Hepatoesplenomegalia relacionada a linfoma de Burkitt en paciente pediátrico / Hepatoesplenomegalia related to Burkitt´s lymphoma in a pediatric patient

RESUMEN El linfoma de Burkitt, se trata de un subtipo poco frecuente del linfoma no Hodgkin, con elevada frecuencia en aquellos pacientes con sida. La hepatoesplenomegalia es un signo clínico de gran importancia para el diagnóstico oportuno de algunas patologías; entre los mecanismos de formación de la hepatoesplenomegalia se encuentra la infiltración celular, ocasionada por la migración de células tumorales. Se presenta por inflamaciones debido a la presencia de infecciones por virus o bacterias las cuales son muy comunes en pacientes con sida. Se presentó un caso de un paciente masculino de 4 años, diagnosticado con VIH positivo, con la configuración correspondiente de criterios clínicos en clasificación C para sida. El cual desarrolló a nivel de cavidad oral un Burkitt primario, que se acompañó de hepatoesplenomegalia. Se pretendió describir la relación y el comportamiento de este tipo de linfoma con la hepatoesplenomegalia, así como la repercusión a nivel del sistema estomatognático, a nivel sistémico y el plan de tratamiento. Por el cuadro clínico e inmunológico del paciente estudiado, se planteó un pronóstico reservado por presentar un cuadro clínico infrecuente, en el que se observó Burkitt; tanto a nivel del sistema estomatognático como a nivel abdominal. Se hizo necesario realizar un diagnóstico oportuno y certero para iniciar el tratamiento a tiempo, se comenzó inmediatamente con tratamiento (AU).

ABSTRACT Burkitt lymphoma (BL) is a rare subtype of non-Hodgkin lymphoma, with high frequency in those patients with AIDS. Hepatosplenomegaly is a clinical sign of great importance for the timely diagnosis of some pathologies; cellular infiltration is found among the mechanisms of hepatosplenomegaly formation; it is caused by the migration of tumor cells. It emerges by inflammations due to the presence of infections by virus or bacteria which are very common in patients with AIDS. The authors present the case of a male patient, aged 4 years, with a positive HIV diagnosis, and the correspondent configuration of clinical criteria in C classification for AIDS, who developed a primary Burkitt lymphoma at the level of oral cavity We present the case of a 4-year-old male patient diagnosed with HIV positive, with the corresponding configuration of clinical criteria in classification C for AIDS; who developed a primary LB at the oral cavity level that was accompanied by hepatosplenomegaly. The authors pretended to describe the relation and behavior of this kind of lymphoma with hepatosplenomegaly, and also the repercussion at the stomatognathic level, at the systemic level and the treatment plan. Due to the clinical and immunological characteristics of the studied patient a reserved prognosis was given because of presenting infrequent clinical characteristics in which a Burkitt was observed both, at the stomatognathic and at the abdominal level. It was necessary to make an opportune and accurate diagnosis to begin the treatment on time (AU).

Hematoma retroperitoneal de causa infrecuente: a propósito de un caso / Retroperitoneal hematoma of unusual cause: a case report

The spontaneous retroperitoneal hematoma (SRH) is defined as a spontaneous hematic collection located in any of the retroperitoneal compartments. The most frequently encountered cause of retroperitoneal hemorrhage is a ruptured abdominal aortic aneurysm, followed by renal and adrenal tumors, along with some other less common causes. Performing accurate diagnosis of patients at admission has proven to be difficult, given that the clinical presentation is ample and variable and may mimic other pictures of abdominal or lower back pain. Imaging techniques, mainly angiography, computed tomography, and MR Imaging, represent a fundamental tool for both timely diagnosis and proper management of the condition. We report the case of a 56-year-old male patient with diagnosis of HIV, who presented with a history of abdominal pain and palpable abdominal mass. After a thorough imaging evaluation, patient was diagnosed with retroperitoneal hematoma, whose histological analysis revealed a Burkitt lymphoma.

El hematoma retroperitoneal espontáneo (HRE) se define como una colección hemática espontánea, situada en cualquiera de los compartimentos retroperitoneales. Dentro de las causas más frecuentes se encuentran las roturas de aneurisma de aorta abdominal, tumores renales y suprarrenales, entre otras. Habitualmente es difícil realizar el diagnóstico al ingreso del paciente, ya que la presentación clínica es variable y puede simular otros cuadros de dolor abdominal o lumbar. Las técnicas de imagen, especialmente la angiografía, tomografía computada y resonancia magnética, son una herramienta fundamental en el diagnóstico y manejo oportuno del cuadro. Presentamos el caso de un varón de 56 años de edad con diagnóstico de VIH en tratamiento, que debutó como un cuadro de dolor y masa abdominal palpable. Luego de un acabado estudio de imágenes, se diagnosticó un HRE cuyo estudio histológico reveló un Linfoma de Burkitt.

Intussuscepção intestinal em adultos jovens: relato de caso e revisão de literatura / Intestinal intussusception in young adults: literature review

Embora sejam a principal causa de obstrução intestinal na população pediátrica, intussuscepções intestinais são eventos raros em adultos e quando acontecem, têm características clínicas diferentes dos seus equivalentes em crianças. O objetivo desse trabalho é de apresentar um caso de um jovem do sexo masculino, de 16 anos, previamente hígido, que procurou o serviço de emergência do Hospital Universitário da Universidade de São Paulo com um quadro de intussuscepção intestinal como primeira manifestação de um linfoma não-Hodgkin difuso de células B de alto grau, tipo Burkitt. Foi realizada uma revisão da literatura pertinente, e aspectos relevantes do caso são discutidos à luz dessas informações.

Although intussusception is the main cause of intestinal obstruction in the pediatric population, it is a rare condition in adults, and when it happens, the clinical characteristics differ a lot from the pediatric group. The purpose of this article is to report a case of a 16 years-old male that was seen at the Emergency Room of the University Hospital of São Paulo with an intestinal intussusception as the first clinical presentation of a Burkitt lymphoma. A literature review was carried out and relevant aspects of the case are discussed.

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.

Fatal secondary pulmonary hypertension due to cardiac involvement in AIDS-associated Burkitt's lymphoma.

Primary cardiac lymphomas are rare lesions in children with acquired immunodeficiency syndrome (AIDS). Most of them are high-grade Burkitt's or Burkitt-like lymphomas. They usually present with congestive cardiac failure, pericardial effusion or tamponade, arrhythmias, with predominant systemic 'B' symptoms and often with widespread extranodal involvement. The clinical profile and operative and pathological findings of a 4-year-old boy with AIDS-associated Burkitt's lymphoma of the heart presenting with acute right heart failure and fatal secondary pulmonary hypertension is reported.

Insuficiência renal aguda como manifestação inicial de linfoma de Burkitt renal / Acute renal failure as first clinical presentation of BurkittÆs renal lymphoma

Relatamos o caso de um jovem de 14 anos que procurou atendimento médico por edema e hipertensão, sendo insuficiência renal aguda secundária a linfoma de Burkitt renal diagnosticada após avaliação laboratorial, de imagem e biópsia de massa tumoral renal.

This case report is from a 14-year-old boy who had edema and hypertension due to a primary renal Burkitt's lymphoma, diagnosed through laboratory and image evaluation and kidney tumor biopsy.

A man with thyrotoxicosis, lymphoma and thymic hyperplasia.

We report a case of non-Hodgkin's lymphoma of Burkitt's type with thyrotoxicosis and thymic hyperplasia in a 27-year old man. As far as we are aware this is the first reported case of Burkitt's lymphoma, thymic hyperplasia and thyrotoxicosis occurring in the same patient.

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.

Non-Hodgkin's lymphoma associated with the acquired immunodeficiency syndrome

Patients with the Acquired Immunodeficiency Syndrome (AIDS) have an increased risk of developing Non-Hodgkin's Lymphomas (NHL). It is estimated that 8-27 of newly diagnosed NHL are associated with AIDS. The majority of these lymphomas are of intermediate or high grade histology. We retrospectively analyzed the clinical manifestations and response to different chemotherapy regimens in patients with AIDS NHL in the San Juan City and San Juan Veterans Administration Hospitals from 1990-1993. Eleven patients (10 M/1F) with an average age of 46 (range 31-68) were analyzed. 64 (7/11) of patients had a prior diagnosis of AIDS before the diagnosis of NHL. Pathology was diffuse large cell in 6, Burkitt's in 4 and immunoblastic in 1.73 of patients presented with Stage III/IV. Sites included gastrointestinal tract (2), skin/soft tissue (2), paranasal sinus (1), kidneys (1). The most common treatment regimen was the modified m-BACOD (64). Complete response (CR) occurred in 46 (5/11) and partial response (PR) in 27 (3/11). 36 of patients developed opportunistic or bacterial infections during treatment. Median survival was 8 months. Two patients are long-time survivors at 24+, 29+ without evidence of NHL at present. This group of patients demonstrate the usual characteristics of NHL in AIDS patients and corroborate that conventional chemotherapy programs are only moderately effective in these patients. It is clear from this data and from other reports that further work will be required to ascertain optimal therapy for the patient with AIDS-related Non-Hodgkin's Lymphoma

Neumonía por citomegalovirus y linfoma de Burkitt: caso clínico y discusión de la literatura / Cytomegalovirus pneumonia and Burkitt lymphoma: clinical case and literature review

A raíz de un caso de un niño con linfoma de Burkitt, en tratamiento quimioterápico con neutropenia, el cual desarrolla un síndrome febril con neumonia intersticial y en el que se demuestra una infección por CMV, los autores discuten el enfrentamiento diagnóstico y terapéutico

Nervous system involvement in Burkitt's lymphoma.

A patient with Burkitt's lymphoma who had nervous system involvement at multiple levels is presented because of the rarity of this condition in India. A high index of suspicion and a rapidly evolving multiple neuraxial involvement led to the diagnosis.

Comprometimento medular por linfoma tipo Burkitt: relato de um caso / Medullary involvement by lymphoma type Burkitt: report of a case

Registro de caso de linfoma tipo Burkitt com comprometimento intrarraqueano em criança de três anos de idade. Consideraçöes sobre esse tipo de tumor säo feitas em funçäo do caso observado e de dados da literatura