Mantle cell lymphoma presenting with lethal atraumatic splenic rupture

Mantle cell lymphoma is characterized by t(11;14) with CCND1-IGH fusion and manifests with a spectrum of disease ranging from relatively indolent to aggressive. Here, we present a case of pleomorphic mantle cell lymphoma with three fusion signals that presented with lethal atraumatic splenic rupture. We discuss on the implications of variant CCND1 signal patterns as well as the epidemiology and pathophysiology of atraumatic splenic rupture.

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).

Quiste esplénico gigante. A propósito de un caso / GIANT SPLENIC CYST. CASE REPORT

La hidatidosis o equinococosis quística es una zoonosis causada por el cestode Echinococcus granulosus, responsable de importante morbilidad y mortalidad en todo el mundo. La presentación esplénica aislada tiene escasos reportes en la literatura mundial.

Hydatidosis or cystic echinococcosis is a zoonosis caused by the cestode of Echinococcus granulosus, responsible for significant morbidity and mortality worldwide. Isolated splenic presentation has few reports in the world literature. The aim of this report was to describe an unfrequent case of isolated splenic hydatidosis due to its etiologic value because of the inexistence of a simultaneous hepatic involvement

Regression of esophageal varices and splenomegaly in two patients with hepatitis-C-related liver cirrhosis after interferon and ribavirin combination therapy

Some recent studies have found regression of liver cirrhosis after antiviral therapy in patients with hepatitis C virus (HCV)-related liver cirrhosis, but there have been no reports of complete regression of esophageal varices after interferon/peg-interferon and ribavirin combination therapy. We describe two cases of complete regression of esophageal varices and splenomegaly after interferon-alpha and ribavirin combination therapy in patients with HCV-related liver cirrhosis. Esophageal varices and splenomegaly regressed after 3 and 8 years of sustained virologic responses in cases 1 and 2, respectively. To our knowledge, this is the first study demonstrating that complications of liver cirrhosis, such as esophageal varices and splenomegaly, can regress after antiviral therapy in patients with HCV-related liver cirrhosis.

Prediction of large esophageal varices in cirrhotic patients using classification and regression tree analysis

OBJECTIVES: Recent guidelines recommend that all cirrhotic patients should undergo endoscopic screening for esophageal varices. That identifying cirrhotic patients with esophageal varices by noninvasive predictors would allow for the restriction of the performance of endoscopy to patients with a high risk of having varices. This study aimed to develop a decision model based on classification and regression tree analysis for the prediction of large esophageal varices in cirrhotic patients. METHODS: 309 cirrhotic patients (training sample, 187 patients; test sample 122 patients) were included. Within the training sample, the classification and regression tree analysis was used to identify predictors and prediction model of large esophageal varices. The prediction model was then further evaluated in the test sample and different Child-Pugh classes. RESULTS: The prevalence of large esophageal varices in cirrhotic patients was 50.8 percent. A tree model that was consisted of spleen width, portal vein diameter and prothrombin time was developed by classification and regression tree analysis achieved a diagnostic accuracy of 84 percent for prediction of large esophageal varices. When reconstructed into two groups, the rate of varices was 83.2 percent for high-risk group and 15.2 percent for low-risk group. Accuracy of the tree model was maintained in the test sample and different Child-Pugh classes. CONCLUSIONS: A decision tree model that consists of spleen width, portal vein diameter and prothrombin time may be useful for prediction of large esophageal varices in cirrhotic patients.

Correlación clínica- para-clínica en un escolar con hipertensión porta de etiología a esclarecer / Clinical-paraclinical in a school with portal hypertension to determine

La hipertensión porta (HTP) es el resultado del incremento de la presión dentro del sistema venoso porta. Se presenta con poca frecuencia en el paciente pediátrico pero es una de las mayores causas de morbilidad y mortalidad en el niño con enfermedad hepática. La mayoría de los pacientes con http presentan un estado hiperdinámico, lo cual aumenta el flujo venoso porta y mantiene la hipertensión. Puede ser secundaria a obstrucción a nivel prehepático, intrahepático o extrahehepático.

Portal hypertension (PH) is the result of increased pressure within the portal venous system. It occurs infrequently in the pediatric patient but it is a major cause of morbidity and mortality in children with liver disease. Most patients with PH have a hyperdynamic state, which increases venous flow and portal hypertension remains. May be secondary to obstruction at prehepatic, intrahepatic or extrahehepatic.

Angiomatosis difusa del bazo como causa de esplenomegalia / Diffuse splenic angiomatosis as a cause of splenomegaly

La angiomatosis difusa del bazo constituye una causa de esplenomegalia no tenida en cuenta habitualmente en el diagnóstico diferencial de este problema clínico. Se ha relacionado con infecciones por diversos microorganismos del tipo Bordetella. Se presenta un paciente del sexo masculino con dolor en hipocondrio izquierdo de 2 meses de evolución y esplenomegalia aislada. Tras la esplenectomía se precisa el diagnóstico de angiomatosis difusa del bazo. No se pudo relacionar esta con infección por Bordetella. Se concluye que este diagnóstico debe tenerse en cuenta ante cualquier paciente con esplenomegalia aislada.

Diffuse splenic angiomatosis is a cause of splenomegaly that is not usually considered in the differential diagnosis of this clinical problem. It has been related to infections caused by different Bordetella microorganisms. A male patient with pain on the left hypochondrium of 2 months of evolution and isolated splenomegaly was presented. After splenectomy, the diagnosis of diffuse splenic angiomatosis was confirmed. It could not be connected with Bortedella infection. It was concluded that this diagnosis should be taken into account in any patient with isolated splenomegaly.

Autoimmune lymphoproliferative syndrome (ALPS): a rare cause of immune cytopenia.

Autoimmune Lymphoproliferative syndrome (ALPS) is an inherited disorder manifesting with autoimmune cytopenia, lymphadenopathy and splenomegaly. The differential diagnosis includes infections, autoimmune disorders or malignancies. The disease is characterized by accumulation of double negative (CD3+ CD4- CD8-) T cells (DNT) in the peripheral blood. We describe a case and review the literature.

Indications de la splenectomie dans une zone a forte endemicite palustre

Dans les zones d'endemie palustre; un fort pourcentage de la population; avoisinant 75; a une splenomegalie palustre hyperreactive et certaines d'entre elles necessitent une splenectomie. Objectif: Chez les patients ayant une splenomegalie enorme d'origine palustre; determiner les circonstances et les complications pour lesquelles la splenectomie est indiquee ou devient un acte indispensable; voire imperatif. Methodes: Parmi les 48 splenectomies realisees en deux ans (mars 1999 a mars 2001) dans le service de chirurgie du Centre Hospitalier de District de niveau II de Moramanga; nous avons etudie 31 dossiers de patients splenectomises pour splenomegalie enorme d'origine palustre. Resultats: Sur les 48 patients splenectomises; 64;58soit 31 patients ont une splenomegalie palustre stade III ou plus. La population active (15 - 55 ans) est la plus touchee. Par ordre de frequence decroissante; les indications d'ordre medical de la splenectomie etaient l'hypersplenisme; la rupture traumatique et la compression. Mais nous avons indique systematiquement la splenectomie pour toute splenomegalie stade IV et V meme en l'absence d'indication d'ordre medical pour des raisons bien analysees adaptees aux regions a forte endemicite palustre. La duree moyenne d'hospitalisation etait de 8 jours et les resultats a court et a moyen terme sont favorables. Conclusion: La splenectomie est indiquee systematiquement en cas de splenomegalie palustre enorme de stade IV et V meme sans les complications medicales ou chirurgicales realisant leurs indications habituelles

Non-endoscopic Predictors of Large Esophageal Varices in Patients with Liver Cirrhosis / 대한소화기학회지

BACKGROUND/AIMS: The aim of this study was to identify non-endoscopic predictors for the presence of large esophageal varices in Korean patients with liver cirrhosis. METHODS: Among 736 patients with liver cirrhosis newly diagnosed between the year 2001 and 2005, 245 patients (171 men and 74 women, mean age of 51.9 years) fulfilled the inclusion criteria and underwent EGD as screening tests for esophageal varices. Fifteen variables were analysed to identify the presence of large esophageal varices. RESULTS: Esophageal varices were noted in 186 patients (75.9%) and large varices in 55 patients (22.4%), while 59 patients (24.1%) had no varices at the time of initial diagnosis of cirrhosis. The causes of liver cirrhosis were viral hepatitis (41.2%), chronic alcoholism (42.4%), viral hepatitis/alcoholism (9.8%), and others (6.6%). Fifty-one percent, 35.1% and 13.9% of the patients belonged to Child-Pugh class A, B, and C, respectively. Variables associated with the presence of large esophageal varices on univariate analysis were the presence of ascites, splenomegaly (long-axis > or =12 cm by ultrasound measure), alcoholism, Child-Pugh class, platelet count, prothrombin time, and albumin. On multivariate analysis, alcohol, splenomegaly, and ascites were significantly associated with the presence of large esophageal varices. If the patients have two of them, sensitivity and negative predictive value were 80% and 91.7%, respectively. Patients without all three factors had no large esophageal varices. CONCLUSIONS: These results suggest that patients who have at least two among ascites, splenomegaly, and alcoholism would have an increased risk of having large esophageal varices.

Pyknodysostosis: visceral manifestations and simian crease.

Pyknodysostosis is a rare autosomal recessive osteosclerosing skeletal disorder caused by mutations in the CTSK gene situated at 1q21 that codes for cathepsin K - a lysosomal cysteine protease. Mutations in this gene affect the metabolism of skeletal system. This causes problems in bone resorption and remodelling and craniofacial abnormalities. In this article we report a case of 12 year old female from Punjab with pyknodysostosis having hepatosplenomegaly and simian crease.

Hipertensión portal idiopática con infarto esplénico. Complicación no descripta / Idiopathic portal hypertension with splenic infarct. An unreported complication

La Hipertensión Portal Idiopática (HPI) es un síndrome clínico-patológico caracterizado por hipertensión portal no cirrótica. Su prevalencia es baja en occidente y no hemos encontrado casos comunicados en nuestro país. La patente histológica característica de HPI es fibrosis y obliteración de las venas porta intrajepáticas (flebosclerosis), fibrosis portal y periportal, y formación de vasos aberrantes. Presentamos un caso que nos fue derivado por hepatoesplenomegalia y pancitopenia. Al tercer día de internación presentó dolor abdominal y mediante una TAC abdominal se le diagnosticó un infarto esplénico . Posteriormente se le realizó una esplenectomía y biopsia hepática. Los parámetros hematológicos mejoraron después de la operación. Los estudios de imágenes y endoscópicos confirmaron la presencia de hipertensión portal: circulación colateral, vena porta permeable y dilatada y várices esofágicas. Los cambios histológicos del hígado concordaron con el diagnóstico de HPI. Los signos y síntomas característicos de HPI son hemorragias digestivas relacionadas con la hipertensión portal y pancitopenia secundaria al hiperesplenismo y su principal causa de muerte es la hemorragia por várices esofágicas. Como complicación infrecuente de la HPI se ha descripto la trombosis de la vena Porta. En cambio el infarto esplénico, es una rara complicación de la hipertensión portal por cirrosis y del transplante hepático, pero no se han publicado casos en pacientes con HPI.

Congenital Monoblastic Leukemia with 9;11 Translocation in Monozygotic Twins: A Case Report

We report an autopsy case of congenital monoblastic leukemia that developed in monozygotic twins. The twin presented with progressive hepatosplenomegaly at 4 weeks after birth. One twin died of massive bleeding and hypovolemic shock before the treatment started. At autopsy, the liver was diffusely enlarged and showed a diffuse whitish discoloration except for the subcapsular and perivenular areas. Microscopic examination disclosed infiltration of histiocyte-like atypical cells along the sinusoids and portal areas of the liver. Spleen, lymph nodes and choroid plexus were also infiltrated by the tumor cells. However, bone marrow involvement of the tumor was minimal although multifocal. On immunohistochemical staining, these atypical cells were reactive for CD68 (PGM-1) and lysozyme, suggesting that the tumor cells might have been derived from mono- histiocyte. Cytogenetic study revealed 9;11 translocation, which is frequently associated with acute monoblastic leukemia. To the best of our knowledge, this is the first report of congenital monoblastic leukemia of monozygotic twins in Korea.

Comportamiento de las anemias hemolíticas no inmunes en pediatría / Behavior of the hemolytic anemias with erythrocitical anomalies

Objetivo. Describir el comportamiento de las anemias hemolíticas por anomalías eritrocitarias en pacientes pediátricos. Diseño. Estudio descriptivo, observacional. Población. Treinta pacientes de ambos sexos. Metodología. Se incluyeron todos los pacientes con diagnóstico de anemia hemolítica no autoinmune atendidos en el servicio de Hematología Pediátrica del uno de enero de 1993 al 31 de diciembre de 1998. Se excluyeron todos los pacientes que al diagnóstico correspondieron a una patología distinta a la estudiada. Se incluyeron las variables de sexo, raza, procedencia, residencia, forma de presentación, complicaciones, procedimientos, diagnóstico y tratamiento. Resultados. Se diagnosticaron dos patologías; drepanocitosis en 15 pacientes y esferocitosis en 15 pacientes, con predominio del sexo femenino. El 84 por ciento de los pacientes son de raza no negroide; únicamente un tercio de los pacientes con drepanocitosis son negros. El 50 por ciento de los pacientes se diagnosticaron en sus primeros 3 años de vida y la forma más común de presentación fue ictericia asociada a hepato, espleno o hepatoesplenomegalia. Las dos complicaciones más frecuentes fueron insuficiencia cardíaca congestiva y neumonía. El 77 por ciento de los pacientes con esferocitosis fueron esplenectomisados. Ningún paciente falleció. Conclusiones. En general, la sobrevida y el seguimiento de los pacientes ha sido satisfactoria y no ha habido ningún fallecido

Hanseníase virchowiana em cardiopata chagásico: relato de necropsia / Generalized hanseniasis in Chagas' myocardiopathy: necropsy study

Relata-se caso de hanseníase virchowiana diagnosticada à necropsia, com alterações cardíacas causadas diretamente por micobactérias, em homem de 34 anos portador de miocardiopatia crônica chagásica. Enfatiza-se opossível papel de mediadores inflamatórios na gênese da disfunção miocárdica; bem como a possibilidade de que fatores associados à insuficiência cardíaca, como esplenomegalia congestiva e infartos esplênicos, possam causar imunodepressão, predispondo à infecção generalizada

Sonographic assessment of spleen size in Saudi patients with sickle cell disease

In patients with SCD, the spleen commonly enlarges during the first two decades of life but then undergoes autosplenectomy due to repeated attacks of vaso-occlusion and infarction. This, however, is not the case in Saudi patients with SCD, where splenomegaly sometimes persists into adult life. Ultrasonography was used to evaluate spleen size in 363 Saudi patients with SCD [340 SCD and 23 sickle BETA-thalassemia]. A total of 363 patients were evaluated. Their ages ranged from 1-60 years [mean 16 years]. Only 24 [6.6%] of our patients had autosplenectomy. The splenic index increased with age until about 40 years of age and then gradually decreased, indicating persistence of splenomegaly in our patients into an older age group. Forty-three patients [11.8%] had marked-massive splenomegaly [splenic index >120 cm[2]] and these had higher HbF levels [mean HbF=22.2%] when compared with those who had autosplenectomy [mean HbF=14.6]. This is significant [P-value=0.0169] and confirms the effect of FbF on persistence of splenomegaly in SCD patients. Ultrasonography is a simple, safe and accurate method of assessing splenic size in patients with sickle cell disease. Patients with persistent splenomegaly should be followed closely for development of complications which may necessitate splenectomy

Right sided restrictive cardiomyopathy in Egyptian peasants with hepatosplenic schistosomiasis: a Retrospective 5- year study

Endomyocardial Fibrosis [EMF] is a common disease in the humid zones of tropical Africa- Recognition of cases outside the tropical zone has been reported with increasing Interest. We report our experience with 30 Egyptian peasants who all had infection with schistosoma. Laboratory investigations, 12-lead electrocardiography and pericardiocentesis were performed in all patients. Pericardial biopsy was performed in some patients. All patients had evidence of infection with schistosoma mansoni, schistosomal hepatic fibrosis. splenomegaly and ascites. All patients had markedly congested neck veins with positive kussmaul's sign in some patients. All patients had no eosinophilia. Atrial fibrillation was detected in 24 patients. Detailed echocardiographic study showed obliteration of the right ventricle [RV], apical RV firbrosis, varying degrees of exudative pericardial effusion in all patients. No extension of fibrosis into RV outflow tract. No fibrosis in left side of heart. Hugely dilated right atrium [RA] with RA spontaneous echo contrast detected in all patients and FM thrombi in only 10 patients. Normal size pulmonary artery without pulmonary hypertension. Minimal to mild tricuspid regurgitation was detected in all patients by Doppler echocardiography. In conclusion, right-sided restrictive card iomyopathy is a special form of EMF detected only in Egyptian peasants without eosinophilia. It always, involved the right side of the heart. Calcification extended to the RV outflow tract in some cases

Retinoids medication and schistosomiasis: a potential beneficial use

The present study dealt with the possible beneficial uses of retinoids medication in modulating the adverse effects induced by schistosomiasis in experimental animals. Retinoic acid [RA] and retinyl palmitate [RP] [two chemically related forms of retinoids] were utilized in this study. The selected daily oral doses of these compounds were 75,000 IU/kg and 30 mg/kg body weight for retinyl palimitate and retinoic acid, respectively, being given for a maximum period of 9 weeks. Retinoids medication courses were initiated one week prior to percutaneous exposure of the host to this parasitic infection to explore any modulating influence of these retinoids on the susceptibility of the host to cutaneous penetration by the cercaria and on the degree of severity of the induced bilharzial lesions. Only retinoic acid RA treatment for 9 weeks had caused a significant reduction of schistosomiasis splenomegaly, a delay in the pathologically induced granulomas in the liver. Both retinoids had impaired the metabolic activity of the parasite, this was reflected by a significant decrease in the hematin pigment deposition in the liver. The liver of infected mice treated with retinoids showed absence of necrotic lesions and presence of giant cells especially in case of retinoic acid