Adult Langerhans cell histiocytosis presenting with multisystem involvement and sarcomatoid features: a case report.

BACKGROUND: Langerhans cell tumors are rare clonal disorders characterized by neoplastic proliferation of dendritic cells that can be further classified into the subtypes Langerhans cell histiocytosis and Langerhans cell sarcoma, which are rare neoplasms exhibiting aggressive features and a poor prognosis. In addition to illustrating the refractoriness and poor outcomes of multisystem Langerhans cell histiocytosis in adults, specific events in this case highlight important characteristics of disease biology that warrant detailed discussion and exposition to a wider audience. CASE PRESENTATION: We describe the case of a 42-year-old Caucasian man with Langerhans cell histiocytosis diagnosed from a lesion on the left arm that presented with constitutional symptoms, early satiety, and weight loss. Esophagogastroduodenoscopy showed extensive esophageal and duodenal involvement by Langerhans cell histiocytosis with features of Langerhans cell sarcoma. He was initially treated for Langerhans cell histiocytosis with low doses of cytarabine until he eventually presented clear transformation to acute monoblastic leukemia with complex karyotype that could not be properly controlled, leading eventually to death. CONCLUSIONS: Langerhans cell histiocytosis remains an exceedingly rare entity in adults, frequently presenting as multisystem disease with risk organ involvement. Langerhans cell sarcoma represents an aggressive subtype with extremely poor prognosis for which intensive acute myeloid leukemia induction should be strongly considered.

Intususcepción recto rectal. Prevalencia en pacientes asintomáticos. / Rectum-rectal intussusception. Prevalence in asymptomatic patients

Introducción: La intususcepción rectal es un hallazgo frecuente en la defecografía tanto en individuos sintomáticos como en asintomáticos. Existe muy poca información con respecto al diagnóstico y tratamiento de esta anormalidad. También existe discrepancia entre las anormalidades anatómicas que pueden acompañar a esta patología, así como el grado de desarrollo de la intususcepción en pacientes asintomáticos. Objetivos: Describir la magnitud de la intususcepción en pacientes asintomáticos y comparar estos con los pacientes sin intususcepción. Métodos: Medimos, con un instrumento graduado en centímetros y milímetros, la pared rectal que sobresale en forma circunferencial hacia distal del borde de un anoscopio circular. Pacientes: Un grupo de 50 pacientes operados de prolapso anal/hemorroidal y 50 pacientes operados por otras patologías anales (fisura, condilomas, estenosis anal). El 49% eran mujeres. El promedio de edad fue de 49.7 años (±4.5 años). Ningún paciente tenía síntomas de obstrucción defecatoria. Resultados: En los pacientes del grupo con prolapso anal/hemorroides encontramos una intususcepción promedio de 1.59 cm comparada con 0.23 cm en los pacientes del grupo operado por otra patología. Estos resultados son estadísticamente significativos (p=0.003). Del grupo estudiado, 33 pacientes presentaban intususcepción- mayor a 1 cm, comparados con los sujetos controles (p=0.001). También fue significativa la diferencia entre aquellos que no tenían intususcepción y aquellos que sí la tenían aunque sea menores a 1 cm (p=0.004). No hubo diferencias significativas entre los dos grupos en cuanto a síntomas de obstrucción defecatoria o alguno que no tuviese relación con la patología por la cual iban a ser intervenidos quirúrgicamente (p=0.286). La longitud de la intususcepción fue de 0.5 a 2.5 cm y estuvo relacionada directamente a la patología subyacente por la cual se operaban los pacientes (fistulas, fisuras, condilomas, vs hemorroides, prolapso mucoso parcial y prolapso mucoso completo) (p = 0.003) e inversamente a los síntomas que presentaban (p < 0.001). Conclusiones: La intususcepción recto-rectal está presente, en diverso grado, en los pacientes con prolapso anal/hemorroidal, por lo que recomendaríamos realizar a todos estos pacientes una defecografía para evaluar el verdadero significado de esta patología. Se necesitan estudios a largo plazo para determinar la evolución real de esta entidad.

Abstract: Introduction: Rectal intussusception is frequently found in defecography both in symptomatic and asymptomatic individuals. There is scarce information about the diagnosis and treatment of this abnormality. There is also discrepancy on the anatomic abnormalities that this pathology may carry as well as the degree of development of intussusception in asymptomatic patients. Objectives:To describe the extent of intussusception in asymptomatic patients, and to compare them with patients without intussusception. Methods: Using an instrument marked in centimeters and millimeters, we measured the rectal wall that protrudes circumferentially towards the distal end of a circular anoscope edge. Patients: A group of 50 patients who had undergone anal / hemorrhoidal prolapse surgery and 50 patients who had undergone other anal surgeries related to pathologies such as anal fissures, condylomata, and stenosis. 49% of the patients were women. The average age was 48.7 years (±4.5 years).No patient presented symptoms of defecatoryobstruction. Results: Among the patients in the group with ana prolapse/hemorrhoids, an average intussusception of 1.59 cm was observed. Among the patients in the group who had undergone surgery for other pathologies, the average was 0.23 cm.These results are statistically significant (p=0.003). 33 patients in the group studied showed an intussusception larger than 1 cm, compared to control subjects (p=0.001).The difference among those without intussusception and those with it, even though it was less than 1 cm (p=0.004), was also significant.There were no significant differences among the groups with regard to the symptoms of defecatory obstruction or symptoms unrelated to the pathology that would cause their surgery (p=0.286). Intussusception length was 0.5 to 2.5 cm. It was directly related to the underlying pathology that required surgery in the patient, i.e. fistulae, fissures, condylomata vs. hemorrhoids, partial mucous prolapse and complete mucous prolapse (p=0.003) and inversely related to the symptoms shown (p< 0.001). Conclusions: Rectum-rectal intussusception is observed, in different degrees, in patients with anal/hemorrhoidal prolapse. Thus, we would recommend to perform a defecography to all these patients in order to assess the true meaning of this pathology. Long term studies will be necessary to determine the real evolution of this condition.

[Cholangitis secondary to migrated metallic coils in the common bile duct]. / Colangitis secundaria a espirales metálicos emigrados al conducto biliar.

Biliary obstructions are infrequently caused by foreign bodies. We present an unusual case of angiographically placed metallic coils into the intrahepatic arteries to provide hemostasis, that subsequently eroded into the common bile duct leading to obstructive jaundice and cholangitis a year later. In patients with history of invasive procedures, the possibility of foreign body migration into the common bile duct should always be considered in the differential diagnosis of obstructive jaundice and cholangitis.

Colangitis secundaria a espirales metálicos emigrados al conducto biliar / [Cholangitis secondary to migrated metallic coils in the common bile duct].

Biliary obstructions are infrequently caused by foreign bodies. We present an unusual case of angiographically placed metallic coils into the intrahepatic arteries to provide hemostasis, that subsequently eroded into the common bile duct leading to obstructive jaundice and cholangitis a year later. In patients with history of invasive procedures, the possibility of foreign body migration into the common bile duct should always be considered in the differential diagnosis of obstructive jaundice and cholangitis.

Colangitis secundaria a espirales metálicos emigrados al conducto biliar. / [Cholangitis secondary to migrated metallic coils in the common bile duct].

Biliary obstructions are infrequently caused by foreign bodies. We present an unusual case of angiographically placed metallic coils into the intrahepatic arteries to provide hemostasis, that subsequently eroded into the common bile duct leading to obstructive jaundice and cholangitis a year later. In patients with history of invasive procedures, the possibility of foreign body migration into the common bile duct should always be considered in the differential diagnosis of obstructive jaundice and cholangitis.

Single-session endoscopic ultrasonography and endoscopic retrograde cholangiopancreatography for evaluation of pancreaticobiliary disorders.

BACKGROUND: Endoscopic ultrasonography (EUS) and endoscopic retrograde cholangiopancreatography (ERCP) often are required in the evaluation and treatment of patients with pancreaticobiliary disorders. Few reports of single-session EUS-ERCP have raised questions regarding its safety and accuracy or about which procedure should be performed first. METHODS: Data from 2005 to 2009 were reviewed from a prospectively maintained EUS-ERCP database at a single tertiary care cancer center. Sensitivity and specificity of EUS and fine-needle aspiration (FNA), bile duct cannulation rate, duration of procedure, and complications were evaluated. RESULTS: Of the 35 patients (15 men and 20 women) studied, 28 had a final diagnosis of malignancy, and 7 had benign disorders. All the patients underwent ERCP and EUS, with FNA performed for 28 patients (80%). For 22 of the 35 patients (62.8%), EUS was the first procedure performed. The sensitivity of EUS-FNA for malignancy was 96.4%. The bile duct cannulation rate during ERCP was 97.1%. Five patients required a precut sphincterotomy for bile duct access, and one patient with chronic pancreatitis had a failed cannulation despite a EUS-guided rendezvous. A stent was successfully placed in 29 patients (96%). No major complications occurred, and no contrast leak was seen when FNA was performed before the cholangiogram. One patient had periduodenal bleeding after FNA, which was managed conservatively. The mean duration of the procedure was 83.7 min. CONCLUSION: Single-session EUS-ERCP can be performed safely and with efficacy similar to that of the procedures performed separately.