Pulmonary function of children with acute leukemia in maintenance phase of chemotherapy / Função pulmonar de crianças com leucemia aguda na fase de manutenção da quimioterapia

OBJECTIVE: The aim of this study was to assess the pulmonary function of children with acute leukemia. METHODS: Cross-sectional observational analytical study that enrolled 34 children divided into groups A (17 with acute leukemia in the maintenance phase of chemotherapy) and B (17 healthy children). The groups were matched for sex, age and height. Spirometry was measured using a spirometer Microloop Viasys(r) in accordance with American Thoracic Society and European Respiratory Society guidelines. Maximal respiratory pressures were measured with an MVD300 digital manometer (Globalmed(r)). Maximal inspiratory pressures and maximal expiratory pressures were measured from residual volume and total lung capacity, respectively. RESULTS: Group A showed a significant decrease in maximal inspiratory pressures when compared to group B. No significant difference was found between the spirometric values of the two groups, nor was there any difference between maximal inspiratory pressure and maximal expiratory pressure values in group A compared to the lower limit values proposed as reference. CONCLUSION: Children with acute leukemia, myeloid or lymphoid, during the maintenance phase of chemotherapy exhibited unchanged spirometric variables and maximal expiratory pressure; However, there was a decrease in inspiratory muscle strength...

OBJETIVO: O objetivo desse estudo foi avaliar a função pulmonar de crianças com leucemia aguda. MÉTODOS: Trata-se de um estudo observacional do tipo analítico transversal com 34 crianças, divididas nos grupos A (17 crianças com leucemia aguda na fase de manutenção do tratamento quimioterápico) e B (17 crianças saudáveis). Os grupos foram pareados em relação ao sexo, idade e altura. A espirometria foi mensurada utilizando um espirômetro Microloop Viasys(r), de acordo com as recomendações da American Thoracic Society e European Respiratory Society. As pressões respiratórias máximas foram mensuradas utilizando um manovacuômetro digital MVD300 (Globalmed(r)). As pressões inspiratória máxima e expiratória máxima foram mensuradas a partir do volume residual e da capacidade pulmonar total, respectivamente. RESULTADOS: O grupo A apresentou diminuição significativa da pressão inspiratória máxima quando comparado ao grupo B. Não foram observadas diferenças entre os dados espirométricos dos dois grupos avaliados, bem como entre os valores de pressão inspiratória máxima e pressão expiratória máxima do grupo A com os limites inferiores propostos como referência. CONCLUSÃO: As crianças com leucemia aguda, linfoide ou mieloide não apresentam mudança das variáveis espirométricas e da pressão expiratória máxima durante o período de manutenção do tratamento quimioterápico; no entanto, há uma diminuição da pressão inspiratória máxima...

Leucemia congénita / Congenital leukemia

La leucemia congénita constituye una entidad rara que se diagnostica entre el momento del nacimiento y los primeros 30 días de vida. Menos del 1 por ciento de las leucemias de la infancia se diagnostican en el neonato. Se caracteriza por la presencia en un recién nacido, de hepatomegalia, esplenomegalia y lesiones hemorrágicas o infiltrativas en piel. El hemograma completo, el examen de la lámina de la sangre periférica, el aspirado de médula ósea, junto con el inmunofenotipaje y los estudios de biología molecular confirmarán el diagnóstico. El trastorno mieloproliferativo transitorio y las infecciones congénitas constituyen los principales diagnósticos diferenciales. El pronóstico de la enfermedad es sombrío. El tratamiento está basado en regímenes de poliquimioterapia intensiva y el trasplante de progenitores hematopoyéticos constituye una opción terapéutica en un grupo de pacientes

Congenital leukemia is a rare but well documented disease which can be diagnosed on the first 30 days of life. The diagnosis requires a high index of clinical suspicion in a newborn with hepatosplenomegaly and hemorragic or infiltratives cutaneous lesions (red, brown or purple papules or nodules) along with further investigations such as complete blood count, peripheral blood smear, bone marrow aspirate and immunopfenotypic profile. A true congenital leukemia has to be distinguished from other conditions such as a transient mieloproliferative disorder described in Down´s syndrome and congenital infections. The prognosis of neonatal leukemia is dismal. Treatment includes intensive multi-agent chemotherapy and supportive care, being the hematopoietic stem cell transplantation an additional treatment option in a group of patients

Acute Appendicitis in Children with Acute Leukemia: Experiences of a Single Institution in Korea

PURPOSE: Acute appendicitis has been reported to be relatively rare in pediatric leukemia patients but there is no official data for this in Korea. And there is no consensus for its treatment in this population. MATERIALS AND METHODS: We conducted a retrospective study of 7 patients diagnosed with appendicitis among 1209 pediatric patients who were diagnosed with leukemia from 1996 to 2008 at a single institution in Korea. RESULTS: The median age at the time of the diagnosis of appendicitis was 12 years (range: 3-15 years), and 3 of the patients were male. The median absolute neutrophil count (ANC) at the time of diagnosis was 0.99x10(9)/L (range: 0-3x10(9)/L). The mean time from the onset of symptoms to the diagnosis was 4 days. All 7 leukemia patients with appendicitis underwent surgery and they demonstrated a survival of 100% without significant complications. CONCLUSION: The incidence of appendicitis in pediatric leukemia patients was 0.57% in our study. Early diagnosis with abdominal ultrasound or computed tomography and early surgical resection in leukemic patient with acute appendicitis may be a safer and more effective treatment option. Even when perforation has already occurred and when the patient has an ANC of 0x10(9)/L, surgical treatment may improve overall survival without incurring significant complications.

Leucemias agudas / Acute leukemias

las leucemias agudas son un conjunto heterogéneo de neoplasia hematológica de origen multifactorial, causada por proliferaciones descontroladas de la célula hematopoyéticas inmaduras que lleva a una deficiente producción de células sanguíneas normales, con el progresivo deterioro del estado general de los pacientes, que si no son tratados a tiempo fallecen en pocas semanas o meses. El mecanismo molecular y genético que desencadena la transformación neoplásica de las células hematopoyéticas, se conoce en profundidad y han sido descritas diferentes variedades en base al origen celular de los blastos. Así mismo existen nuevas y mejores herramientas de diagnóstico, que permiten establecer con precisión el subtipo de leucemia aguda en cada caso, así como el pronóstico de la enfermedad. Estos avances han aumentado considerablemente la tasa de curaciones, especialmente en los niños. No obstante, aun queda mucho por hacer en el campo de la terapéutica. Las drogas actualmente disponibles para la quimioterapia, no consiguen erradicar completamente el clon leucémico y poseen muchísimos efectos colaterales. Hoy día, la leucemia aguda, continua siendo una enfermedad temible y potencialmente mortal. Un análisis profundo de las situaciones de los pacientes leucémicos en nuestro país, y los factores que impiden diagnosticar y tratar adecuadamente a estos pacientes, debería llevarnos a idear nuevas estrategias que nos permitan ofrecer a estos pacientes todas las alternativas diagnósticas y terapéuticas actualmente disponibles.

Leucemia Eosinofilica: reporte de un caso clínico / Eosinophilic leukemia: on pourpose of a case

Se describe un caso de leucemia eosinofilica en niñade 11 años sin alteraciones croosómicas, en fase de crisis blástica. Con cuadro clínico de compromiso gastrointestinal cutáneo, asociado a 21216 eosinófilos/mL en sangre periférica en un infiltrado del 60porciento en médula ósea.

Leucemia y síndrome de Down. Presentación de un caso / Down's syndrome and leukemia

El síndrome de Down se acompaña con cierta frecuencia de leucemia mieloblastica. En este informe se hace notar la diferencia entre la leucemia verdadera y la presencia de un síndrome mielodisplásico. Se reporta un caso clínico

Value of some tumor markers in CNS infiltration of childhood leukemia and lymphoma

Some tumor markers were evaluated in serum and CSF of thirty-one children with acute lymphoblastic leukemia [ALL] and non-Hodgkin's lymphoma [NHL]. Ten of the patients had clinical sings of central nervous system [CNS] infiltration. Significantly lower levels of carcinoembryonic antigen [CEA]; beta-2-microglobulin [beta-2-MG], beta- glucuronidase lysozyme and immunoglobulins IgG, IgM and IgA were detected in serum and CSF of patients compared with pretherapy levels. Children with clinical evidences of CNS infiltration [32.3%] showed higher level of all studied markers in CSF compared with those without infiltration, but the differences were significant only in beta-2-MG, lysozyme and immunoglobulins. In 29% of patients without clinical evidences of CNS involvement, high levels of these markers in CSF were detected which may indicate early infiltration. Patients with relapses [14.2%] exhibited significant increase of CSF markers. The levels were higher than pretherapy levels. It was concluded that determination of CEA, beta-2-MG, beta- glucuronidase, lysozyme and immunoglobulins in CSF and serum of patients with ALL and NHL are valuable tools in early detection of relapses

CSF cytokines, nitric oxide and prostaglandins in diagnosis and pathophysiology of childhood CNS leukemia and lymphoma

To clarify the role of cytokines, nitric oxide [NO] and prostaglandin- E2 [PG-E2] in diagnosis and pathogenesis of infiltration of central nervous system [CNS] in cases with leukemia and lymphoma, the levels of these indices in the cerebrospinal fluid [CSF] of those children were determined. Their levels were correlated with other laboratory studies of CSF and clinical criteria. This study included twenty- seven children with acute leukemia [twenty with acute lymphoblastic leukemia [ALL] and seven with acute myeloid leukemia [AML]] and fifteen with non- Hodgkin's lymphoma [NHL]. Clinical evidence of CNS involvement was present in seventeen patients with acute leukemia and seven cases with NHL. The study revealed significantly elevated levels of two types of cytokines [TNF-alpha, IL-6] NO and PG-E2 in CSF of children with acute leukemias and lymphoma compared with control group [children with tension headache or meningism]

Serum atrial natriuretic peptide, lipid profile and lipid peroxidation in adriamycin therapy of leukemia and lymphoma

Serum atrial natriuretic peptide [ANP], triglycerides, cholesterol and lipid peroxides were studied in thirty-one cases of acute lymphoblastic leukemia [ALL] [including fifteen newly diagnosed cases and sixteen relapses cases], twenty-nine cases of non-Hodgkin's lymphoma [NHL] comprised fifteen newly diagnosed cases and fourteen relapsed cases, ten oncogenic controls and ten healthy controls. Adriamycin was included in therapy of studied cases and not in that of oncogenic controls. Beside full clinical examination and routine investigations to diagnose ALL and NHL cases, ECG and echocardiography were done to all cases and controls. Serum ANP, triglyceride, cholesterol and lipid peroxides were measured before adriamycin therapy, three weeks after the first remission, three weeks after the second remission and in serum of the control group. It was concluded that serum ANP in patients treated with adriamycin is helpful diagnostic tool for early detection of myocardial damage and early management even in the absence of echocardiographic changes as the rise in ANP level is related to the drug dose and not related to the malignancy itself. Serial determinations of serum triglyceride, cholesterol and lipid peroxides are helpful for early detection and prevention of myocardial damage

Leucemia Congénita, Presentación de Dos Casos y revisión de la Literatura / Congenital Leukemia. Report of Two Cases and Review of the Literature

La leucemia congénita es una enfermedad bastante rara en la práctica clínica con un curso rápido y fatal. Sus características clínicas y biológicas difieren de las encontradas en niños de mayor edad. Se han observado casos tanto de tipo mieloide como linfoide,pero la leucemia mieloide aguda parece predominar. Se presentan dos casos de leucemia congénita clasificados morgológicamente; el primero correspondiente a una leucemia mieloide aguda M3 y el segundo a una leucemia linfoide aguda L1. En ambos pacientes se destacaron hallazgos como hepatoesplenomegalia, palidez mucocutánea generalizada y leucocitosis (más de 100x10 9/L). Los estudios citogenéticos no revelaron ninguna anomalía cromosómica esturctural, pero en el caso 1 se encontró hiperdiploidía

Epidemiología de los padecimientos oncohematológicos por mortalidad hospitalaria en Sinaloa / Epidemiology in oncohematologic diseases based upon hospital mortality in Sinaloa

Se revisaron 25,449 muertes hospitalarias en base a certificados de defunción, ocurridas en la Delegación Estatal del Instituto Mexicano del Seguro Social en Sinaloa, de 1980 a 1991. Por cáncer fallecieron 3.115 (12.2 por ciento): del aparato digestivo 3.2 por ciento, respiratorio 2.6 por ciento, genitourinario 2.2 por ciento y hematológico 2.0 por ciento. De las 510 defunciones por padecimientos oncohematológicos, el 62.5 por ciento correspondió a las leucemias, destacando la linfoide aguda, cuya presentación fue más frecuente en el grupo de 5 a 14 años. Los casos de leucemian mieloide aguda fueron la mitad de los de linfoide, igual en uno y otro sexo y en todos los grupos de edad. En cuanto a las leucemias crónicas, la mieloide es dos veces más frecuente que la linfoide, siendo ambos padecimientos de adultos mayores de edad. La leucemia minocítica, la linfocítica crónica y la eritroleucemia son padecimientos poco frecuentes. Los linfomas significaron la quinta parte del total de las defunciones y su tendencia es presentarse en los grupos de mayor edad. La enfermedad de Hodgkin fue un padecimiento encontrado en niños y jóvenes, con una proporción por sexos de tres a uno a favor del masculino. El mieloma múltile predomina en los dos grupos de mayor edad, sin diferencias por sexo. En la mortalidad se ha observado un decremento hasta del 60 por ciento en los padecimientos oncohematológicos, principalmente leucemias y linfomas, probablemente debido a detecciones más oportunas y programas terapéuticos más intensivos, siendo estable por cáncer en general. Se comparan estos hallazgos con otros informes y se elaboran recomendaciones para la programación de actividades asistenciales, educativas y de investigación

Manifestaciones sistémicas de las enfermedades hematológicas: parte I / Systemic manifestations of the hematological diseases: part I

Desde un punto de vista fisiopatológico es bien reconocido el rol primordial que cumplen los diferentes componentes celulares de la sangre y los factores plasmáticos de la coagulación en la mantención de las funciones orgánicas normales. Ello implica que fallas en estos sistemas provocarán efectos nocivos, en forma difusa, en diversos territorios. En el niño, en el que los procesos de crecimiento y desarrollo exigen una continua y rápida interrelación de los procesos fisiológicos, la existencia de trastornos de ésta índole adquiere un particular relieve