[Colorectal cancer in children: report of three cases]. / Cáncer colorrectal en niños: reporte de tres casos.

Colorectal cancer (CRC) is extremely infrequent in children and adolescents. There is little information about this entity, mainly case reports and review articles. We describe three cases of children with poor-differentiated colorectal carcinoma and advanced disease at onset. The presenting symptoms were abdominal pain and constipation, with a median of latency of symptoms of 4-48 months. None of these patients had operable disease at onset; having a disease progression despite therapy in two cases. This study reaffirms poor prognosis of pediatric CRC, probably due to an aggressive tumoral biology and advanced stage at diagnosis. Therapeutic guidelines are based in adult treatment; therefore, efforts should be made to improve tools in early diagnosis and future therapies for a better survival in childhood.

Linfoma plasmablástico: un caso con enfermedad rectal y compromiso de médula ósea en un paciente VIH positivo / Plasmablastic lymphoma: a case of rectal disease with bone marrow involvement in a HIV positive patient

El linfoma plasmablástico es una variante agresiva del linfoma difuso de células grandes B, descrito inicialmente en pacientes VIH positivos asociados a lesiones en cavidad oral. Este corresponde al 2% de LNH asociado a VIH. Esta entidad actualmente representa un reto para el diagnóstico y el tratamiento, presentando o un pobre pronóstico o. El presente reporte describe un paciente con HIV en tratamiento con TARGA, un recuento CD4 en 490 células/ml, asociado a un Linfoma plasmablástico con compromiso rectal y médula ósea. El paciente recibe el régimen EPOCH por 6 ciclos con respuesta completa...

Plasmablastic lymphoma is an aggressive form of lymphoma diffuse large B cell Lymphoma, initially described in HIV positive patients associated with lesions in the oral cavity. It is about 2% of NHL associated with HIV. This entity currently represents a challenge for the diagnosis and treatment, showing a poor long-term prognosis. This report describes a patient with VIH on HAART and CD4 count in 490 cells/ml associated with Plasmablastic lymphoma that involves rectum and bone marrow. The patient received 6 cycles of EPOCH regimen with complete response...

Cáncer colorrectal en niños: reporte de tres casos / Colorectal cancer in children: report of three cases

El cáncer colorrectal (CCR) es extremadamente infrecuente en niños y adolescentes. Existe poca literatura publicada sobre esta entidad, principalmente de reportes de casos y artículos de revisión. Reportamos tres casos de niños con carcinoma de colon de histología poco diferenciada y enfermedad avanzada al momento del diagnóstico. La sintomatología más común fue el dolor abdominal y estreñimiento con una latencia de síntomas de 4 a 48 meses. Ningún paciente fue operable al debut; presentando progresión de enfermedad a pesar del tratamiento en dos casos. Este estudio reafirma el pobre pronóstico del CCR pediátrico, probablemente debido a factores como una biología tumoral agresiva y estadio avanzado al debut. Las directrices terapéuticas están basadas en manejo de adultos, aunque es necesario mejorar las herramientas de diagnóstico y las terapias que incrementen la sobrevida en niños y adolescentes.

Colorectal cancer (CRC) is extremely infrequent in children and adolescents. There is little information about this entity, mainly case reports and review articles. We describe three cases of children with poor-differentiated colorectal carcinoma and advanced disease at onset. The presenting symptoms were abdominal pain and constipation, with a median of latency of symptoms of 4-48 months. None of these patients had operable disease at onset; having a disease progression despite therapy in two cases. This study reaffirms poor prognosis of pediatric CRC, probably due to an aggressive tumoral biology and advanced stage at diagnosis. Therapeutic guidelines are based in adult treatment; therefore, efforts should be made to improve tools in early diagnosis and future therapies for a better survival in childhood.

[Plasmablastic lymphoma: a case of rectal disease with bone marrow involvement in a HIV positive patient]. / Linfoma plasmablástico: un caso con enfermedad rectal y compromiso de médula ósea en un paciente VIH positivo.

Plasmablastic lymphoma is an aggressive form of lymphoma diffuse large B cell Lymphoma, initially described in HIV positive patients associated with lesions in the oral cavity. It is about 2% of NHL associated with HIV. This entity currently represents a challenge for the diagnosis and treatment, showing a poor long-term prognosis. This report describes a patient with VIH on HAART and CD4 count in 490 cells/ml associated with Plasmablastic lymphoma that involves rectum and bone marrow. The patient received 6 cycles of EPOCH regimen with complete response.

[Colonic intussusception by leiomyosarcoma]. / Intususcepción colónica por leiomiosarcoma.

We report the case of female patient, 34 years old, occupation Secretary. BACKGROUND: Polycystic ovary and chronic anemia. No family history of cancer. The patient reported three weeks abdominal pain, fever, bloody loose stools, nausea and vomiting. Abdominal pain is located in flank and right lower quadrant, is colicky, intensity 5 / 10, associated with abdominal distension. On physical examination, vital functions indicated T 38.6 ° FC 98 x min, FR 18 x min, BP 120/80, was awake, she looked pale, in generally fair condition, lucid and oriented. The abdomen was distended, the increased bowel sounds, tympanic to percussion, with tenderness in the right abdomen (more intense in the right iliac fossa), palpable mass is not defined edges lower right quadrant of about 6cm. CLINICAL DIAGNOSIS: abdominal pain syndrome (appendicular mass, intestinal obstruction, intussusceptions). In examinations auxiliars highlighted in 9.1 g of hemoglobin with decreased corpuscular constants. Reviewed by history "rectal bleeding", DRE: yellow stool, no trace of blood, so that colonoscopy was deferred. Reassessed at 24 hours, we decide surgery with a presumptive diagnosis of intestinal obstruction, intussusception. In surgery, we identified a transverse colon tumor (with colo-colonic intussusception) and 10cm of colon was resected and meso tumor: TT anastomosis was performed in transverse colon. The lesion was a tumor of the middle region of the transverse colon, proliferative fibroid appearance, which almost completely obstructed the intestinal lumen, measuring about 7x5cm. The study of pathology with immunohistochemistry indicated that the tumor corresponded to leiomyosarcoma of the colon. Was discharged in good condition. The rarity of this type of malignancy and this type of presentation led us to make this report.

Intususcepción colónica por leiomiosarcoma / Colonic intussusception by leiomyosarcoma

Reportamos el caso de una paciente mujer de 34 años, secretaria, con historia de anemia crónica y ovario poliquístico; sin historia familiar de cáncer. La paciente refería desde tres semanas antes del ingreso: náusea, vómito, dolor abdominal, fiebre y deposiciones sueltas con sangre. El dolor abdominal estaba localizado en flanco y fosa iliaca derecha, era tipo cólico, de intensidad 5/10 y asociado a distensión abdominal. En el examen clínico las funciones vitales indicaban T 38,6 °, FC 98 x min, FR 18 x min y PA 120/80; estaba despierta, lucida, pálida, en regular estado general. El abdomen estaba distendido, los ruidos hidroaéreos aumentados, timpánico a la percusión y con dolor a la palpación en hemiabdomen derecho (más intenso y con rebote positivo en fosa iliaca derecha); se palpaba masa de bordes no definidos en cuadrante inferior derecho, de aproximadamente 6cm. Diagnóstico clínico: Síndrome doloroso abdominal (¿plastrón apendicular, obstrucción intestinal: intususcepción?). En los exámenes auxiliares resaltaba la hemoglobina en 9,1 gr, con las constantes corpusculares disminuidas. El tacto rectal fue negativo, por lo que la colonoscopia fue diferida. Reevaluada a las 24 horas se decide cirugía. Se identificó una tumoración de colon transverso (con intususcepción colo-colónica), se resecó 10cm de colon y meso de tumoración; se realizó anastomosis T-T de colon transverso. La lesión era una tumoración proliferativa de 7x5 cm, que obstruía la luz del intestino casi totalmente. El estudio de anatomía patológica con inmunohistoquímica indicó que la lesión invaginada correspondía a un leiomiosarcoma de colon. Salió de alta en buenas condiciones. La rareza de éste tipo de neoplasia maligna de colon y lo esporádico de este tipo de presentación, nos indujo a realizar el presente reporte.

We report the case of female patient, 34 years old, occupation Secretary. Background: Polycystic ovary and chronic anemia. No family history of cancer. The patient reported three weeks abdominal pain, fever, bloody loose stools, nausea and vomiting. Abdominal pain is located in flank and right lower quadrant, is colicky, intensity 5 / 10, associated with abdominal distension. On physical examination, vital functions indicated T 38.6 ° FC 98 x min, FR 18 x min, BP 120/80, was awake, she looked pale, in generally fair condition, lucid and oriented. The abdomen was distended, the increased bowel sounds, tympanic to percussion, with tenderness in the right abdomen (more intense in the right iliac fossa), palpable mass is not defined edges lower right quadrant of about 6cm. Clinical diagnosis: abdominal pain syndrome (appendicular mass, intestinal obstruction, intussusceptions). In examinations auxiliars highlighted in 9.1 g of hemoglobin with decreased corpuscular constants. Reviewed by history “rectal bleeding”, DRE: yellow stool, no trace of blood, so that colonoscopy was deferred. Reassessed at 24 hours, we decide surgery with a presumptive diagnosis of intestinal obstruction, intussusception. In surgery, we identified a transverse colon tumor (with colo-colonic intussusception) and 10cm of colon was resected and meso tumor: TT anastomosis was performed in transverse colon. The lesion was a tumor of the middle region of the transverse colon, proliferative fibroid appearance, which almost completely obstructed the intestinal lumen, measuring about 7x5cm. The study of pathology with immunohistochemistry indicated that the tumor corresponded to leiomyosarcoma of the colon. Was discharged in good condition. The rarity of this type of malignancy and this type of presentation led us to make this report.

[Plummer-Vinson syndrome: report of a case and review of literature]. / Síndrome de Plummer-Vinson: reporte de caso y revisión de la literatura.

A 39-year-old woman was admitted to our hospital with an eight-month history of dyspnea on exertion, weakness and increasing fatigue. She reported repeated episodes of menometrorrhagia and underwent a myomectomy. She is not a vegetarian. Her menstrual bleeding: 3-5 days per month. Two months ago, she complained of burning sensation of the tongue upon swallowing food and noted brittle nails. She tolerated soft foods. On physical examination, she was pale; her nails were very thin, fragile and somewhat concave. Her oral examination showed angular stomatitis, depapillated tongue and glossitis. The clinical diagnosis was anemia and dysphagia. Laboratory tests were: Hb: 7.0g/dL, MCV: 57.42fL, MCH: 15.82 pg; leukocytes: 4,980; reticulocytes: 2.18%, reticulocyte index: 0.1%, serum iron: 21ug/dl, total iron binding capacity (TIBC): 286, transferrin saturation: 7% and serum ferritin: 27ng/ml. The peripheral blood smear showed anisocytosis and hypochromic microcytic cells. Thevenon test was negative. Abdominal ultrasound: uterine myoma. A barium swallow X-ray showed a 2-mm linear filling defect between the 4th and 5th cervical vertebrae in the anteroposterior and lateral view; it protruded from the anterior wall and reduced esophageal lumen by 60%. In the endoscopy, we found a fibrous web in the cricopharyngeal area. Serial dilatations were performed over a guidewire using Savary-Gilliard dilators with diameter up to 14 mm, improving dysphagia. She was treated with transfusional therapy and parenteral iron. She was discharged with ferrous sulfate and folic acid. The Plummer-Vinson syndrome, Paterson-Brown-Kelly or sideropenic dysphagia is characterized by dysphagia, irondeficiency anemia and upper esophageal web. The syndrome is described as very rare.

Tumores estromales gastrointestinales: evaluación clinicopatológica y sobrevida en el Hospital Rebagliati / Gastrointestinal stromal tumors: clinicopathologic and survival evaluation in Rebagliati Hospital

OBJETIVO: Determinar las manifestaciones clínicas, radiológicas, histopatológicas y sobrevida de los pacientes con Tumor Estromal Gastrointestinal (GIST) en el Hospital Nacional Edgardo Rebagliati Martins (HNERM), de Lima, Perú. MATERIAL Y MÉTODOS: El presente es un estudio descriptivo, retrospectivo. El cual se realizó a partir de las historias clínicas de 103 pacientes con GIST confirmado por inmunohistoquímica que fueron evaluados y tratados en el HNERM, desde Enero del 2002 hasta Diciembre de 2010. RESULTADOS: En los 103 pacientes el promedio de edad fue 64 años (entre 30 y 88 años). Predominó en mujeres (52%). El tiempo de enfermedad promedio fue 7 meses. La forma de presentación más frecuente fue sangrado digestivo (48.3%). El diagnóstico se hizo más por endoscopía (50.5%). La prevalencia por órganos fue más frecuente en estómago 56.3%. El promedio de tamaño fue 98mm, (49% entre 50mm y 100mm), el tumor de mayor tamaño alcanzaba 260 mm. El estadio tumoral más frecuente fue localizado (70.9%). Presentaron otro cáncer asociado el 9.7% de pacientes El patrón histológico predominante fue fusiforme 73.8%. El 84.5% tuvo bajo índice mitótico. La inmunohistoquímica mostró la expresión de KIT (CD 117) 94.17%, CD 34 77.5%, Vimentina 96.6%, NSE 84.9%, Alfa actina 52.7%, CD56 44.4%, S-100 32.3% y Actina 20.0%. La característica tomográfica más frecuente fue tumor heterogéneo (43.6%). La distribución según clasificación de riesgo fue: muy bajo riesgo 3.9%, bajo riesgo 28.2%, riesgo intermedio 37.7% y alto riesgo 30.1%. La resección quirúrgica completa se realizó en 87.4% de pacientes, 4.9% de pacientes recibió Imatinib. La sobrevida global acumulada a 5 años fue 31.07%. En el análisis bivariado se encontró asociación estadística entre el haber sobrevivido con: ausencia de cáncer asociado p= 0.004, CD 34 p=0.01, índice mitótico bajo p=0.00 y tratamiento quirúrgico recibido p= 0.000. En el análisis multivariado se encontró asociación estadística de mayor sobrevida con: los de menor tamaño del tumor p=0.015 (IC -3.67, -0.41), estadio tumoral localizado p=0.036 (IC -5.83, -0.19), menor índice mitótico p=0.038 (IC -0.86, 0.02), paciente asintomático p=0.009 (IC 1.25, 8.62), no recidiva del tumor p=0.01 (IC -8.49, -1.17) y el no presentar metástasis p=0.001 (IC 2.66, 10.62). CONCLUSIONES: Los resultados de nuestro estudio fueron similares a lo que reporta la literatura internacional. Los factores que se asociaron a mayor sobrevida fueron: haber recibido tratamiento quirúrgico, pacientes con menor tamaño tumoral, estadio tumoral localizado, índice mitótico bajo, paciente asintomático, no recidiva del tumor, no metástasis y no cáncer asociado.

OBJECTIVE: To determine the clinical, radiological, histopathological manifestations and survival of patients with gastrointestinal stromal tumor (GIST)in the National Hospital Edgardo Rebagliati Martins (HNERM) from Lima, Perú. MATERIAL AND METHODS: This is a descriptive and retrospective study, which was based on the medical records of 103 patients with confirmed GIST with immunohistochemical. All the patients were evaluated and treated at the HNERM, from January 2002 until December 2010. RESULTS: In 103 patients between 30 and 88 years the average age was 64 years. The tumor was more frequent in females (52%). The mean disease duration was 7 months. The most frequent form of presentation was gastrointestinal bleeding (48.3%). The diagnosis was made more by endoscopy (50.5%). The prevalence of GISTs by organs was more frequent in stomach (56.3%). The average size of the tumors was 98mm, 49% had a size between 50mm and 100mm, the largest tumor was 260 mm. Tumor stage more frequent was localized (70:9%). GIST associated with another cancer was 9.7% of patients. The predominant histologic pattern was fusiform (73.8%). The 84.5% had low mitotic index. Immunohistochemistry showed expression KIT (CD 117) was 94.17%, CD34 77.5%, Vimentin 96.6%, NSE 84.9%, alpha actin 52.7%, CD56 44.4%, S-100 32.3% and Actin 20%. The tomographic characteristic more frequent was heterogeneous tumor (43.6%).The distribution according to risk classification was: very low risk 3.9%, low risk 28.2, intermediate risk 37.7% and high risk 30.1%. Complete surgical resection was performed in 87.4% of patients and 4.9% of patients received imatinib. The cumulative overall survival at 5 years was 31.07%. In bivariate analysis statistical association was found between surviving with: no presence of cancer associated p = 0.004, CD 34 p = 0.01, low mitotic index p = 0.00 and received surgical treatment p = 0.000. In multivariate analysis one found statistical association of longer survival with smaller tumor size p = 0.015 (CI -3.67, -0.41), localized tumor stage p = 0.036 (CI -5.83, -0.19), lower mitotic index p = 0.038 (CI -0.86, 0.02), asymptomatic patient p=0.009 (CI 1.25, 8.62), no tumor recurrence p = 0.01 (CI -8.49, -1.17), and no metastasis p = 0.001 (CI 2.66, 10.62). CONCLUSIONS: The results of our study were similar to what was reported in international literature. Factors that were associated with longer survival were receiving surgical treatment, patients with smaller tumor size, tumor stage localized, low mitotic index, asymptomatic patient, not tumor recurrence, not metastasis and no cancer associated.

Síndrome de Plummer-Vinson: reporte de caso y revisión de literatura / Plummer-Vinson Syndrome: case report and review of literature

Presentamos a una mujer de 39 años que acude a nuestro hospital por disnea de esfuerzo, adinamia y cansancio progresivo desde hace 8 meses. Antecedente quirúrgico de miomectomia por menometrorragias frecuentes. Niega ser vegetariana. Régimen catamenial: 3-5 días cada mes. Hace 2 meses se añade ôardorõ en lengua al comer. Nota uñas quebradizas. Toleraba alimentos blandos. Al examen físico había moderada palidez; las uñas eran muy delgadas, frágiles y algo cóncavas. En la boca se encontró estomatitis angular, lengua depapilada y glositis. El diagnóstico clínico era síndrome anémico y disfagia. Exámenes auxiliares: Hb: 7.0g/dL; VCM: 57.42fL; HCM: 15.82pg; leucocitos: 4,980; reticulocitos: 2.18%, índice reticulocitario: 0.1%, hierro sérico: 21ug/dl, transferrina (TIBC): 286, saturación de transferrina: 7%, ferritina sérica: 27ng/ml. La lámina periférica demostró anisocitosis, hipocromía y microcitosis. Thevenon en heces negativo. Ecografía abdominal: mioma uterino. La radiografía esofágica con bario demostró una imagen lineal por defecto de relleno de 2 mm a altura entre vértebras C4 y C5 en vista anteroposterior y lateral; se extendía a cara anterior y reducía la luz esofágica en 60%. Durante la endoscopía, evidenciamos una membrana fibrosa estenosante en la región cricofaríngea. Multiples dilataciones fueron realizadas progresivamente mediante una guía con bujías dilatadoras Savary-Gilliard hasta 14 mm, mejorando la disfagia. Recibió terapia transfusional y hierro parenteral. Salió de alta con sulfato ferroso y ácido fólico. El síndrome de Plummer-Vinson, Paterson-Brown-Kelly o disfagia sideropénica es definido por disfagia, anemia ferropénica y membrana esofágica alta. El síndrome es descrito como muy raro.

A 39-year-old woman was admitted to our hospital with an eight-month history of dyspnea on exertion, weakness and increasing fatigue. She reported repeated episodes of menome trorrhsgis snd underwent a myomectomy. She is not a vegetarian. Her menstrual bleeding: 3-5 days per month. Two months ago, she complained of burning sensation of the tonge upon swallowing food and noted brittle nails. She tolerated soft foods. On physical examination, she was pale; her nails were very thin, fragile and somewhat concave. Her oral examination showed angular stomatitis, depapillated togue and glossitis. The clinical diagnosis was anemia and dysphagia. Laboratory tests were: Hb: 7.0g/dL, MCV: 57.42fL, MCH: 15.82 pg; leukocytes:4,980; reticulocytes:2.18%, reticulocyte index:0.1%, serum iron:21ug/dl, total iron binding capacity (TIBC):286, transferrin saturation: 7% and serum ferritin: 27ng/ml. The peripheral blood smear showed anisocytosis and hypochromic microcytic cells. Thevenon test was negative. Abdominal ultrasound: uterine myoma. A barium swallow X-ray showed a 2-mm linear filling defect between the 4th and 5th cervical vertebrae in the anteroposterior and lateral view; it protruded from the anterior wall and reduced esophageal lumen by 60%. In the endoscopy, we found a fibrous web in the cricopharyngeal area. Serial dilatations were performed over a guidewire using Savary-Gilliard dilators with diameter up to 14 mm, improving dysphagia. She was treated with transfusional therapy and parenteral iron. She was discharged with ferrous sulfate and folic acid. The Plummer-Vinson syndrome, Paterson-Brown-Kelly or sideropenic dysphagia is characterized by dysphagia, iron-deficiency anemia and upper esophaegal web. The syndrome is described as very rare.

[Spleen infarction and S hemoglobinopathies S in the high altitude lands]. / Infarto de bazo y hemoglobinopatía S en la altura.

The hemoglobin S is a consequence of the substitution of valine for glutamic acid at position 6 of beta globin chain. The problem arises when some individuals with Hb S is moved to the mountains and exposed to hypoxia. The decrease in oxygen saturation distorts the red blood cell with HbS-shaped crescent (sickle cell). Sickle cell (rigid and fragile) tends to adhere to the other red blood cells, generating a series of intravascular alterations that can lead to tissue ischemia or infarction. The spleen by type of movement and lack of lateral communications between the branches of the splenic artery was the most susceptible to sickle cell crisis. Splenic infarction at altitude corresponding to different circumstances can evolve in three stages: a) Acute (focal, uncomplicated), b) massive attack (more than 50% of parenchyma) and c) spontaneous rupture.Early diagnosis is crucial, allowing the quick and timely introduction of various measures, including adequate hydration and oxygenation continues until its evacuation to lower altitude locations. These measures would reduce the phenomenon of sickle and some patients may overcome this acute trance without major complications. The delay in diagnosis leads to action that can exacerbate tissue hypoxia and cause ischemia or infarction of various organs. A large population of black and mixed race of African descent living in the Peruvian coast, 10% and 2% respectively have hemoglobin S; Caucasian subjects with Mediterranean ancestry this hemoglobin also can carry. It is therefore essential to disseminate within the clinicians working in regions of high status and to thus prevent potentially fatal complications in patients with Hb S; is also essential to promote preventive measures for individuals with African or Mediterranean ancestry know their sickle cell status before traveling to places above 2,500 m.

Infarto de bazo y hemoglobinopatía S en la altura / Spleen infarction and hemoglobin S in height

La hemoglobinopatía S es un desorden hereditario resultado de una mutación en el gen beta-S que se expresa con la sustitución de un aminoácido en la cadena beta de globina. El problema se presenta cuando algún sujeto con hemoglobinopatía S se expone a la hipoxia de altura. La disminución de la saturación de oxígeno forma polímeros de Hb S que deforman al glóbulo rojo en forma de ômedia lunaõ (célula falciforme o drepanocito). Las células falciformes (rigidas y frágiles) tienden a adherirse a otros glóbulos rojos incrementando la viscosidad y estásis sanguínea, generando oclusión vascular e infarto en los tejidos. El bazo por su tipo de circulación es un órgano susceptible de la crisis falciforme. El infarto esplénico en la altura -en correspondencia a diferentes circunstancias- puede evolucionar en tres etapas: a) Infarto agudo (focal, no complicado), b) infarto masivo (compromiso de mas del 50% del parénquima) y c) infarto con disrupción capsular. El diagnóstico precoz es fundamental, permite la instauración oportuna de diversas medidas, especialmente una adecuada hidratación, oxigenación y rápida evacuación a localidades de menor altitud. Con estas medidas se atenua el fenómeno falciforme y algunos pacientes pueden superar este trance sin mayores complicaciones. El retardo diagnostico conlleva a tomar medidas que incluso pueden exacerbar la hipoxia tisular. Importantes poblaciones de raza negra y mestiza con ancestro africano viven en la costa peruana, 10% y 2% respectivamente tienen hemoglobinopatía S; sujetos de raza blanca con ancestro mediterráneo también pueden portar esta hemoglobina. Es indispensable difundir el conocimiento de esta entidad para que la tengan presente los médicos que laboran en regiones de altura; asimismo es primordial impulsar medidas preventivas para que los individuos con ancestro africano o mediterráneo conozcan su estatus sickle cell antes de viajar a lugares por encima de 2.500 m.

The hemoglobin S is a consequence of the substitution of valine for glutamic acid at position 6 of beta globin chain. The problem arises when some individuals with Hb S is moved to the mountains and exposed to hypoxia. The decrease in oxygen saturation distorts the red blood cell with HbS-shaped crescent (sickle cell). Sickle cell (rigid and fragile) tends to adhere to the other red blood cells, generating a series of intravascular alterations that can lead to tissue ischemia or infarction. The spleen by type of movement and lack of lateral communications between the branches of the splenic artery was the most susceptible to sickle cell crisis. Splenic infarction at altitude corresponding to different circumstances can evolve in three stages: a) Acute (focal, uncomplicated), b) massive attack (more than 50% of parenchyma) and c) spontaneous rupture. Early diagnosis is crucial, allowing the quick and timely introduction of various measures, including adequate hydration and oxygenation continues until its evacuation to lower altitude locations. These measures would reduce the phenomenon of sickle and some patients may overcome this acute trance without major complications. The delay in diagnosis leads to action that can exacerbate tissue hypoxia and cause ischemia or infarction of various organs. A large population of black and mixed race of African descent living in the Peruvian coast, 10% and 2% respectively have hemoglobin S; Caucasian subjects with Mediterranean ancestry this hemoglobin also can carry. It is therefore essential to disseminate within the clinicians working in regions of high status and to thus prevent potentially fatal complications in patients with Hb S; is also essential to promote preventive measures for individuals with African or Mediterranean ancestry know their sickle cell status before traveling to places above 2,500 m.

[Gastrointestinal stromal tumors: clinicopathologic and survival evaluation in Rebagliati Hospital]. / Tumores estromales gastrointestinales: evaluación clinicopatológica y sobrevida en el Hospital Rebagliati.

OBJECTIVE: To determine the clinical, radiological, histopathological manifestations and survival of patients with gastrointestinal stromal tumor (GIST) in the National Hospital Edgardo Rebagliati Martins (HNERM) from Lima, Perú. MATERIAL AND METHODS: This is a descriptive and retrospective study, which was based on the medical records of 103 patients with confirmed GIST with immunohistochemical. All the patients were evaluated and treated at the HNERM, from January 2002 until December 2010. RESULTS: In 103 patients between 30 and 88 years the average age was 64 years. The tumor was more frequent in females (52%). The mean disease duration was 7 months. The most frequent form of presentation was gastrointestinal bleeding (48.3%). The diagnosis was made more by endoscopy (50.5%). The prevalence of GISTs by organs was more frequent in stomach (56.3%). The average size of the tumors was 98 mm, 49% had a size between 50 mm and 100 mm, the largest tumor was 260 mm. Tumor stage more frequent was localized (70:9%). GIST associated with another cancer was 9.7% of patients. The predominant histologic pattern was fusiform (73.8%). The 84.5% had low mitotic index. Immunohistochemistry showed expression KIT (CD 117) was 94.17%, CD34 77.5%, Vimentin 96.6%, NSE 84.9%, alpha actin 52.7%, CD56 44.4%, S-100 32.3% and Actin 20%. The tomographic characteristic more frequent was heterogeneous tumor (43.6%).The distribution according to risk classification was: very low risk 3.9%, low risk 28.2, intermediate risk 37.7% and high risk 30.1%. Complete surgical resection was performed in 87.4% of patients and 4.9% of patients received imatinib. The cumulative overall survival at 5 years was 31.07%. In bivariate analysis statistical association was found between surviving with: no presence of cancer associated p = 0.004, CD 34 p = 0.01, low mitotic index p = 0.00 and received surgical treatment p = 0.000. In multivariate analysis one found statistical association of longer survival with smaller tumor size p = 0.015 (CI -3.67, -0.41), localized tumor stage p = 0.036 (CI -5.83, -0.19), lower mitotic index p = 0.038 (CI -0.86, 0.02), asymptomatic patient p=0.009 (CI 1.25, 8.62), no tumor recurrence p = 0.01 (CI -8.49, -1.17), and no metastasis p = 0.001 (CI 2.66, 10.62). CONCLUSIONS: The results of our study were similar to what was reported in international literature. Factors that were associated with longer survival were receiving surgical treatment, patients with smaller tumor size, tumor stage localized, low mitotic index, asymptomatic patient, not tumor recurrence, not metastasis and no cancer associated.

[Jejunal GIST with obscure gastrointestinal bleeding]. / GIST yeyunal con sangrado digestivo de origen oscuro.

We report the case of a woman of 84 years with a history of cardiac arrhythmia and hemorrhoids. She had multiple hospitalizations and transfusions for symptomatic iron deficiency anemia, endoscopic studies showed only small diverticula and colon polyps. He was later hospitalized with bloody stools red wines, upper endoscopy and colonoscopy showed gastritis, small colonic ulcers, colonic polyp and multiple diverticula. Readmitted with bleeding of obscure origin, on that occasion showed gastritis, antral erosions, small ulcers, colon polyps and colon ulcers in the process of healing, capsule endoscopy showed angiodysplasia in jejunum, anterograde enteroscopy detected some erythematous lesions in proximal jejunum without evidence of bleeding. Again hospitalized for melena and abdominal.

[Celiac disease and intestinal obstruction by T cell lymphoma]. / Enfermedad celiaca y obstrucción intestinal por linfoma de células T.

A male patient, 55 years old, born in Ayacucho, with Spanish ancestors, was hospitalized through emergency referring abdominal pain, and 10 kilograms weight loss. Six months before he was diagnosed as having irritable bowel syndrome. His previous diseases were rheumatoid arthritis and intolerance to lactose. Laboratory results were: Hb 12 gr./dL, white cells 5200 per mm3, albumin 2.7 gr./dL, erythrocyte sedimentation rate 32 mm/hr., and tumor markers were negative. Radiographic study of the small bowel showed barium fragmentation, and a focal dilation in distal jejunum. Chest X-ray and CT scan of thorax, abdomen and pelvis were normal. Colonoscopy was normal for colonic mucosa, but in ileum it showed an irregular mucosa, little nodules and fewer folds than usual. Biopsy from ileum demonstrated unspecific inflammation. Upper endoscopy showed gastritis, a duodenum scar ulcer and an irregular mosaic pattern pink and white. Duodenum biopsy demonstrated short villi, chronic inflammation and an increase in the number of intraepithelial lymphocytes, all these was consistent with celiac disease Marsh 3. Antibodies anti-endomisium and anti-transglutaminase were positive. After some days he developed signs of bowel obstruction and was operated.

[Acute pancreatitis and acalculous cholecystitis associated with viral hepatitis A]. / Pancreatitis y colecistitis alitiásica agudas asociadas a hepatitis viral A.

We report the case of a 14 year-old male from Lima. He is a student with a history of bronchial asthma since age 4 receives conditional salbutamol, corticosteroids used for asthma attacks (a crisis in 2010, 1 month ago) Refuses surgery or transfusions. He presented with a two weeks for abdominal pain, nausea, fever, and jaundice. Epigastric pain is colicky and radiated back to righ upper quadrant, refers in addition to nausea and fever, for ten days notice jaundice of skin and sclera. On examen he was lucid, with jaundice of skin and mucous membranes. There was no palpable lymph nodes, abdomen with bowel sounds, soft, depressible, liver span of 15cm, positive Murphy, no peritonitis. The laboratory findings showed hemoglobin 13gr, MCV 90, platelets 461.000/mm3, WBC 4320/mm, lymphocytes 1700 (39%). total bilirubin: 8.8, B Direct: 7.6, ALT (alanine aminotransferase): 3016, AST (aspartate aminotransferase): 984, alkaline phosphatase: 250, albumin: 3.34gr%, globulin: 2.8, amylase: 589 (high serum amylase), TP: 17, INR: 1.6, VHA IgM positive. 89 mg glucose, urea 19 mg%, creatinine 0.5 mg Hemoglobin 13gr, MCV 90 Platelet 461000/mm3, WBC 4320/mm, Lymphocytes 1700 (39%). The nuclear magnetic resonance showed hepatomegaly associated with thickening of gallbladder wall without stones up to 11mm inside. No bile duct dilatation, bile duct 4mm, pancreas increased prevalence of body size. Mild splenomegaly and free fluid in the space of Morrison and right flank. Abdominal ultrasound revealed a gallbladder wall thickness (11mm), without stones in his light. Pancreas to increase volume with peripancreatic fluid free perivesicular with a volume of 430 cc. Findings consistent with acute acalculous cholecystitis and acute pancreatitis. CT-scan showed enlarged pancreas with predominance of body and tail with peripancreatic edema; the gallbladder was thickening. We report this case because the extrahepatic manifestations of viral hepatitis A infection are uncommon, specially the associated with acute acalculous cholecystitis and acute pancreatitis simultaneous.

Enfermedad Celiaca y Obstrucción Intestinal por Linfoma de Células T / Celiac disease and intestinal obstruction by T cell lymphoma

Paciente varón de 55 años, raza blanca (ascendencia española), natural y procedente de Ayacucho, que ingresó por una enfermedad de seis meses de evolución, caracterizada por dolor abdominal tipo cólico y baja ponderal de 10 Kg. Había estado hospitalizado seis meses antes y dado de alta con el diagnóstico de síndrome de intestino irritable. Entre sus antecedentes refería intolerancia a la lactosa y artritis reumatoidea. Los exámenes mostraron: Hb:12 g/dl, leucocitos: 5260 cel/mm, abastonados: 11%, albúmina: 2,7 mg/ml y VSG: 33mm/h. El resto de exámenes -incluyendo los marcadores tumorales- fueron normales. El tránsito gastrointestinal mostraba las asas delgadas con fragmentación del bario y dilatación focal moderada de un asa yeyunal distal. La tomografía de tórax, abdomen y pelvis sin alteraciones; Rx tórax normal. La colonoscopia fue normal; el íleon tenía pocos pliegues y pequeñas nodulaciones, las biopsias indicaron "ileitis inespecífica". La endoscopia mostró gastritis y una cicatriz de úlcera duodenal; la mucosa duodenal mostraba áreas con aspecto de mosaico rosado-blanquecino. La biopsia duodenal evidenció acortamiento de vellosidades, infiltrado inflamatorio crónico e incremento de linfocitos intraepiteliales, hallazgos compatibles con los criterios de celiaquía Marsh-tipo 3. Los anticuerpos IgA antiendomisio y antitransglutaminasa tisular estaban incrementados. Durante su hospitalización aumentó el dolor y aparecieron signos de obstrucción. En la laparotomía se encontraron una tumoración yeyunal estenosante y una perforación adyacente. El espécimen mostró un linfoma intestinal de células T. Se ha demostrado que existen más celiacos subclínicos que celiacos con esprue clásico; el conocimiento de esta situación nos debe llevar a tenerla presente por sus complicaciones o asociaciones, una de las cuales es el linfoma primario intestinal.

A male patient, 55 years old, born in Ayacucho, with Spanish ancestors, was hospitalized through emergency referring abdominal pain, and 10 kilograms weight loss. Six months before he was diagnosed as having irritable bowel syndrome. His previous diseases were rheumatoid arthritis and intolerance to lactose. Laboratory results were: Hb 12 gr./dL, white cells 5200 per mm3, albumin 2.7 gr./dL, erythrocyte sedimentation rate 32 mm/hr., and tumor markers were negative. Radiographic study of the small bowel showed barium fragmentation, and a focal dilation in distal jejunum. Chest X-ray and CT scan of thorax, abdomen and pelvis were normal. Colonoscopy was normal for colonic mucosa, but in ileum it showed an irregular mucosa, little nodules and fewer folds than usual. Biopsy from ileum demonstrated unspecific inflammation. Upper endoscopy showed gastritis, a duodenum scar ulcer and an irregular mosaic pattern pink and white. Duodenum biopsy demonstrated short villi, chronic inflammation and an increase in the number of intraepithelial lymphocytes, all these was consistent with celiac disease Marsh 3. Antibodies anti-endomisium and anti-transglutaminase were positive. After some days he developed signs of bowel obstruction and was operated. A tumor was found in jejunum with a bowel perforation. Pathological study showed a small bowel T-cell lymphoma. Fortunately this patient did well, and was sent home to continue treatment on ambulatory basis. Celiac disease is more common than what is thought, and it has been demonstrated that there are more persons with subclinical celiac disease, than those with the typical clinical pattern. It is necessary to be aware of this disease to improve diagnosis in order to avoid late complications as small bowel lymphoma.

GIST Yeyunal con Sangrado Digestivo de Origen Oscuro / Jejunal GIST with obscure gastrointestinal bleeding

Reportamos el caso de una mujer de 84 años con antecedentes de arritmia cardiaca y hemorroides. Tenía múltiples hospitalizaciones y transfusiones por anemia ferropénica sintomática; los estudios endoscópicos solo demostraron divertículos y pequeños pòlipos de colon. Posteriormente fue hospitalizada por presentar heces sanguinolentas de color rojo vinoso; la endoscopia alta indicó gastritis y la colonoscopia mostró pequeñas úlceras colónicas, un pólipo colónico y múltiples divertículos. Meses después, reingresó con hemorragia de origen oscuro; en esa ocasión se demostraron: gastritis, erosiones antrales, pequeños pólipos colónicos y úlceras colónicas en vías de cicatrización; la cápsula endoscópica mostró probable angiodisplasia en yeyuno medio, la enteroscopia anterógrada detectó en yeyuno proximal algunas lesiones eritematosas sin evidencia de sangrado activo. Volvió a ser hospitalizada por melena y dolor abdominal, la endoscopia alta mostró angiodisplasias gástricas y duodenales que fueron tratadas. El último ingreso indicó un tiempo de enfermedad de dos años, el episodio se caracterizó por presentar deposiciones rojo vinosas y anemia. La endoscopia mostró angiodisplasia gástrica, que fue tratada con termocoagulación con argón plasma. En el examen no presentó signos de descompensación hipovolémica. Enfocado como un problema de hemorragia digestiva de origen oscuro se repitieron varios exámenes endoscópicos sin resultados. Resangró estando hospitalizada, se realizó cápsula endoscópica que demostró sangrado agudo en yeyuno, se complementó con nueva enteroscopia anterógrada que mostró lesiones ulceradas de yeyuno, se marcó el área con tinta china y se indicó laparotomía exploratoria. En la intervención quirúrgica se encontró en borde antimesentérico de yeyuno, una tumoración redondeada (6x6x4.5 cm) de crecimiento extraluminal, que comprometía la pared sin adherirse a otras estructuras; se realizó resección del tumor y anastomosis yeyuno-yeyunal. El estudio histológico -con inmunohistoquímica- del espécimen mostró que se trataba de un Tumor Estromal Intestinal (GIST), de riesgo intermedio, patrón histológico fusiforme, con escasas mitosis, dependiente de la capa muscular propia del intestino delgado. En conclusión el presente caso se trata de una mujer con un GIST yeyunal cuya presentación clínica fue una hemorragia de origen oscuro que constituyó un problema diagnóstico y que gracias al advenimiento de los nuevos procedimientos endoscópicos (enteroscopia y cápsula endoscópica) fue localizada y posteriormente extirpada quirúrgicamente.

We report the case of a woman of 84 years with a history of cardiac arrhythmia and hemorrhoids. She had multiple hospitalizations and transfusions for symptomatic iron deficiency anemia, endoscopic studies showed only small diverticula and colon polyps. He was later hospitalized with bloody stools red wines, upper endoscopy and colonoscopy showed gastritis, small colonic ulcers, colonic polyp and multiple diverticula. Readmitted with bleeding of obscure origin, on that occasion showed gastritis, antral erosions, small ulcers, colon polyps and colon ulcers in the process of healing, capsule endoscopy showed angiodysplasia in jejunum, anterograde enteroscopy detected some erythematous lesions in proximal jejunum without evidence of bleeding. Again hospitalized for melena and abdominal pain, upper endoscopy revealed gastric and duodenal angiodysplasia were treated. The last entry indicated a time of two years disease, the current episode with wine-red colored stools, Hb: 8.4 g, for which he received two units of PG. Endoscopy showed gastric angiodysplasia, which was treated with thermocoagulation (argon plasma). In the entrance examination showed no signs of hypovolaemic decompensation. Approached as a problem of obscure gastrointestinal bleeding were repeated several endoscopic examinations without results. She re-bled being hospitalized, capsule endoscopy was performed showing acute bleeding in the jejunum, complemented by new anterograde enteroscopy that showed ulcerated lesions of the jejunum, the area was marked with indian ink. Exploratory laparotomy was indicated. In the surgical intervention it was in edge antimesentérico of yeyuno, a round tumor (6x6 cm) of extraluminal growth, which compromised the wall without sticking to other structure, Resection of the tumor and jejuno-jejunal anastomosis was realized. The histological study with immunohistochemistry showed an Intestinal Stromal Tumor (GIST), intermediate risk, histological pattern fusiform, with scarce mitosis; the lesion was dependent on the muscularis propria of the small intestine. In conclusion, this case involves a woman with a jejunal GIST whose clinical presentation was hemorrhage of unknown origin which was a diagnostic problem and thanks to the advent of new endoscopic procedures (enteroscopy and capsule endoscopy) could locate the place of injury and subsequent surgery.

Pancreatitis y colecistitis alitiásica agudas asociadas a hepatitis viral A / Acute pancreatitis and acalculous cholecystitis associated with viral hepatitis A

Presentamos el caso de un paciente varón de 14 años, natural y procedente de Lima, estudiante, con antecedente de asma bronquial: desde los 4 años recibe salbutamol condicional y usa corticoides durante sus crisis (una crisis el 2010, hace un mes). Niega cirugía o transfusiones. Refiere desde hace dos semanas dolor epigástrico tipo cólico irradiado hipocondrio derecho y espalda; refiere además náusea y fiebre; diez días antes de su ingreso presenta ictericia de piel y escleras. Al examen físico el paciente estaba lúcido, en regular estado general, con ictericia de piel y mucosas. No se palparon adenopatías; el abdomen tenía ruidos hidroaéreos normales, era blando, depresible, span hepático de 15cm, Murphy positivo, no peritonismo. Los exámenes auxiliares mostraron glucosa 89 mg por ciento, urea 19 mg por ciento, creatinina 0,5 mg por ciento, hemoglobina 13gr por ciento, VCM 90, plaquetas 461.000/mm3, WBC 4320/mm, linfocitos 1700 (39 por ciento). bilirrubina total 8,8 mg/dl, Bilirrubina Directa : 7,6 mg/dl TGP : 3016 U/L TGO : 984 U/L Fosfatasa alcalina 250 U/L , albumina 3,34 gr por ciento, globulina 2,8 gr por ciento , amilasa 589 U/L, Tiempo de protrombina 17 segundos, INR: 1,6 , VHA Ig M positivo, anticuerpos de hepatitis B y C negativos, serología TORCH y virus EB negativos. La colangioresonancia magnética nuclear mostraba hepatomegalia asociada a engrosamiento de la vesícula biliar con pared de hasta 11mm sin litiasis en su interior. No dilatación de vías biliares, colédoco de 4mm, páncreas aumentado de tamaño a predominio de cuerpo. Leve esplenomegalia y líquido libre en el espacio de Morrison y flanco derecho. En la ecografía abdominal resaltaba la vesícula biliar de 83x35mm pared de 9mm sin litiasis en su luz, edema peri portal, páncreas con incremento de su volumen, con líquido libre peripancreatico y perivesicular con volumen de 430cc. Hallazgos consistentes con colecistitis aguda y pancreatitis aguda. La Tomografía axial computarizada mostraba el páncreas aumentado de tamaño, con edema peripancreático.

We report the case of a 14 year-old male from Lima. He is a student with a history of bronchial asthma since age 4 receives conditional salbutamol, corticosteroids used for asthma attacks (a crisis in 2010, 1 month ago) Refuses surgery or transfusions. He presented with a two weeks for abdominal pain, nausea, fever, and jaundice. Epigastric pain is colicky and radiated back to righ upper quadrant, refers in addition to nausea and fever, for ten days notice jaundice of skin and sclera. On examen he was lucid, with jaundice of skin and mucous membranes. There was no palpable lymph nodes, abdomen with bowel sounds, soft, depressible, liver span of 15cm, positive Murphy, no peritonitis. The laboratory findings showed hemoglobin 13gr, MCV 90, platelets 461.000/mm3, WBC 4320/mm, lymphocytes 1700 (39 percent). total bilirubin: 8.8, B Direct: 7.6, ALT (alanine aminotransferase): 3016, AST (aspartate aminotransferase): 984, alkaline phosphatase: 250, albumin: 3.34gr percent, globulin: 2.8, amylase: 589 (high serum amylase), TP: 17, INR: 1.6, VHA IgM positive. 89 mg glucose, urea 19 mg percent, creatinine 0.5 mg Hemoglobin 13gr, MCV 90 Platelet 461000/mm3, WBC 4320/mm, Lymphocytes 1700 (39 percent). The nuclear magnetic resonance showed hepatomegaly associated with thickening of gallbladder wall without stones up to 11mm inside. No bile duct dilatation, bile duct 4mm, pancreas increased prevalence of body size. Mild splenomegaly and free fluid in the space of Morrison and right flank. Abdominal ultrasound revealed a gallbladder wall thickness (11mm), without stones in his light. Pancreas to increase volume with peripancreatic fluid free perivesicular with a volume of 430 cc. Findings consistent with acute acalculous cholecystitis and acute pancreatitis. CT-scan showed enlarged pancreas with predominance of body and tail with peripancreatic edema; the gallbladder was thickening. We report this case because the extrahepatic manifestations of viral hepatitis.

[Frantz tumor: a pancreatic pseudopapillary solid neoplasia]. / Tumor de Frantz: neoplasia sólida pseudopapilar de páncreas.

We report a case of solid and papillary epithelial neoplasm of the pancreas in a 35-year-old female. She presented with epigastric pain and weight loss, these symptoms developed 3 months before. Physical examination revealed mild pain on deep palpation of mesogastrium, without palpable abdominal mass; rest of the examination was normal. The laboratory data showed hemoglobin 13.3 gr/dl; WBC, platelets, blood coagulation test, blood biochemistry, electrolytes, liver function test were normals. Urine test: 14-16 leukocytes per field, urocultive negative, Ca 19.9: 21.2 (0-37). Her serology for hydatic cyst (arc V and inmunoblot) was negative.Abdominal ultrasound showed between body and tail of the pancreas a solid hypoechoic image. In abdominal CT was detected in the body of the pancreas the presence of two hypodense lesions with average density of 25 UH and mediate 50 and 22 mm in diameter. The nuclear magnetic resonance imaging identified the body of the pancreas, bilobed cyst of 45 and 25 mm in diameter, with isointensity in T1, hyperintensity in T2, unchanged after fat saturation, with peripheral contrast enhancement. Operation findings showed a cystic tumor in the body of pancreas without signs of infiltration to other organs. Therefore, distal pancreatectomy and splenectomy were carried out. The tumor was 60 x 46 x 35 mm and a half oval, encapsulated, irregular consistency, which the court left necrotic-looking material flow. The study showed the pattern typical hyalinized perivascular pseudopapillary with stroma, in immunohistochemical studies positive immunoreactivity was observed in cyclin D1 and progesterone receptor, also were positive for vimentin, enolase neuronoespecífica and CD-56. The proliferation index assessed by Ki-67 was less than 1% in tumor cells. The solid pseudopapillary neoplasm of the pancreas is a low-grade malignancy, relatively rare, representing between 0.9 and 2.7% of all pancreatic malignancies. It mainly affects young women (89% of cases), with a mean age at diagnosis of 28 years. Most have specific symptoms related to intra-abdominal mass and more than one third are usually discovered incidentally.

[Obscure digestive bleeding by ileal carcinoid tumor]. / Sangrado digestivo oscuro por tumor carcinoide ileal.

The patient is an 82 year-old female with a history of osteoarthritis, hypothyroidism and anemia for 14 years (receiving blood transfusions). She was admited to our hospital with a nine months history of malaise, anorexia, fatigue and weakness, associated with intermitten episodes of abdominal pain. She was diagnosed anemia and occult blood positive stools. Physical examination revealed a patient in generally fair condition, obese, with mild edema of lower limbs, no changes in the evaluation of chest, cardiovascular, abdomen, etc. Laboratory data was unremarkable, except for iron deficiency anemia. The upper endoscopy showed duodenal ulcer scar, fundic polyposis and chronic gastritis. Colonoscopy revealed some diverticula, a small sessile polyp and internal hemorrhoids. The diagnosis of obscure gastrointestinal bleeding was made. The CT scan of the abdomen showed gallstones and fatty liver; a radiograph of intestinal transit detected a lesion apparently protruded intestinal loop for distal jejunum; enteroscopy was performed (with one team ball) anterograde and retrograde achieving assess distal jejunum and distal ileum without observing any injuries. The study of capsule endoscopy showed a polypoid tumor intestinal with evidence of having bleeding. Surgery detected the tumor in proximal ileum. The surgical specimen findings showed three tumors 0.7 mm, 10 mm and 15 mm on the proximal ileum. The microscopic examination revealed that these lesions were neuroendocrine tumors (carcinoid). The Ileal carcinoid tumor may rarely presented with obscure gastrointestinal bleeding.