Linfoma de Burkitt Primário de Mama: Relato de Caso / Primary Breast Burkitt Lymphoma: Case Report / Linfoma de Burkitt Primario de Mama: Relato de Caso

Introdução: O linfoma primário de mama (LPM) representa cerca de 0,5% das neoplasias mamárias, sendo considerado um tipo raro de tumor. Alguns dos tipos de LPM, por sua vez, possuem ligação intensa com o período gravídico e pós-parto em virtude do estímulo hormonal. O objetivo deste estudo é relatar um caso de LPM com apresentação rara de linfoma de Burkitt, considerando propostas terapêuticas eficazes para o seguimento. Relato do caso: Paciente do sexo feminino, 23 anos, portadora de tumoração periareolar em mama direita com aspecto de casca de laranja e crescimento rápido há um mês, queixas flogísticas no local da lesão, bom estado geral e sem outros sintomas associados. O quadro relatado iniciou-se sete meses após a expulsão fetal com feto morto. A paciente foi submetida à biópsia da lesão e encaminhada para seguimento ambulatorial sem sucesso, necessitando de retorno ao ambiente hospitalar por piora das condições clínicas e extensão da tumoração. Houve diagnóstico de linfoma de Burkitt, com tratamento multidisciplinar, sendo submetida a protocolo CODOX-M de quimioterapia, com óbito após 22 dias de acompanhamento hospitalar. Conclusão: Este relato demonstra uma situação rara em uma paciente jovem, ressaltando a importância de investigar as alterações mamárias, de maneira eficaz, para um diagnóstico precoce correto e um tratamento adequado, em todas as faixas etárias

Introduction: Primary breast lymphoma (PML) represents about 0.5% of breast cancers, being considered a rare type of tumor. Some of the types of PML, in turn, have an intense connection with the pregnancy and postpartum period due to hormonal stimulation. The aim of this study is to report a case of PML with a rare presentation of Burkitt's lymphoma, considering effective therapeutic proposals for follow-up. Case report: A 23-year-old female patient with a peri-areolar tumor in the right breast with orange peel aspect and rapid growth for one month, phlogistic complaints at the lesion site, good general condition and no other associated symptoms. The reported condition started seven months after fetal expulsion with a dead fetus. The patient underwent lesion biopsy and was referred to an outpatient follow-up with unsuccessful outcome, requiring return to the hospital due to worsening of clinical conditions and extension of the tumor. There was a diagnosis of Burkitt's lymphoma, with multidisciplinary treatment. She underwent the CODOX-M chemotherapy protocol, and died after 22 days of hospital follow-up. Conclusion: This report demonstrates a rare situation in a young patient, emphasizing the importance of effective investigation of breast changes so that correct early diagnosis and appropriate treatment can be made for all age groups

Introducción: El linfoma primario de mama (LMP) representa aproximadamente el 0,5% de los cánceres de mama, siendo considerado un tipo de tumor poco común. Algunos de los tipos de LPM, a su vez, tienen una conexión intensa con el embarazo y el posparto debido a la estimulación hormonal. El objetivo de este estudio es reportar un caso de LMP con rara presentación de linfoma de Burkitt, considerando propuestas terapéuticas efectivas para el seguimiento. Relato del caso: Paciente, 23 años, con un tumor periareolar en la mama derecha con aspecto de piel de naranja y rápido crecimiento durante un mes, quejas flogísticas en el sitio de la lesión, buen estado general y ningún otro. síntomas asociados. La condición reportada comenzó siete meses después de la expulsión fetal con un feto muerto. El paciente fue sometido a biopsia de la lesión y fue derivado sin éxito a seguimiento ambulatorio, requiriendo el retorno al entorno hospitalario por empeoramiento de la clínica y extensión del tumor. Hubo un diagnóstico de linfoma de Burkitt, con tratamiento multidisciplinario. Se sometió al protocolo de quimioterapia CODOX-M y murió a los 22 días de seguimiento hospitalario. Conclusión: Este informe demuestra una situación poco común en una paciente joven, enfatizando la importancia de investigar de manera efectiva los cambios en los senos para un diagnóstico temprano correcto y un tratamiento adecuado en todos los grupos de edad

Analysis of serum immunoglobulin level in children with Burkitt lymphoma / 中华儿科杂志

Objective: To summarize changes of serum immunoglobulin levels before and after chemotherapy in children with Burkitt lymphoma (BL), so as to investigate the effects of chemotherapy and rituximab on serum immunoglobulin levels in children with BL. Methods: Clinical data of 223 children with newly diagnosed Burkitt lymphoma at Beijing Children's Hospital from January 2009 to April 2017 were analyzed retrospectively. They were treated according to the modified LMB 89 regimen and some of them received combined rituximab therapy during the chemotherapy. The serum immunoglobulin (IgA, IgM, IgG) before chemotherapy, at the time of discontinuing chemotherapy, as well as 6, 12, 24, 36 months after chemotherapy were collected. Changes of serum IgA, IgM and IgG with time among different treatment groups were compared using repeated measures ANOVA. Results: According to risk group, 223 children were devided into group B（n=53）and group C（n=170）. Before chemotherapy, 109 cases (48.9%) were combined with hypogammaglobulinemia. The serum IgA, IgM, and IgG levels of all the patients were (0.9±0.7), 1.2 (0.5, 1.3) and (7.2±2.9) g/L before chemotherapy, (0.5±0.4), 0.2 (0.1, 0.3) and (6.3±2.3) g/L at the time of discontinuing chemotherapy (t=13.63, Z=-11.99, t=4.57, all P<0.05). There were statistical difference in IgA, IgM levels of group B and IgA, IgM, IgG levels of group C before chemotherapy and at the time of discontinuing chemotherapy (t=8.86, Z=-6.28, t=11.19, Z=-10.15, t=4.50, all P<0.05). The differences of serum IgA and IgG levels at the time after chemotherapy among patients treated with chemotherapy alone and those treated with chemotherapy combined rituximab in group B and C were significant (F=5.38, P=0.002 and F=4.22, P=0.007). Conclusions: Approximately half of children with BL have already existed hypogammaglobulinemia at initial diagnosis prior to the start of treatment. The modified LMB 89 regimen have significant effect on humoral immunity of children with BL. In the process of immune reconstruction after chemotherapy, rituximab has more significant effect on serum IgA and IgG levels in BL patients.

Treatment outcome in children with central nervous system-positive Burkitt lymphoma using only intrathecal and systemic chemotherapy combined with rituximab / 中华医学杂志(英文版)

BACKGROUND@#With current chemotherapy treatment, >90% of survival has been obtained for Burkitt lymphoma (BL). In this study, the demographic characteristics and treatment outcomes are presented for 78 children in China with central nervous system-positive (CNS+) BL.@*METHODS@#This retrospective study consecutively enrolled 78 CNS+ BL patients in Beijing Children's Hospital (BCH) from 2007 to 2019 who received the BCH B-cell non-Hodgkin's lymphoma regimen (modified by French-American-British mature lymphoma B-cell 96 [FAB/LMB96] C1 arm ± rituximab). Clinical characteristics, methods of disease detection in the CNS, and outcomes were evaluated. Univariate and multivariate analyses were used to assess prognostic factors.@*RESULTS@#The median age of 65 boys and 13 girls at the time of diagnosis was 5.7 years (ranging from 1 to 14 years). Patients were followed up for a median time of 34 months (ranging from 1 to 72 months). Bone marrow invasion was found in 38 (48.7%) patients. There were 48 (61.5%), 44 (56.4%), and 25 (32%) patients with cranial nerve palsy, intracerebral mass (ICM), and para-meningeal extension, respectively. Abnormal cerebrospinal fluid (CSF) morphology and CSF immunophenotype appeared in 15 (19.2%) and 15 (19.2%) patients, respectively. There were 69 (88.5%) patients treated with chemotherapy combined with rituximab, and nine patients were treated solely with chemotherapy. Finally, five patients died of treatment-related infection, recurrence occurred for 13, and one developed a second tumor. The 3-year overall survival and event-free survival rates were 78.9% ± 4.7% and 71.4% ± 6.0%, respectively. Treatment with chemotherapy only, ICM positivity, and >4 organs involved at diagnosis were independent risk factors.@*CONCLUSIONS@#Rituximab combined with a modified LMB96 regimen has greatly increased the efficacy of treatment for Chinese children with CNS+ BL, and with the continuous collection of outcome data, treatment-related complications are decreasing. For further verification, a large sample multicentre randomized controlled study should be performed to explore a treatment scheme for Chinese children with even greater efficacy.

Clinical Characteristics and Prognosis Affecting-Factors of the Patients with Burkitt Lymphoma / 中国实验血液学杂志

OBJECTIVE@#To investigate the clinical characteristics and treatment outcome of patients with Burkitt lymphoma.@*METHODS@#The clinical data of 27 patients with Burkitt Lymphoma were collected and retrospectively analyzed, the clinical characteristics, laboratory data, survival and the factors affecting the prognosis were also analyzed.@*RESULTS@#Among the 27 patients (mainly for adults), the median age was 30 (15-83) years old, the ratio of male and female was 3.5∶1. There was no EB virus infection in all the patients, 92.6% of the patients showed extranodal organs involvement, 40.7% of them were leukemic stage, 85.2% patients belonged to Ⅲ and Ⅳ stage, 74.1% patients belonged to high/high-middle risk according to IPI index. In the terms of molecular biology, five patients were treated with next-generation sequencing test, and the MYC gene mutations were detected out in alt the patients, and the most common mutations were CCND3, ID3 and TP53. The overall response rate (ORR) for all the patients was 85.2%, the complete response (CR) rate was 63.0%, and the partial response rate was 22.2%, the 5-year progression-free survival rate and overall survival rate of the patients was 76.6% and 76.6%, respectively, which showed that the efficacy of the patients in high-dose methotrexate treatment group was higher than that in the non-high high-dose methotrexate treatment group. For the patients treated with LMB89 chemotherapy, the CR was 78.6%, ORR was 100%, the 5-year survival rate was 92.9%, which was superior to the patients treated with other regimens. Auto-hematopoietic stem cell transplantation as consolidation treatment could improve the prognosis for those patients who could not tolerate high-dose chemotherapy. Univariate analysis showed that ECOG score, the level of LDH>500 U/L, WBC level, CNS involvement, short-term effect and LMB89 regimen were the risk factors affecting the prognosis of the patients.@*CONCLUSION@#The adult Burkitt lymphoma are highly aggressive. For the patients in high-dose methotrexate treatment group, especially LMB89 regimen can improve the survival of the patients, and to choose HSCT as a consolidation treatment can be a choice for those patients who could not tolerate high-dose chemotherapy.

Immunodeficiency-associated Burkitt's lymphoma in pediatric patients: a clinical case report

Burkitt's lymphoma, a form of non-hodgkin lymphoma, is a neoplastic monoclonal proliferation of lymphoid cells in areas of the immune system. it can occur in HIV-positive patients, as AIDS is related to the development of non- hodgkin lymphoma. burkitt's lymphoma is a rare subtype, highly prevalent in patients with AIDS. incisional biopsy, in situ hybridization and computerized axial tomography are the appropriate tests to determine the characterize of the lesions. the case of a 4-year-old HIV-positive patient, who developed burkitt's lymphoma of the oral cavity, is reported in this paper. the aim of this case report is to describe the course of the pathology, taking into account its clinical imaging characteristics and treatment.

Linfoma de Burkitt en un escolar con infección perinatal por VIH lentamente progresiva / Burkitt's lymphoma in a school boy with slow progression perinatally acquired HIV

Resumen Las manifestaciones clínicas en los niños con infección por el virus de la inmunodeficiencia humana (VIH) de transmisión perinatal, pueden ser de inicio precoz o tardío. El linfoma asociado a VIH es una manifestación tardía que se asocia a estadios avanzados de inmunosupresión. Se presenta el caso de un escolar de 9 años con diagnóstico de novo de infección por VIH que debutó con un linfoma de Burkitt. En niños, la frecuencia de esta asociación es de 1-2% con pocos casos reportados en la literatura médica.

Children with perinatal human immunodeficiency virus (HIV) infection can present early or late clinical disease. HIV-associated lymphoma is a later manifestation that is associated with advanced immunosuppression (acquired immunodeficiency syndrome -AIDS). This is a case of a 9-year-old boy with recent diagnosis of HIV with Burkitt's lymphoma as first clinical manifestation. In children, the frequency of this association is very low and there are few cases reported.

Linfoma de ovario / Ovarian lymphoma

Introducción: el 50 porciento de la patología oncológica en pediatría corresponde a masas o tumores sólidos, alrededor del 20 porciento del total se ubica en abdomen. Los tumores que se presentan con mayor frecuencia en el abdomen son el nefroblastoma o tumor de Wilms, el linfoma tipo Burkitt, el neuroblastoma y los tumores germinales de ovario. Objetivo: presentar un caso pediátrico con tumor abdominal. Caso clínico: se presenta un caso de una niña de 8 años de edad con antecedentes de salud aparente, atendida en el Hospital Público de la Universidad de Mbarara, Uganda. Resultado: paciente pediátrica que fue ingresada con una historia de 3 meses de dolor abdominal, pérdida ligera de peso y masa abdominal de crecimiento rápido, fue sometida a una laparotomía exploradora donde se resecó la masa tumoral, la que histológicamente arrojó ser un linfoma ovárico, como hallazgo durante la intervención quirúrgica se diagnosticó una agenesia de útero. Conclusión: la paciente se encuentra después del tratamiento con quimioterapia en seguimiento, lleva aparentemente hace 5 meses una vida normal (noviembre 2011)

Introduction: 50 percent of pediatric oncologic pathology corresponds to mass or solid tumors, reaching about 20 percent of total abdomen. The tumors that most frequently occur in the abdomen are nephroblastoma or Wilms tumor, Burkitt's lymphoma, neuroblastoma, and ovarian germ cell tumors. Objective: to present a pediatric case with abdominal tumor. Case report: we report a case of an 8 year old girl with a history of apparent good health. This patient was assisted the Public Hospital of Mbarara University, Uganda. Result: a female pediatric patient was admitted with a 3-month history of abdominal pain, mild weight loss, and rapid growth of an abdominal mass. This patient underwent an exploratory laparotomy where the tumor mass was removed. It was histologically confirmed as an ovarian lymphoma. It was diagnosed uterus agenesis. Conclusion: after chemotherapy, the patient is controlled, and she has an apparently normal life for already 5 months (November 2011)

Patient compliance in the treatment of burkitt's lymphoma in rural Zambia: a retrospective study on 80 burkitt's lymphoma patients in Katete, Zambia

Background : In African settings the treatment results of Burkitt's lymphoma (BL) seem to be less favourable compared with Western settings. The aim of this retrospective study was to analyse some factors that affect the treatment of BL. Patients and Methods : Over a 16 year period; data were extracted of 80 patients. Results : Complete remission 5; very good partial response 35; partial response 16; no response 10; data missing 34. Of all patients; 56did have a positive response to treatment. However; 51of this subgroup of patients did not finish treatment. There was no difference in completion of treatment between patients living in Katete district finishing treatment vs. living outside Katete district (respectively 25vs. 32; P = 0.7148). Conclusion: There is potential for higher cure rates for BL in tropical settings if full effort is put into compliance since a majority of patients; even while having a good prognosis; abandon treatment. Large distance to hospital makes no difference in completing the chemotherapy course

Linfoma de Burkitt: informe de un caso diagnosticado por laparotomía / Burkitt's lymphoma: report of a case diagnosed by laparotomy

El linfoma de Burkitt no endémico es un tumor de frecuente localización abdominal, por lo que se debe tener presente ante cualquier masa intraabdominal. Aunque el tratamiento de elección es la quimioterapia, existe controversia respecto del papel que debe desempeñar la cirugía, especialmente en casos donde el diagnóstico se establece durante una laparotomía exploradora. Este fue el caso de una adolescente de 14 años que presentó una masa hipogástrica y otra menor en la fosa ilíaca derecha. Algunos autores no aceptan la cirugía y añaden que puede retrasar y complicar el tratamiento quimioterápeutico, mientras que otros autores defienden la cirugía reductora de masa tumoral asociada a la quimioterapia. Esta controversia es especialmente importante cuando el diagnóstico se establece en el curso de una laparotomía exploradora, durante la cual hay que decidir si extirpar la masa tumoral o no hacerlo. En nuestro caso decidimos extirpar todo el tumor macroscópico. La buena evolución de nuestra paciente y los resultados comunicados apoyan esta postura(AU)

Non-endemic Burkitt's lymphoma is a tumor of frequent abdominal localization that should be taken into consideration before any intraabdominal mass. Although chemotherapy is the election treatment, there is controversy as regards the role surgery should play, specially in those cases where the diagnosis is established by explorative laparotomy. This was the case of a 14-year-old adolescent that presented a hypogastric mass and another lower mass in the right iliac fossa. Some authors do not accept surgery and state that it may delay and complicate chemotherapy, whereas other authors defend the tumoral mass-reducing surgery associated with chemotherapy. This controversy is particularly important when the diagnosis is made in the course of an explorative laparotomy and it should be decided wether to remove the tumoral mass or not. In our case, it was decided to remove the whole macroscopic tumor. The good evolution of our patients and the results attained support this position(AU)

Apresentação atípica do linfoma de Burkitt: relato de caso / Uncommon presentation of Burkitt's lymphoma: a case report

Linfoma de Burkitt é um linfoma de células B altamente agressivo e proliferativo do tipo não-Hodgkin. Relatamos o caso de um adulto jovem com perda de peso e massa abdominal. A tomografia computadorizada do abdome evidenciava grande massa abdominal com calcificações. Nãohavia sido feito qualquer tipo de tratamento. O diagnóstico foi confirmado com estudo anatomopatológico e imuno-histoquímico.

Burkitt's lymphoma is a rapidly proliferating, highly aggressive B-cell lymphoma of non-Hodgkin subtype. We present a case of a young adult with weight loss and abdominal mass. A computed tomography of abdomen showed a bulky abdominal mass withcalcifications. It has not previously been done any type of treatment. The diagnostic was made by pathology and immunohistochemistry.

Ictericia obstructiva asociada a linfoma de Burkitt en un adulto inmunocompetente / Obstructive jaundice associated Burkitt's lymphoma mimicking pancreatic carcinoma

El compromiso primario del páncreas en los linfomas es muy poco frecuente, sin embargo, en los estadios avanzados de los linfomas no Hodgkin la invasión secundaria de la glándula es observada con mayor frecuencia. El objetivo de esta presentación es describir un caso de linfoma de Burkitt en un adulto inmunocompetente que presentó como manifestación relevante colestasis extrahepática secundaria probablemente a infiltración pancreática difusa y tumores cutáneos cuya histología permitió hacer el diagnóstico. Luego de una dosis única de hidrocortisona de 100mg, mejoró la ictericia, disminuyeron las enzimas de colestasis, las lesiones cutáneas y disminuyó el tamaño del páncreas en la ecografía y en la tomografía computada. Existen en la literatura reportes aislados de casos de linfoma tipo Burkitt que se asocian a ictericia obstructiva secundaria y a infiltración pancreática o del hilio hepático, tratándose en su mayoría de casos pediátricos o de individuos afectados por el virus de la inmunodeficiencia humana (VIH). Creemos que el interés de este caso radica en la rápida respuesta a dosis bajas de corticoides de la colestasis, lo que evitó la necesidad de un procedimiento quirúrgico tanto diagnóstico como terapéutico de la obstrucción biliar, como está referido en la literatura, permitiendo instaurar rápidamente el tratamiento quimioterapéutico específico de esta entidad sin maniobras quirúrgicas o endoscópicas.

The primary compromise of the pancreas in lymphomas is uncommon. However, in advanced stages of Non- Hodgkin’s lymphomas (LNH) the secondary invasion of the pancreas is observed more frequently. Jaundice due to extrahepatic cholestasis as a presentation form is extremely rare, with only few cases described in the literature. The aim is to present a case of an obstructive jaundice as an expression of Burkitt’s lymphoma probably due to a diffuse pancreatic infiltration in an adult without immunodeficiency with a rapid response of cholestasis to low dose of hydrocortisone. Skin tumor simultaneously present with jaundice allowed the histologic diagnosis with skin biopsies. After a unique dose of 100 mg hydrocortisone, jaundice improved and cholestatic enzymes decreased, pancreas became smaller and common bile duct diameter became normal at ultrasound and CT scan, also skin tumors turn pale and diminished in size. There are isolated reports of Burkitt’s lymphoma cases with associated obstructive jaundice due to pancreatic infiltration or by compression by lymph nodes of the bile ducts, many of them are pediatric cases or immunodepressed HIV patients. In the case presented, surgical resection of the pancreatic infiltration and biliary drainage, either surgical or endoscopic during the same procedure was not necessary for the histopathologic diagnosis of the illness like is described in the literature. The diagnosis was suspected by the rapid decrease of cholestatic features after a single dose of hydrocortisone and the histology was easy done by a skin biopsy. We think the interest in this case is the quick response to low doses of corticoids, which avoided the necessity of surgical procedure for the diagnosis of the biliary tree obstruction, allowing a quick implementation of the specific chemotherapeutic treatment of the lymphoma without any surgical or endoscopic procedures to heal the jaundice.

Burkitt's lymphoma of the breast and ovary, case report

A 17 year old Sudanese female with breast lump and ovarian mass proved to be Burkitt's lymphoma of the breast and ovary. She received 8 cycles of CHOP chemotherapy and Intrathecal methotrexate and achieved complete response. Three years after treatment she is well without recurrence. A lymphoma should always be considered in the differential diagnosis of a breast tumor, especially in very young patients. It needs a different work-up and treatment

Le lymphome de Burkitt en milieu hospitalier du Burkina Faso: Aspects thérapeutiques et évolutifs

Une étude rétrospective portant sur 41 dossiers d'enfants hospitalisés et traités pourlymphome de Burkitt, sur une période de 10 ans, a été effectuée dans les services de pédiatrie et de chirurgie maxillo-faciale de l'hôpital de Bobo-Dioulasso aux fins d'étudier les aspects thérapeutiques et évolutifs de la maladie. Les moyens thérapeutiques ont été la chirurgie d'ablation des volumineuses tumeurs (19,5%) et la monochimiothérapie au cyclophosphamide (83%) selon les procédés de Burkitt(64,7%) et de N'gu (35,3%). La létalité sous chimiothérapie était de 8,8%. L'évolution immédiate a montré 58,8% de rémission complète, 26,5% de rémission partielle, 11,8% de résistance au traitement

In vitro resistance of leukaemic blasts to prednisolone in bcr-abl positive childhood acute lymphoblastic leukaemia.

BACKGROUND & OBJECTIVES: Although the outcome of children with acute lymphoblastic leukaemia (ALL) has improved dramatically over the last decade, some children still fare poorly and relapses are seen. The sensitivity of leukaemic cells to corticosteroids has emerged as an important prognostic factor in ALL. The t(9,22) translocation, resulting in the bcr-abl fusion gene, is a non-random translocation found in B-lineage acute lymphoblastic leukaemia. It is also known to be an independent poor prognostic factor for long-term disease free survival. We studied the association between the presence of bcr-abl fusion gene and in vitro prednisolone resistance in children with B-lineage acute lymphoblastic leukaemia at diagnosis. METHODS: A total of 23 children (aged 1-16 yr, median age: 12 yr) with B-lineage acute lymphoblastic leukaemia at diagnosis were included in the study. The presence of bcr-abl fusion gene was determined by reverse transcriptase-polymerase chain reaction (RT-PCR) and the in-vitro resistance to prednisolone was measured by short term colorimetric methyl thiazol tetrazolium (MTT) assay. RESULTS: A median LD50 (lethal dose for 50% cells) for prednisolone in bcr-abl positive children (n=7) was 1.6 mg/ml (range: 0.25-5.0 mg/ml) and that of bcr-abl negative children (n=16) was 0.35 mg/ml (range 0.62-1.0 mg/ml). The median LD50 for prednisolone differed significantly between the bcr-abl positive and negative groups of children with acute lymphoblastic leukaemia (P<0.005). INTERPRETATION & CONCLUSION: This is probably the first report to show that leukaemic blasts of bcr-abl positive children with ALL are about four-fold resistant to prednisolone as compared to blasts from bcr-abl negative children. This suggests that one of the reasons for the poor prognosis of bcr-abl positive ALL could be a lower steroid sensitivity.

Non endemic Burkitt's lymphoma of the tonsil

Non-endemic Burkitt's lymphoma [NEBL] is a high-grade non Hodgkin's lymphoma of B-cell origin found sporadically outside Africa. Involvement of extra nodal head and neck sites occurs very infrequently. Two cases of palatine tonsillar swelling are described. Both cases presented mainly with unilateral pharyngeal swelling and change of vioice for few weeks

Linfoma de Burkitt / Burkit's Lymphoma

O Linfoma de Burkitt é a neoplasia näo-Hodgkin mais comum na infância. Incide em cerca de 7:1.000.000 criança e afeta em especial meninos, maiores de 5 anos, com pico entre 7 e 11 anos. A forma de apresentaçäo mais comum é abdominal, com frequente envolvimento do sistema nervoso e medula óssea. O diagnóstico clínico-laboratorial é confirmado por exames de imageamento tumoral, em especial a tomografia computadorizada. Os autores apresentam o caso de uma criança de 2 anos de idade com Linfoma de Burkitt gastrointestinal, tratada com cirurgia e quimioterapia e com seguimento clínico satisfatório em 6 meses de acompanhamento

Síndrome de lisis tumoral en pacientes pediátricos con linfoma de Burkitt / Tumor lysis syndrome in pediatric patients with Burkitt´s lymphoma

Objetivos: determinar la prevalencia, manifestaciones clínicas de laboratorio de los pacientes con lisis tumoral en el linfoma de Burkitt y reseñar su tratamiento. Métodos: Revisión retrospectiva de los pacientes con linfoma de Burkitt (n=99) ingresados en 2 protocolos de tratamiento en el Hospital de Pediatría J. P. Garrahan entre 1988 y 1996. El tratamiento y la prevención de la lisis tumoral se basaron en una norma previamente aprobada. Todos los pacientes recibieron como primer tratamiento una prefase con moderadas dosis de quimioterapia. Resultados: Doce pacientes (12.1 por ciento) presentaron lisis tumoral, 11 al diagnóstico y 1 durante la evolución. Todos tenían enfermedad tumoral extensa. La hiperuricemia fue la alteración màs frecuente (n=11) siendo sus valores màs altos al diagnóstico, tendiendo a normarlizarse en los días subsiguientes. Siete pacientes presentaron kiperkalemia y 6 hiperfosfatemia, tendiendo a aumentar luego de iniciado el tratamiento. Cuatro pacientes presentaron hipocalcemia (3 de ellos con tetania). En 7 pacientes se detectó elevación de la creatinina (en dos de ellos desde el diagnóstico). Cuatro pacientes requirieron hemodiálisis por falta de tratamiento conservador (en 2 de ellos asociado a sepsis). Un paciente falleció por lisis tumoral e hiperkalemia y otros 2 fallecieron por lisis tumoral asociada a fallo multiórganico y sepsis; la mortalidad global por este síndrome fue del 3 por ciento del total de casos. Conclusiones: La lisis tumoral es una complicación frecuente de severidad variable en estos pacientes. Si bien la morbilidad fue grande, la mortalidad directamente atribuida a la lisis tumoral fue baja.

B-Lineage acute lymphoblastic leukemia of childhood. An institutional experience

A total of 119 children (1990-95) with acute lymphoblastic leukemia (ALL) B-lineage either CD10+or CD10- were registered into a single non-randomized chemotherapy protoco. Only untreated patients with standard risk were included in the study. Their ages ranges from 1.8-10 years with a mean of 5.1 years. There were 82 (68 percent) children with early pre B-All, 35 (29 percent) with pre B-All and 2 (1.6 percent) with transititional pre B-All (p<0.00001). The patients were divided according to CD10 reactivity, either + (94 children) or -(25 patients). The event-free survival (EFS) at 60 months for the CD10+children was of 78 percent (alive 73/94), while for the CD10- was 71 percent (alive 18/25) (p=0.6) and 74 percent for both groups. The factors that influenced favorably the survival in the CD10+group were the age between 3 to 5.99 years (p<0.00001), sex (either male or female), leukocyte count between 10.24.9 x 10-9/l (p<0.00001), LDH under 300 U/I (p<0.00001) and L1 bone marrow cytomorphology (p<0.00001). In the CD10- patients, the EFS was favorably influenced by the female sex (p=0.04), leukocyte count under 10 x 10-9/l (p=0.05) and LDH < 300 U/l (p=0.02). CNS infiltration was documented in 4.2 percent (5/119). Mortality secondary to chemotherapy was seen in 7 percent. In conclusion, this is the first large series in Mexican children with B-lineage ALL published. Because of the relatively small number of patients in each group (pre B and transitional pre B), all the patients in the current serieswere treated alike. When the 119 patients were divided only on the basis of CD10 reactivity, the EFS for both groups (CD10+ and') was similar; therefore, the reactivity to CD10 has no prognostic value in this type of ALL

Infecciones en el paciente neutropénico / Infections in neutropenic patients

Estudio prospectivo de 74 períodos consecutivos de neutropenia severa (menor o igual a 500 u/L) en 38 pacientes hematológicos del Hospital Clínico de la U. de Chile. La leucemia aguda constituye la principal enfermedad de base y la quimioterapia fue utilizada en el 81 por ciento de los episodios. En 60 períodos (81 por ciento) se logró demostrar infección y 51 de ellos (69 por ciento) cursaron febriles. Destacamos la presencia de 9 episodios de infección de curso afebril por constituir un hecho excepcional en estos pacientes. En los 60 períodos de neutropenia con infección se logró demostrar en el 80 por ciento uno o más focos, destacando el pulmonar, orofaríngeo, mucocutáneo y abdominal. El estudio microbiológico fue positivo en el 45 por ciento de los episodios febriles; se aislaron 34 microorganismos (bacilos Gram (-) 62 por ciento, cocáceas Gram (+) 26,3 por ciento, hongos 8,8 por ciento y virus 2,9 por ciento. Se constataron 11 bacteremias, todas por bacilos Gram (-) predominando K. pneumoniae y E. coli. El esquema ceftazidima-amikacina fue el más utilizado y se modificó en más del 50 por ciento de los casos (se asoció vancomicina, anfotericina B, u otro, o se reemplazó por imipenem, vancomicina y anfotericina B), con éxito terapéutico en el 75 por ciento de los casos. Se produjeron 17 fallecimientos, 13 de causa infecciosa. La mortalidad se asoció al diagnóstico de leucemia aguda, neutropenia prolongada, foco pulmonar y bacteremia por Gram (-). Los episodios febriles sin foco ni bacteriología se asociaron a buen pronóstico