsis due to Candida infection and the other, diagnosed with high risk ALL, died from multiorgan failure after high doses of methotrexate and ARA-C. CONCLUSIONS: Patients with Down syndrome diagnosed with acute leukemia show a higher incidence of treatment-related complications, which affects their prognosis. Consequently, individualized treatment of these children in qualified units is essential.
Subject(s)
Down Syndrome/complications , Leukemia, Megakaryoblastic, Acute/complications , Precursor Cell Lymphoblastic Leukemia-Lymphoma/complications , Child , Child, Preschool , Female , Humans , Infant , Leukemia, Megakaryoblastic, Acute/mortality , Leukemia, Megakaryoblastic, Acute/therapy , Male , Precursor Cell Lymphoblastic Leukemia-Lymphoma/mortality , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapyABSTRACT
Antecedentes: Los niños con síndrome de Down tienen mayor riesgo de leucemia aguda que el resto de la población infantil. Recientemente se ha descrito una evolución favorable de las leucemias mieloides agudas (LMA) en estos niños, mientras que las leucemias linfoblásticas agudas (LLA) tienen un pronóstico similar al del resto. Las complicaciones atribuidas a la quimioterapia son la principal causa de morbi-mortalidad, lo que ocasiona modificaciones de los protocolos de tratamiento. Objetivos: Caracterizar las leucemias agudas en niños con síndrome de Down en nuestro centro y la evolución clínica de éstos. Métodos y resultados: Entre 1990 y 2002 se han diagnosticado 214 leucemia aguda en el Hospital Niño Jesús (40 LMA y 174 LLA). De ellos, 8 casos (3,8 por ciento) eran pacientes con síndrome de Down. Las LMA 2/40 representaron el 5 por ciento del total mientras que las LLA un 6/174 el 3,4 por ciento. La toxicidad hematológica por el tratamiento fue la complicación más habitual, condicionando una elevada frecuencia de procesos infecciosos: neumonías (5/8) y bacteriemias (5/8). Además, las complicaciones obligaron a interrumpir y/o reducir las dosis del tratamiento en todos los pacientes. Dos niñas fallecieron como consecuencia de la toxicidad del tratamiento. Una diagnosticada de LMA desarrolló una sepsis fulminante por Candida, y la otra, diagnosticada de LLA de alto riesgo, falleció por fallo multiorgánico tras recibir altas dosis de metotrexato y arabinósido de citocina (Ara-c).Conclusiones Los pacientes con síndrome de Down diagnosticados de leucemia aguda presentan una mayor incidencia de complicaciones relacionadas con el tratamiento lo que condiciona de gran modo su pronóstico. Por ello, es importante un tratamiento individualizado de estos niños en unidades cualificadas (AU)
Subject(s)
Humans , Infant , Male , Child, Preschool , Child , Female , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Leukemia, Megakaryoblastic, Acute , Down SyndromeABSTRACT
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Subject(s)
Female , Male , Child , Humans , Combined Modality Therapy/classification , Combined Modality Therapy/methods , Combined Modality Therapy , Radiotherapy/methods , Brain Neoplasms/diagnosis , Brain Neoplasms/therapy , Neoplasms/diagnosis , Neoplasms/therapy , Neoplasms/epidemiology , Brain Neoplasms/surgery , Brain Neoplasms/physiopathology , Brain Neoplasms/pathologyABSTRACT
La listeriosis, infección sistémica causada por Listeria monocytogenes, es una enfermedad poco frecuente. Se ha observado que la inmunosupresión celular predispone a sufrir esta infección con mayor frecuencia, siendo ejemplo de este tipo de afectación la producida en los pacientes sometidos a tratamiento quimioterápico. Por este motivo se ha observado un aumento de su frecuencia en los últimos años. Presentamos el caso de una niña diagnosticada de leucemia linfoblástica aguda, que desarrolló una sepsis y meningitis por Listeria tratada con ampicilina y gentamicina, con evolución favorable (AU)
