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

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.