Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0021831 (enteropathy)
 4,403 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report on a 28 year old Turkish woman, who was admitted to our hospital with the symptoms of malabsorption and protein-loosing enteropathy. Histologically, on duodenal biopsy, a lymphoplasmacellular infiltration of the submucosa with partial to subtotal atrophy of the villi was found. An immunoproliferative small intestinal disease (IPSID) was diagnosed. A short remission whilst on a glutenfree diet and tetracycline therapy, was followed by a laparatomy because of ileus in the small intestine. A high-grade-malignant Non-Hodgkin's Lymphome of B-cell type with intracellular production of alpha-Heavy-Chains (AHCD) was diagnosed histologically. Following chemotherapy with CEOP-IMVP-Dexa (Cyclophosphamide, Epidoxorubicin, Vincristine, Prednisolone, Ifosfamide, VP-16, Dexamethason, Methotrexat) the patient is still in complete remission three years after starting the therapy. We discuss here a case of AHCD in IPSID, the differential diagnosis of protein losing enteropathy and malabsorption, and we also present conservative (diet, medical treatment) and operative therapies.
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PMID:Malabsorption associated with a high-grade-malignant non-Hodgkin's lymphoma, alpha-heavy-chain disease and immunoproliferative small intestinal disease. 779 20

A 73-year-old male with melena was admitted to our hospital. Computed tomography (CT) scan revealed the thickening of the jejunal and ileal walls and swelling of the mesenteric lymph nodes. Type II enteropathy-associated T-cell lymphoma (EATL) was diagnosed based on the pathological analysis of the resected specimen. Positron emission tomography and CT scan showed complete remission (CR) after surgery, and he further received CHOP therapy. However, 2 months after the completion of the therapy, the patient's disease relapsed, and he presented with abdominal pain. Ifosfamide, dexamethasone, etoposide, and cytarabine therapy was administered, and the second CR was observed in the patient. Subsequently, the patient was administered high-dose chemotherapy (MCEC) with autologous peripheral blood stem cell transplantation (auto-PBSCT). The treatment was well tolerated. Engraftment was performed on day9, and he was discharged on day17 after auto-PBSCT. However, at 6 months after auto-PBSCT, the second relapse of the disease was observed in the patient. He received salvage therapy; however, the patient died because of disease progression. Because of the dismal prognosis of EATL treated with conventional chemotherapy, the feasibility and efficacy of auto-PBSCT have been investigated. To the best of our knowledge, there is no report on an elderly patient (age >70 years) with EATL who underwent auto-PBSCT. Thus, more data should be collected and analyzed to confirm that this therapy could be a promising treatment option for elderly patients with EATL.
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PMID:[Early relapse after autologous peripheral blood stem cell transplantation in an elderly patient with enteropathy-associated T-cell lymphoma]. 3007 99