Immunoproliferative Small Intestinal Disease : Report of Two Cases

Abstract

Immunoproliferative small intestinal disease (IPSID) or alpha heavy chain disease is a subtype of mucosa-associated lymphoid tissue (MALT) lymphoma that occurs almost exclusively in the Middle East and Mediterranean basin. It tends to be a disease of young males. Herein, we report two unrelated Thais with IPSID who have never visited the Middle East. The first case was a 50-year-old woman presenting with generalized abdominal pain for a few months, passing watery stool every other day, without blood or mucus. Her blood test showed pancytopenia and hyperglobulinemia. Colonoscopy revealed a large halfcircumferential clean based ulcer, with friable surrounding tissue. The cecum microscopic pathology was consistent with IPSID. The stool had no parasite. She was treated with right-half colectomy, prednisolone, danazol, tetracycline, and omeprazole. The patient survived a further year and 9 months. The second case was a 72-year-old man presenting with acute abdominal pain due to gut obstruction for 3 days. Exploratory laparotomy was immediately performed for a part of the small bowel that was affected for resection. Microscopic pathology was compatible with IPSID. His blood tested positive for latent syphilis but normal serum globulin. After the operation, he was treated with tetracycline and cyclophosphamide, and he survived for a further two years. These two cases were IPSID diagnosed outside the Middle East and Mediterranean Region; the ages of the cases were not young, at 50 and 72 years. Regarding the site of involvement, the colon in the first case was considered an unusual site for IPSID. As for infection that might contribute to the occurrence of IPSID, there was no infective diarrhea in the first case and only latent syphilis in the second case.