Chronic diarrhea associated with high serum level of immunoglobulin A and diffuse infiltration of plasma cell in small intestine: A case report.

RATIONALE: Chronic diarrhea in adult patients due to various causes is very common in clinic, but patient suffering with mal-absorption due to immunoproliferative small intestinal disease was rarely reported in China. PATIENT CONCERNS AND DIAGNOSES: A 35-year-old female presented with more than three years history of chronic diarrhea, rickets, high serum value of immunoglobulin A protein, and anemia. Bone marrow aspiration suggested that the patient was in a sideropenic and megalobastic anemia stage. Duodenal and ileac biopsies revealed atrophy and blunting villi. The bowel lamina propria was infiltrated with slightly increased intraepithelial lymphocytes and mainly with diffuse plasma cells. The following enzyme labeling immunohistochemistry results were strongly positive to alpha-heavy-chain. Computed tomography manifested she had diffuse thickening of small intestine wall. At last a diagnosis of immunoproliferative small intestinal disease was made. INTERVENTIONS AND OUTCOMES: On the first month, the patient was treated with vitamin D supplements, calcium, magnesium, potassium, iron, folic acid, mecobalamin replacements and microflora probiotics. The patient frequency of water diarrhea alleviated slightly, but her weight loss, anxiety neurosis and other disorders were still severe. After taking with prednisone (40 mg per day, and gradually reduced to the lowest dose) for another month, the symptoms was gradually subsided. LESSONS: The study shows that immunohistochemical staining for alpha-heavy chain proteins should be completed on small intestine biopsy specimens if the patient is suspected a diagnosis of immunoproliferative small intestinal disease.

Immunoproliferative Small Intestinal Disease Associated with Overwhelming Polymicrobial Gastrointestinal Infection with Transformation to Diffuse Large B-cell Lymphoma.

Immunoproliferative small intestinal disease (IPSID) is an extra-nodal B-cell lymphoma most commonly described in the Mediterranean, Africa, and Asia. It is associated with poverty and poor sanitation, and is rarely encountered in developed countries. A 26-year-old previously healthy, Marshallese male was transferred to our facility with a 6-month history of watery diarrhea, weakness, and cachexia refractory to multiple short courses of oral antibiotics. Stool cultures grew Campylobacter jejuni and Vibrio fluvialis. Endoscopic evaluation showed histologic evidence of Helicobacter pylori gastritis and gross evidence of whipworm infection found in the colon. Mesenteric lymph node biopsy cultures grew Escherichia coli. Histopathology and immunohistochemical stains of the small intestine were consistent with IPSID. He subsequently transformed to diffuse large B-cell lymphoma (DLBCL) with tonsillar involvement despite treatment with rituximab and an extended course of antibiotics. Systemic chemotherapy with six cycles of rituximab, cyclophosphamide, vincristine, doxorubicin, prednisone, and lenalidomide, resulted in remission of his diffuse B cell lymphoma. This case is illustrative of IPSID developing in a previously healthy individual due to overwhelming polymicrobial gastrointestinal infection by C. jejuni and other enteric pathogens with subsequent transformation to an aggressive DLBCL. IPSID should be considered in residents of developing countries presenting with refractory chronic diarrhea, weight loss, and mesenteric lymphadenopathy.

Immunoproliferative small intestinal disease presented with ascites and edema.

Immunoproliferative small intestinal disease (IPSID) is a rare disorder, which can progress to malignancy and invasion. Herein, a male patient is presented with hypoalbuminemic ascites and a history of chronic diarrhea five years before. Small intestinal biopsy and immunohistochemical study suggested the diagnosis of IPSID; the patient was then successfully treated with antibiotics. Considering the favorable therapeutic response of IPSID to antibiotics during primary stages, clinicians should be aware of its various presentations in order to initiate treatment at an early

Immunoproliferative small intestinal disease with protein loss complicated with duodenal T cell lymphoma during progression.

A 52-year-old man was admitted to our hospital in October 2001 with abdominal pain. Abdominal X-ray indicated a diagnosis of ileus. Histopathological and immunological examination resulted in a diagnosis of immunoproliferative small intestinal disease (IPSID). He was treated with THP-COP therapy (pirarubicin, cyclophosphamide, vincristine, and prednisolone), which resulted in complete remission. Outpatient follow-up revealed hypoalbuminemia in May 2003 and upper gastrointestinal endoscopy showed duodenal mucosal nodularity. He was diagnosed with relapsed IPSID and salvage chemotherapy was started. Follow-up endoscopy confirmed that the therapy was effective, but uncovered another duodenal mucosal nodularity. Immunohistochemical staining revealed T-cell lymphoma. Chemotherapy was discontinued and the patient died in December 2004.

Immunoproliferative small intestinal disease: prolonged 30-year course without development of lymphoma.

Immunoproliferative small intestinal disease (IPSID) is mostly found in young adults of low socioeconomic class in developing countries. This condition is characterized by a dense lymphoplasmacytic infiltrate beneath the epithelium in the duodenal and proximal jejunal mucosa and in the mesenteric lymph nodes. In two thirds of cases, the involved lymphocytes elaborate an anomalous alpha-heavy chain protein. The etiology of this disease is unclear, although various parasitic, genetic, and toxic mechanisms have been proposed. Half of all IPSID patients will be found at diagnosis to have a concurrent intestinal B-cell lymphoma, and most of the remaining patients develop frank lymphoma within a few years. Although most reports of IPSID are from developing nations or indigent immigrant populations within Western countries, four cases of an IPSID-like condition have been documented in white women. Furthermore, although many IPSID patients progress to high grade indeterminate-type lymphoma within a few years of initial presentation, there have been occasional reports of long term survival without lymphomatous conversion. Here, we present an atypical case of IPSID--a California native who, though of Mexican heritage, had resided in the United States his entire life and did not belong to an indigent population. This patient had biopsy-proven IPSID that progressed over 30 yr but never exhibited lymphomatous conversion despite end stage intestinal stasis and recurrent obstruction, culminating in death. Our case calls into question some current assumptions about the prelymphomatous nature of this disease.

[Mediterranean lymphoma mimicking Crohn's disease]. / Lymphome méditerranéen simulant une maladie de Crohn.

We report an uncommon localization of mediterranean lymphoma of the terminal ileum in a 28 year-old male patient. Ultrasound and Computed Tomography showed moderate regular and symmetrical intestinal wall thickening simulating Crohn's disease. We highlight the role of computed tomography in the diagnosis, staging and detection of complications.

Nodular glomerulosclerosis with deposition of monoclonal immunoglobulin heavy chains lacking C(H)1.

The objective of this study was to further characterize the clinical and immunopathologic features of heavy chain deposition disease (HCDD), a recently described entity. Four patients were diagnosed as having HCDD on a kidney biopsy. All presented with nodular glomerulosclerosis with deposition of gamma1 heavy chains lacking CH1 epitopes, but without light chains. Two different patterns were observed in the serum. First, patients 1 and 2 had a circulating monoclonal IgGlambda containing a short gamma1 heavy chain lacking CH1 epitopes, with an apparent molecular weight of 40 kD consistent with a complete CH1 deletion. Biosynthetic experiments also showed that the deleted heavy chain was produced in excess compared with light chains, and was secreted in vitro together with half Ig molecules, although these abnormal components were not detected by Western blot analysis of whole serum. Second, patients 3 and 4 had a circulating monoclonal IgG1lambda with an apparently normal, nondeleted heavy chain subunit, but serum fractionation followed by immunoblotting revealed an isolated monoclonal gamma1 chain lacking CH1 epitopes. These data strongly suggest that renal deposition of a CH1-deleted heavy chain circulating in low amounts in the serum as a free unassembled subunit is a major feature of HCDD. The CH1 deletion is most likely responsible for the premature secretion in blood of the heavy chain by a clone of plasma cells.

Pigmented nails and Strongyloides stercoralis infestation causing clinical worsening in a patient treated for immunoproliferative small intestinal disease: two unusual observations.

Immunoproliferative small intestinal disease (IPSID) is commonly reported from developing countries with poor socioeconomic conditions, hygiene, and high frequency of gastrointestinal infections and infestations. The disease requires anti-malignant chemotherapy in lymphomatous stage. Reported here is a 20-year old man with IPSID lymphoma who responded to anti-malignant chemotherapy initially, but later deteriorated due to Strongyloides stercoralis infestation, which was treated successfully with mebendazole. Importance of an early recognition and adequate treatment for gastrointestinal infections and infestations before anti-malignant chemotherapy for this disease is highlighted considering the occurrence of this disease in the developing world. The patient developed alternate brown black and white lines in the finger nails after combination chemotherapy, which has not been reported earlier in this disease; the nail changes disappeared 6 months after the withdrawal of doxorubicin suggesting this drug as the cause for such nail changes during anti-malignant combination chemotherapy.

Immunoproliferative small intestinal disease in a 16-year-old boy presenting as severe malabsorption with excellent response to tetracycline treatment.

Immunoproliferative small intestinal disease (IPSID) is a rare lympho-proliferative disorder of the upper small intestine. It is considered a special form of MALT lymphoma with propensity to malignant transformation. This disorder is rare in pediatric literature. We report a case of IPSID in a 16-year-old boy with low-grade malignant transformation, presenting as severe malnutrition and a possible association with Helicobacter pylori. The patient responded well to an extended treatment with tetracycline and eradication of H. pylori.

[Libyan patient with chronic diarrhea]. / Libyscher Patient mit chronischen Durchfällen.

A 22-year-old Libyan patient suffering from chronic diarrhea presented with an alpha-heavy chain paraprotein and a lympho-plasmacellular lymphoma infiltration of the duodenal mucosa. These findings supported the diagnosis of "immunoproliferative small intestinal disease" (IPSID). In this disease, that occurs almost solely in countries with low socioeconomic status, a diffuse infiltration of small intestinal mucosa by neoplastic lymphoid cells causes chronic malabsorption. About 65% of patients exhibit a paraprotein in serum, urine or jejunal juice that consists of the heavy chain of immunoglobulin A (alpha-heavy chain). In advanced stages, IPSID resembles histologically and clinically high grade lymphoma: some patients develop masses in the gut wall, an abdominal lymphadenopathy and involvement of other organs including bone marrow. The disease is believed to be triggered by a chronic infectious antigenic stimulus. Thus, in early stages in some patients cure may be achieved by antibiotic therapy alone. In advanced disease, chemotherapy including anthracyclins is necessary.

Premalignant conditions of the small intestine.

Cancer of the small intestine is rare compared with other sites in the gastrointestinal tract. Of the four major primary small-bowel tumors (adenocarcinomas, lymphomas, carcinoid, and leiomyosarcomas), adenocarcinomas and lymphomas are associated with diseases that seem to increase the risk of developing these malignancies. In the case of immunoproliferative small intestinal disease and celiac disease, both of which are thought to predispose patients to the development of primary lymphoma, treatment of the predisposing conditions seems to decrease the risk of developing subsequent malignancy. Recognition of the increased risk associated with other conditions, such as immunodeficiency syndromes, nodular lymphoid hyperplasia, Crohn's disease, the gastrointestinal polyposis syndromes, hereditary nonpolyposis colon cancer, neurofibromatosis, long-standing ileostomy, and urinary diversion procedures, may lead to early diagnosis and improved survival.

[Immunoproliferative small intestinal disease (IPSID): IPSID-like lymphomas, IPSID equivalents?, in 12 Spanish patients]. / Enfermedad inmunoproliferativa del intestino delgado (EIPID): linfomas "EIPID-símiles"--EIPID-equivalentes?--en doce pacientes españoles.

BACKGROUND: Immunoproliferative small intestine disease (IPSID) encompasses a primary intestinal lymphoma of underpriviliged populations of North Africa, Middle East, etc. This epidemiological feature strongly implicates environmental and host (genetic) factors in its pathogenesis. IPSID can be distinguished on clinicopathological grounds from "Western-type" intestinal lymphomas. "IPSID-like" lymphomas had been sporadically identified, i.e., patients with original clinico-analytical data of IPSID (chronic diarrhoea, malabsortion, clubbing of fingers, diffuse intestinal involvement, etc.) without its conventional histopathologic (lymphoplasmocytic or plasmocytic infiltration) and immunological (alpha-heavy-chain paraprotein) background. PURPOSE: The aim of this study has been: 1) to identify, in a series of small intestine lymphomas, a group of patients with a long-lasting history of chronic diarrhoea and a clinico-biologic pattern of "IPSID-like" lymphoma; 2) to analyze its clinicopathological profile; 3) to search for differences with the pattern of the remaining cases ("Western-type" lymphomas) and 4) To suggest a possible epidemiological significance. PATIENTS AND METHODS: Patients considered were 12 Spanish caucasians with primary intestinal lymphoma and a long-lasting history of chronic diarrhoea vs 31 cases of "Western-type" intestinal lymphomas admitted in our Hospital over a 33-year period. Statistical significance of differences in clinico-biological features (symptoms/signs, analytical data, patterns of involvement, histopathology, immunophenotype and tumor staging) between these two groups was evaluated using X2 test. RESULTS: The results of this retrospective study allow us to delineate a relatively homogeneous "IPSID-like" group (12 cases) among 43 cases of primary small intestine lymphoma diagnosed between 1960 and 1993. The clinico-pathological behavior of these patients was significantly different from that exhibited by the 31 cases of so-called "Western-type" lymphomas. CONCLUSIONS: It is suggested that they may represent a group of patients suffering an evanescent "IPSID-equivalent" disorder (last case diagnosed in 1975), that probably has evolved in similar but not identical epidemiological circumstances to those present in the "Third World" countries of our Mediterranean area.

Non-secretory alpha chain disease involving stomach, small intestine and colon.

A case of alpha chain disease, involving stomach, small and large intestine, and caecum with poor prognosis is reported. Endoscopic examination revealed gastric erosion, edematous mucosa with enlarged villi of duodenum and jejunum, multiple hyperplastic lymph follicles of terminal ileum and thickening mucosa of caecum. Light microscopy revealed a conspicuous infiltration of plasma cells and lymphocytes in gastric, duodenal, jejunal and caecal lamina propria. Immunohistochemistry demonstrated alpha heavy chain protein devoid of light chain in these plasma cells. The patient developed paralytic ileus and died of septic shock on the 179th hospital day.