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).

Use of Iodized Oil and Gelatin Sponge Embolization in Splenic Artery Coiling Reduces Bleeding from Laparoscopic Splenectomy for Cirrhotic Portal Hypertension Patients with Complicating Hypersplenic Splenomegaly: A Comparative Study.

OBJECTIVE: Laparoscopic splenectomy (LS) is relatively contraindicated in cirrhotic portal hypertension (CPH) patients with complicating hypersplenic splenomegaly (HS). This study aimed to evaluate the effectiveness and safety of incorporation of iodized oil and gelatin sponge embolization to conventional splenic artery embolization (cSAE) with coiling in CPH/HS patients undergoing LS. PATIENTS AND METHODS: Between April 2012 and March 2014, eligible CPH/HS patients (n = 56) were assigned to preoperative modified SAE (mSAE) with LS (mSAE+LS group, n = 16), cSAE with LS (cSAE+LS group, n = 20) or LS alone (LS group, n = 20). Main outcome measures included frequency of conversion to laparotomy, operative time, intraoperative bleeding, and transfusion. RESULTS: The three groups had similar baseline characteristics (all P > .05). mSAE and cSAE similarly decreased LS conversion frequency (mSAE+LS versus cSAE+LS versus LS, 0.0% versus 10.0% versus 30.0%, P = .030) and operative time (155 ± 23 minutes versus 170 ± 26 minutes versus 221 ± 42 minutes, P < .001) compared with LS alone. mSAE significantly reduced bleeding (178 ± 22 mL versus 250 ± 27 mL versus 328 ± 67 mL, P < .001) compared with cSAE and LS alone. The three groups had similar postoperative recovery times and surgical morbidities (all P > .05). CONCLUSIONS: In CPH/HS patients, preoperative SAE reduced LS conversion frequency and reduced operative time compared with LS alone, while mSAE further decreased volume of blood loss.

ß-Thalassemia intermedia: a comprehensive overview and novel approaches.

ß-Thalassemia intermedia is a clinical condition of intermediate gravity between ß-thalassemia minor, the asymptomatic carrier, and ß-thalassemia major, the transfusion-dependent severe anemia. It is characterized by a significant clinical polymorphism, which is attributable to its genetic heterogeneity. Ineffective erythropoiesis, chronic anemia, and iron overload contribute to the clinical complications of thalassemia intermedia through stepwise pathophysiological mechanisms. These complications, including splenomegaly, extramedullary erythropoiesis, iron accumulation, leg ulcers, thrombophilia, and bone abnormalities can be managed via fetal hemoglobin induction, occasional transfusions, chelation, and in some cases, stem cell transplantation. Given its clinical diversity, thalassemia intermedia patients require tailored approaches to therapy. Here we present an overview and novel approaches to the genetic basis, pathophysiological mechanisms, clinical complications, and optimal management of thalassemia intermedia.

Complementary Indicators for Diagnosis of Hepatic Vein Stenosis After Pediatric Living-donor Liver Transplantation.

INTRODUCTION: Although hepatic vein stenosis after liver transplantation is a rare complication, the complication rate of 1% to 6% is higher in pediatric living-donor liver transplantation than that in other liver transplantation cases. Diagnosis is very important because this complication can cause hepatic congestion that develops to liver cirrhosis, graft loss, and patient loss. However, this is unlikely in cases where there are no ascites or hypoalbuminemia. OBJECTIVES: Eleven of 167 patients who had undergone pediatric living-donor liver transplantation were identified in the outpatient clinic at Jichi Medical University as having suffered from hepatic vein stenosis, and were enrolled in the study. METHODS: We conducted a retrospective study in which we reviewed historical patient records to investigate the parameters for diagnosis and examine treatment methods and outcomes. RESULTS: The 11 patients were treated with 16 episodes of balloon dilatation. Three among these received retransplantation and another 2 cases required the placement of a metallic stent at the stenosis. Histological examination revealed severe fibrosis in four of nine patients who had a liver biopsy, with mild fibrosis revealed in the other five grafts. Furthermore, hepatomegaly and splenomegaly diagnosed by computed tomography, elevated levels of hyarulonic acid, and/or a decrease in calcineurin inhibitor clearance were found to be pathognomonic at diagnosis, and tended to improve after treatment. CONCLUSIONS: Diagnosis of hepatic vein stenosis after liver transplantation can be difficult, so careful observation is crucial to avoid the risk of acute liver dysfunction. Comprehensive assessment using volumetry of the liver and spleen and monitoring of hyarulonic acid levels and/or calcineurin inhibitor clearance, in addition to some form of imaging examination, is important for diagnosis and evaluation of the effectiveness of therapy.

Research and control of advanced schistosomiasis japonica in China.

Among the three main schistosomes (Schistosoma japonicum, Schistosoma mansoni, and Schistosoma haematobium) known to infect humans, S. japonicum causes the most serious pathological lesions. In China, only schistosomiasis japonica is transmitted. From the 1950s, massive epidemiological investigations and active control measures for schistosomiasis japonica have been carried out. At the early stage of schistosomiasis control program, there were about 12 million schistosomiasis patients, and about 5% of schistosomiasis patients belong to advanced patients, which was 600,000. After more than a half century of active schistosomiasis control work, the schistosomiasis situation has been reduced markedly. The nearest epidemiological investigation showed that, by the end of 2012, there were still 240,000 schistosomiasis patients with the descent rate of 98% and 30,000 advanced patients with the descent rate of 95%. This paper reviews the rich experiences of advanced schistosomiasis research and control in China, including that the epidemiology researches confirm there is a family aggregation of advanced schistosomiasis and advanced schistosomiasis patients have no significance to the schistosomiasis transmission in transmission-interrupted areas but still are an infection source in endemic areas; pathogenic mechanism researches verify that genetic factors and immunoregulation play important roles in the disease developing process; ultrasound image examinations are used not only in the diagnosis and differential diagnosis of advanced schistosomiasis but also in the guidance of treatment and evaluation of therapeutic effects and, furthermore, in the risk predictions of portal hypertension and upper gastrointestinal hemorrhage; clinical practices demonstrate that praziquantel can be used in most of advanced schistosomiasis patients, and the therapy not only can interrupt the schistosomiasis transmission somewhat but also is favorable for liver fibrosis improvement; the ascetic fluid concentration afflux is used in the therapy for obstinate ascites, and endoscopic varices ligation is used in the treatment of upper gastrointestinal bleeding, and both have good effects; hundreds and thousands of severe splenomegaly advanced schistosomiasis patients received splenectomy, and the long-term survival rate is more than 90%, most of them are basically cured from the disease and their labor force recovers, some dwarf patients begin growing and developing again, and some sterile women became fertile; the researches of traditional Chinese medicine in the treatment of liver fibrosis have made progress, such as Cordyceps sinensis showing some anti-fibrosis effect in the animal experiments and primary clinical trials; the animal experiments and epidemiological investigations indicate that schistosome infection is one of the carcinogenesis risk factors, especially for liver cancer. In conclusion, these experiences and lessons are plentiful and worth sharing with the peers of other endemic countries for reference.

Adverse prognostic features in 251 children treated for acute myeloid leukemia.

Potential predictors of event-free survival (EFS) were assessed in 251 consecutively diagnosed children treated for acute myeloid leukemia (AML) on three successive clinical trials. The lack of significant differences in 4-year EFS for these studies (20% +/- 4%, 29% +/- 4%, and 20% +/- 7%) permitted combined analysis of presenting features. Splenomegaly (P = .002), coagulation abnormalities (P = .001), leukocyte count > or = 10 x 10(9)/L (P = .002), and age > 14 years (P = .01) were statistically significant predictors of a poorer EFS by univariate analysis and retained significance in multivariate analysis. Age < 2 years and monocytic leukemias (often cited as adverse factors in AML) showed no prognostic influence in this study. The estimated relative risk of failure for a child with a single adverse feature at diagnosis was at least 1.4 times greater than that for a patient with no adverse features. For children with two or more adverse features, the relative risk increased by more than threefold. These clinical variables, alone or in combination, may identify important subgroups of patients with AML at high risk for failure and for whom improved or alternative therapies are especially important.

The biological suppression of malaria: an ecological and nutritional interrelationship of a host and two parasites.

The interrelationship of malaria and severe ascariasis was studied in Anjouan children with a previously described syndrome of enlarged parotids, localized forehead edema, heavy infestation with Ascaris lumbricoides, and unusual freedom from malaria. After treatment of 37 such children with the ascaricide piperazine, 35 had resolution of parotid enlargement and forehead edema, but 19 developed attacks of malaria. Children treated with placebo had neither resolution of clinical findings nor attacks of malaria. We propose that suppression of the malaria in these children is a nutritional consequence of severe ascariasis and may represent an ecological balance for optimum co-survival of the host and the two parasites.