Distal Ileal Lymphoma Presenting Ileocecal Intussusception with Spontaneous Reduction / 대한소화기학회지

Intussusception is a rare disease in adults. A demonstrable etiology is found in approximately 85% of all cases, and approximately 40% of them are caused by malignant tumors. A 65-year-old patient visited the outpatient department with mild abdominal pain without other symptoms. The initial laboratory test and simple X-ray showed normal findings. CT revealed intussusception in the ileocecal area. The initial colonoscopic biopsy revealed atypical cells. Follow up colonoscopy showed spontaneous reduction of the intussusception. Diffuse large B-cell lymphoma was suspected in the second colonoscopic biopsy. An elective operation was performed. This case reports a case of a spontaneous reduction of adult intussusception with a brief review of literature.

Distal Ileal Lymphoma Presenting Ileocecal Intussusception with Spontaneous Reduction / 대한소화기학회지

Intussusception is a rare disease in adults. A demonstrable etiology is found in approximately 85% of all cases, and approximately 40% of them are caused by malignant tumors. A 65-year-old patient visited the outpatient department with mild abdominal pain without other symptoms. The initial laboratory test and simple X-ray showed normal findings. CT revealed intussusception in the ileocecal area. The initial colonoscopic biopsy revealed atypical cells. Follow up colonoscopy showed spontaneous reduction of the intussusception. Diffuse large B-cell lymphoma was suspected in the second colonoscopic biopsy. An elective operation was performed. This case reports a case of a spontaneous reduction of adult intussusception with a brief review of literature.

Spectrum of malabsorption syndrome among adults & factors differentiating celiac disease & tropical malabsorption.

Background & objectives: Aetiology of malabsorption syndrome (MAS) differs in tropical and temperate countries over time; clinical and laboratory parameters may differentiate between various causes. This study was undertaken to investigate the spectrum of MAS among Indian adults and to find out the features that may help to differentiate between TM and celiac disease. Methods: Causes of MAS, and factors differentiating tropical malabsorption (TM) from celiac disease (CD) were determined in 275 patients. Results: Using standard criteria, causes in 275 patients [age 37.5+13.2 yr, 170, (61.5%) male] were, TM 101 (37%), CD 53 (19%), small intestinal bacterial overgrowth 28 (10%), AIDS 15 (5.4%), giardiasis 13 (5%), hypogammaglobulinemia 12 (4%), intestinal tuberculosis 7 (2.5%), strongyloidiasis 6 (2%), immunoproliferative small intestinal disease 5 (2%), Crohn's disease 6 (2%), amyloidosis 4 (1.5%), intestinal lymphangiectasia 3 (1%) and unknown 22 (8%). On univariate analysis, patients with CD were younger than TM (30.6+12 vs. 39.3+12.6 yr, P<0.001), had lower body weight (41.3+11.8 vs. 49.9+11.2 kg, P<0.001), longer diarrhoea duration (median 36 inter-quartile range 17.8-120 vs. 24-months, 8-48, P<0.01), lower stool frequency (6/day, 5-8 vs. 8, 5-10, P<0.05), lower haemoglobin (9.4+3.2 vs. 10.4+2.7 g/dl, P<0.05), higher platelet count (2,58,000, range 1,35,500-3,23,500 vs. 1,60,000, 1,26,000-2,58,000/mm3, P<0.05), and more often had hepatomegaly (9/53, 17% vs. 4/101, 4%, P<0.01), and subtotal or partial villous atrophy (36/50, 72% vs. 28/87, 32%, P<0.001). Younger age (<35 yr), longer diarrhoea duration, higher platelet count and villous atrophy were significant on multivariate analysis. Interpretation & conclusions: TM and CD are common causes of MAS among Indian adults. Younger age (<35 yr), longer diarrhoea duration, higher platelet count and villous atrophy were found to be associated with CD.

Immunoproliferative small intestinal disease [IPSID]

This study describes the frequency, demographics, clinical presentation, endoscopic findings, histopathological features, treatment and outcome of 'Immunoproliferative small intestinal disease' [IPSID]. Archives contained a total of 27 cases of IPSID diagnosed and treated over an 18-year period. A M: F ratio of 2.4:1 was seen with a mean and median ages of 28.7 and 25 years. Most patients [68.8%] presented with abdominal pain and diarrhoea. In the majority [62.5%], duodenum was the primary site of involvement. Endoscopy showed polypoidal, raised or flat lesions. Biopsy findings included blunting or flattening of villi with dense plasma cell infiltrate and lymphoepithelial lesions. Twenty-four cases were categorized as stage A and B [benign and intermediate] and three were categorized as stage C [malignant, diffuse large B-cell lymphoma with plasmacytoid features]. Stage A and B patients responded well to antibiotic treatment [tetracycline] with regression of the lesions while for stage C patients standard CHOP chemotherapy was administered

Is immunoproliferative small intestinal disease uncommon in India?

Till date only three series of immunoproliferative small intestinal disease (IPSID) describing 22 patients have been reported from India. Seven patients with IPSID in two tertiary referral centers in India are included in the study. Diagnosis was based on typical clinical features [diarrhoea (7/7), weight loss (7/7), clubbing (6/7), fever (3/7), abdominal pain and lump (3/7)], biochemical evidence of malabsorption and duodenal biopsy findings. All patients were young males (mean age 29.8 +/- 11.8 years, range 17-53). Atypical features included gastric involvement (1/7), colonic involvement (1/7) and appearance of pigmented nails following anti-cancer chemotherapy (1/7) which disappeared six months after omitting doxorubin from chemotherapy regimen. Parasitic infestation was common. Ascaris lumbricoides (1/7), Giardia lamblia and hookworm (1/7), Strongyloides stercoralis and Trichuris trichura (1/7). In the latter patient S. stercoralis became disseminated after anti-malignant chemotherapy. One patient had gastric H. pylori infection. Four of the seven patients who were misdiagnosed as tropical sprue were treated with tetracycline. This raises doubt on efficacy of tetracycline alone in treatment of IPSID. One other patient was misdiagnosed and treated as intestinal tuberculosis. Early diagnosis and administration of chemotherapy may improve survival in this disease.

Linfomas intestinales / Intestinal lymphomas

Los linfomas primarios del intestino delgado son tumores raros, ocupan del 20 al 40 por ciento de las neoplasias malignas de esa región. Son un grupo heterogéneo de tumores con características clínicas y patológicas variables. Son neoplasias de adultos, se presentan con mayor frecuencia de los 20 a los 60 años. Los síntomas se desarrollan insidiosamente y pueden incluir pérdida de peso, diarrea, sangrado rectal, dolor abdominal, vómito y constipación. La enfermedad celiaca, la enfermedad inflamatoria intestinal y los estados de inmunodeficiencia se consideran factores predisponentes. El comportamiento biológico, el cuadro clínico, la morfología, el inmunofenotipo y el tratamiento son diferentes de los de los linfomas originados en ganglios linfáticos. Para clasificarlos correctamente es necesario definir el tipo histológico, el grado histológico, la resecabilidad del tumor y si se logra o no la remisión después del tratamiento combinado

A Case of Mucosa-associated Lymphoid Tissue Lymphoma in Small Intestine / 대한소화기내시경학회지

Mucosa-associated lymphoid tissue (MALT) lymphoma is the disease of distinctive clinicopathologic entities most of which are different from current lymph-node based lymphoma classification. According to the circulatory properties of the lymphocytes and specific immunoglobulin isotype distribution, MALT is defined as the central lymphoid tissue and is opposed to peripheral somatic lymphoid tissue. It occurs most often in the gastrointestinal mucosa and the bronchial mucosa and may occur in other organs such as salivary gland, thyroid gland, conjunctiva, skin. The most common site of MALT lymphoma is gastrointestinal tract but non-gastrointestinal MALT lymphoma may present. The characteristics of pathology are reactive follicles surrounded by the diffuse infiltration of centrocyte-like (CCL) cells and lymphoepithelial lesion due to the gland invasion of CCL cells. It is a localized disease and has a long survival, Isaacson et al suggested the classification of primary gastrointestinal MALT lymphoma as low grade, high grade, immunoproliferative small intestinal disease (IPSID). The distribution is more often in the stomach than in the intestine. Intestinal MALT lymphomas have less favorable clinicnl courses than the gastric MALT lymphomas. Recurrences may appear in the same organ or in other extranodal sites. We report clinical, pathologic findings, and clinical course in a case of primary small intestinal MALT lymphoma in terminal ileum with literature review.

Enfermedad Inmunoproliferativa del Instestino Delgado / Immunoproliferative small intestinal disease

Se presenta el caso de una pacientes con una enfermedad inmunoproliferativa de intestino delgado, tipo enfermedad de cadenas alfa, con el objetivo de dar a conocer una entidad infrecuente, que constituye el segundo caso en Cuba. La misma se presento con una historia de cuaros diarreicos relacionados con infestacion por giardia Lamblia y se diagnostico mediante biopsia yeyunal por via oral. Se destaca la mejoria clinica experimentada tras la terapeutica utilizada sobre la base de tetraciclinica a dosis de 1 g y prednisona 60 mg por via oral, aun se mantiene asintomatica despues de 6 meses de observacion

Linfomas del intestino delgado y cirugía / Primary small bowel lymphomas and surgery

Se presentan 45 casos de linfomas primarios de intestino delgado estudiados entre 1983 y febrero de 1995, de los cuales 2 correspondieron a la variedad Hodgkin (4,4 por ciento) y 43 a linfomas No Hodgkin (95,6 por ciento), incluyendo en éstos tres casos de enfermedad linfoproliferativa de intestino delgado. Se destaca que la localización más frecuente fue el yeyuno (51,2 por ciento), seguida del ileón y el duodeno encontrándose localizaciones múltiples en 23,2 por ciento. Las complicaciones obstructivas, perforativas y hemorrágicas fueron en el 93 por ciento de los pacientes lo que motivó la indicación quirúrgica. Se pone énfasis sobre la necesidad de la laparotomía exploradora con el fin de lograr el estudio histopatológico correcto, estadificar la lesión y evaluar la posibilidad de resección con criterio curativo o paliativo. Se analiza la supervivencia global de los linfomas No Hodgkin, que demuestra una diferencia significativa entre los operadores con criterio paliativo y curativo, 26 por ciento para los primeros y 47 por ciento en los segundos a los 70 meses, marcando también una significativa diferencia cuando existe o no compromiso ganglionar, 27 por ciento contra 52 por ciento a los 5 años

Immunoproliferative small intestinal disease: a frequently missed diagnosis.

Immunoproliferative small intestinal disease (IPSID) is a poorly recognized cause of malabsorption syndrome in India. Clinicopathological features of five patients with IPSID seen over a two-year period are described. Our data suggest that IPSID is commonly misdiagnosed as intestinal tuberculosis due to lack of awareness and reluctance to obtain small bowel biopsies. Empirical institution of anti-tubercular chemotherapy not only leads to delayed diagnosis but also possibly alters the natural history of the disease, resulting in an intermediate phase of amelioration followed by a terminal phase of lymphomatous transformation. The disease is therefore usually diagnosed at an advanced stage and hence is associated with a relatively poor outcome.

Enfermedad inmunoproliferativa primaria del intestino delgado (IPSID) / Inmunoproliferative small intestinal disease: presentation and review of two cases

Se presentan dos casos de linfoma primario del intestino delgado, evaluados y tratados en el hospital Vargas de Caracas, destacando los hallazgos clínicos y paraclínicos encontrados. Ambos casos mostraron una afección difusa del intestino delgado (IPSID), siendo clasificados histológicamente como linfoma no Hodgkin estadio IV, y tratados con quimioterapia. Se hace una revisión de esta patología

Aspectos clínicos e histopatológicos de la enfermedad inmunoproliferativa del intestino delgado: presentación de tres casos / Clinical and pathological aspects of immunoproliferative sammal intestinal disease: report of three cases

Se reportan tres casos de enfermedad inmunoproliferativa del intestino delgado (EIPID), diagnosticados en el Hospital "Víctor Lazarte E." IPSS Trujillo - Perú, en base a criterios clínicos e histopatológicos, estableciéndose por la evolución y respuesta al tratamiento que dos de ellos coresponden a enfermedad de cadena alfa (ECA) y el tercero a linfoma inmunoblástico. Se enfatizan las características clínicas, inmunológicas e histopatológicas de EIPID.

Linfoma intestinal de mediterraneo reporte de un caso y revisión de la literatura

Esta patología es una variante de las enfermedades del intestino delgado inmunoprofiferativa. Es extremadamente rara en Occidente. Esta asociada con mala absorción, perdida de peso y dolor abdominal. Compromete el duodeno y yeyuno. Se caracteriza por infilitrado linfoplasmocitario difuso de la mucosa del intestino delgado. Es endémico en el mediterraneo y Oriente Medio. Los fragmentos de cadena pesada alfa pueden o no estar presentes en el suero o fluído intestinal. Poseen un nivel de sobrevida mayor que los de Occidente. El tratamiento consiste en Quimioterapia y Radioterapia. Se revisa un caso de un paciente de 49 años de edad con esta patología, su tratamiento y seguimiento durante 9 años motivo de la presente publicación. Además se incluye una extensa bibliográfia.

Immunoproliferative small intestinal disease (IPSID) in Thailand.

Immunoproliferative small intestinal disease (IPSID) is prevalent in the Mediterranean region and in many Third World countries but is rare in Southeast Asia. Between 1980-1990, 4 cases of IPSID were admitted to Ramathibodi Hospital, Bangkok. Three were males and the mean age was 32 +/- 20.2 years. All patients presented with chronic diarrhea of 7 months to 6 years duration, and weight loss of 15 to 31 kg. All were malnourished, three cachectic, and one patient showed growth retardation. Intestinal parasites were found in all cases: two had multiple infections and three had uncommon protozoal infections (coccidium, cryptosporidium). Barium radiographs revealed intestinal mucosal fold thickening with malabsorption pattern in all cases. Alpha chain IgA was detected in one patient. The remainder underwent exploratory laparotomy and the histological finding was of plasma lymphocytic infiltration of the small intestinal mucosa. All patients responded to oral tetracycline with complete remission occurring in one case. During the follow-up period, 3 cases had progressive retractable clinical courses but all died 2 to 5 years after the diagnosis. The causes of death in these patients were secondary bacterial infection (1 case), intestinal tuberculosis (1 case), fungal infection (1 case) and immunoblastic sarcoma in another case. The results of this study confirm the occurrence of IPSID in Thailand. IPSID responds to oral antibiotic therapy and complete remission may be achieved during the early reversible benign phase, thus an awareness of its occurrence is of clinical importance.