Immunoproliferative small intestinal disease in south india. Analysis of dna from the mucosal tissue of three patients with immunoproliferative small intestinal disease ipsid and alpha chain disease, two of whom had early stage disease responsive to antibiotics, showed monoclonal heavy and. Immunoproliferative small intestinal disease immunoproliferative small intestinal disease ipsid is a disorder related to extranodal malt lymphoma that is prevalent in populations living around the mediterranean sea. Immunoproliferative small intestinal disease medigoo. Malt lymphoma called immunoproliferative small intestinal disease. Asignificant increase ofawl9 and b12 antigens was noted amongthe patients compared with the control. Hcd is the gastrointestinal form known as immunoproliferative small intestine disease or ipsid, but it has also been reported in the respiratory tract, and other areas of the body. The most common type of heavy chain disease is the iga type, known as. Patients and method six patients had immunoproliferative small intestinal disease ipsid indicated by the presence of alphaheavy.
Immunoproliferative small intestinal disease ipsid is a rare bcell mucosaassociated lymphoid tissue malt lymphoma that involves mainly the proximal small intestine, and is characterized by the synthesis of a monotypic truncated immunoglobulin aheavy chain lacking associated light chains 1,2. In the recent who classification of hematopoietic and lymphoid tissue, ipsid is considered as a variant of extranodal mucosaassociated lymphoid tissue malt lymphoma. Khojasteh and colleagues described immunoproliferative small intestinal disease ipsid as a thirdworld lesion june 9 issue. Immunoproliferative small intestinal disease originally called mediterranean lymphoma and subsequently alpha chain disease is a slowly progressive low grade primary small intestinal bcell lymphoma characterized by the synthesis of a truncated alpha heavy chain without light chain by the neoplastic cells. Aug 07, 2011 immunoproliferative small intestinal disease ipsid, a term adopted by who in 1978, is a variant of extranodal marginal zone lymphoma of mucosa associated lymphoid tissue malt in which defective heavy chains are secreted. Immunoproliferative small intestinal disease in algerians.
Phenotypic analysis and whole genome sequencing identified campylobacter coli. Patients and method six patients had immunoproliferative smallintestinal disease ipsid. In the west, this disease is less frequent, accounting for 20% of all extranodal, and 30% of gastro intestinal lymphomas 2. Immunoproliferative small intestinal disease and bcell malt. In early to mid 1960s it was referred to as mediterranean lymphomas, during the late 1960s the term. We report a case of the nonsecretory variant of immunoproliferative small intestinal disease involving the distal small bowel and the mesenteric and retroperitoneal lymph nodes in a 19yearold woman from mexico. The results were compared with 35 disease controls and 120 normal controls.
A group of 21 patients with immunoproliferative small intestinal disease ipsid, 10 with alpha heavy chain paraproteinaemia and 11 without it, were hla typed. Read immunoproliferative small intestinal disease in algerians. Immunoproliferative small intestinal disease ipsid is a prelymphomatous process that is unusual among persons native to the united states. Immunoproliferative small intestinal disease an overview. Molecular and immunohistochemical studies demonstrated an association with campylobacter jejuni. Oral manifestation of immunoproliferative small intestinal. What does immunoproliferative small intestinal disease mean. Twentytwo cases of immunoproliferative small intestinal disease ipsid were studied with an im munoperoxidase technique. Immunoproliferative small intestinal disease ipsid was recently added to the growing list of infectious pathogenassociated human lymphomas. Immunoproliferative small intestinal disease ipsid is a rare lowgrade bcell lymphoma arising from mucosa associated lymphoid tissue malt, representing approximately onethird of intestinal lymphoma. Despite the obvious long standing gingival inflammation, minimal destruction of alveolar bone had occurred.
Information and translations of immunoproliferative small intestinal disease in the most comprehensive dictionary definitions resource on the web. Alpha heavy chain disease hcd is the most prevalent form of heavy chain diseases, a rare family of syndromes associated with or representing a b cell malignancy variant. Subsequently most cases are characterized by a loss of ability to synthesize light chains. Immunoproliferative small intestinal disease ipsid is a rare disorder, which can progress to malignancy and invasion. Immunoproliferative small intestine disease definition.
Herein, a male patient is presented with hypoalbuminemic ascites and a. Immunoproliferative small intestinal disease ipsid was recently added to the growing list of infectious pathogenassociated human. Pdf on sep 8, 2015, bivas biswas and others published immunoproliferative small intestinal disease. Immunoproliferative small intestinal disease ipsid is a special form of mucosa. Clinicopathological features of 12 patients with histologically confirmed disease were analysed. Defined as enteropathyassociated tcell lymphoma type in world health organization who classification of tumors of haematopoietic and lymphoid tissues in 2008. Summary immunoproliferative small intestinal disease ipsid is a rare disorder, which can progress to malignancy and invasion.
Nonsecretory variant of immunoproliferative small intestinal. Campylobacter has been associated with immunoproliferative small intestinal disease ipsid, on the basis of 16s rdna sequencing, in situ hybridization, and immunohistochemistry. Immunoproliferative small intestinal disease without a. In middleeastern and mediterranean countries immunoproliferative small intestinal disease is endemic, whereas in other parts of the world including northwestern europe. A patient with a previous medical history of immunoproliferative small intestinal disease ipsid presented with persistent red and ulcerated lesions on her attached gingiva. It involves the small intestine, and patients usually present. A new form of this lymphoma, immunoproliferative small intestinal disease ipsid, has been shown to be geographically confined primarily to mediterranean and middle eastern countries 3, 4. In the west, this disease is less frequent, accounting for 20% of all extranodal, and 30% of gastrointestinal lymphomas 2.
Immunoproliferative small intestinal disease must also be distinguished from celiac sprue, tropical sprue, aids enteropathy, and whipples disease, because all of these can be associated with villus changes and increased infiltration of the lamina propria with round cells. In this disease the small intestine fills with plasma cells that secrete an incomplete immunoglobulin, one in which an alpha heavy chain of the immunoglobulin molecule is formed without an associated light chain. Campylobacter coli cultured from the stools of a patient with. Analysis of dna from the mucosal tissue of three patients with immunoproliferative small intestinal disease ipsid and alpha chain disease, two of whom had early stage disease responsive to antibiotics, showed monoclonal heavy and light chain gene rearrangements in all cases. The infiltrates produces an aberrant immunoglobulin iga, a. This study describes the frequency, demographics, clinical presentation, endoscopic findings, histopathological features, treatment and outcome of immunoproliferative small intestinal disease ipsid. Campylobacter coli cultured from the stools of a patient. Immunoproliferative small intestinal disease is primarily a disease of older children and young adults, with a mean age at presentation of 25 to 30 years. Purpose the clinicopathologic findings in 45 adult chinese patients with primary smallintestinal lymphoma psil are described and compared with those in western countries and in underdeveloped nations. Immunoproliferative disorders, are disorders of the immune system that are characterized by the abnormal proliferation of the primary cells of the immune system, which includes b cells, t cells and natural killer nk cells, or by the excessive production of immunoglobulins also known as. Longterm infection with the hepatitis c virus hcv seems to be a risk factor for. Immunoproliferative small intestinal disease ipsid jcpsp. Immunoproliferative small intestinal disease with duodenojejunal.
Immunoproliferative small intestinal disease and primary small. Immunoproliferative small intestinal disease ipsid. Aw19, b12in immunoproliferative small intestinal disease. Pdf immunoproliferative small intestinal disease ipsid. Immunoproliferative small intestinal disease in an. Immunoproliferative small intestinal disease without achain. Summary agroup of 21 patients with immunoproliferative small intestinal disease ipsid, 10 with alpha heavy chain paraproteinaemia and 11 without it, were hlatyped. Cytological study of a sample obtained from the mesenteric nodes under us guidance provided a probable diagnosis of immunoproliferative small intestinal disease ipsid. Immunoproliferative small intestinal disease in an american patient with lymphoma and. Immunoproliferative small intestine disease definition of. Immunoproliferative small intestinal disease is a distinctive lymphoproliferative disorder. Immunoproliferative small intestinal disease ipsid, also known as alpha chain disease, is a rare disease. In middleeastern and mediterranean countries immunoproliferative small intestinal disease is endemic, whereas in other parts of the world including northwestern europe and north america celiac. Immunoproliferative small intestinal disease in an american.
Immunoproliferative small intestinal disease associated with. Ipsid is a variant of the bcell lymphoma of mucosaassociated lymphoid tissue malt, which involves mainly the proximal small. Read immunoproliferative small intestinal disease and primary small intestinal lymphoma. Immunoglobulin gene rearrangement in immunoproliferative. It predominantly affects young people in the mediterranean region. Immunoproliferative small intestinal disease ipsid is a special variant of, extranodal marginal zone bcell lymphoma, which affects the small intestine. A significant increase of aw19 and b12 antigens was noted among the patients compared with the control subjects. Here, for the first time, we have cultured campylobacter from the stools of a patient with ipsid.
Immunoproliferative small intestinal disease ipsd is a prevalent, debilitating illness in many developing countries particularly middle east and the. Immunoproliferative small intestinal diseasea rare extranodal. Histopathologically it is characterized by infiltration of small bowel wall with lymphocytes and plasma cells. Immunoproliferative small intestinal disease and primary. Immunoproliferative small intestinal disease associated with overwhelming polymicrobial gastrointestinal infection with transformation to diffuse large b cell. Among these disorders, it is the only disease associated with a specific and characteristic abnormal protein, and also an identifiable, at least in some patients, early phase with a benignlooking histopathologic expression. Medical definition of immunoproliferative small intestinal disease. Pdf immunoproliferative small intestinal disease in.
Immunoproliferative small intestinal disease ipsid, a term adopted by who in 1978, is a variant of extranodal marginal zone lymphoma of. Immunoproliferative small intestinal disease, also known as alpha chain disease, is a mucosaassociated lymphoidtissue malt lymphoma characterized by infiltration of the bowel wall with a. The distinctive patholog ical feature of immunoproliferative small intestinal disease, i. Hla aw19, b12 in immunoproliferative small intestinal disease. Immunoproliferative small intestinal disease associated. Nonhodgkin lymphoma causes, risk factors, and prevention. Immunoproliferative small intestinal disease description of immunoproliferative small intestinal disease. Of 100 cases of primary intestinal lymphoma associated with ipsid, 48 involved the duodenum and upper jejunum. This type of lymphoma, which is also sometimes called mediterranean abdominal lymphoma, typically occurs in young adults in eastern mediterranean countries.
Patients were mostly young males with median age of 24. Meaning of immunoproliferative small intestinal disease. Immunoproliferative small intestinal disease in an american patient with lymphoma and macroamylasemia meyer blumstein, simmy bank, ronald e. Delineation of the lymph nodes and intestinal wall involvement was less precise than with sonography. Immunoproliferative small intestinal diseasea rare.
The plasma cell infiltrate of the small intestine in alpha. Immunoproliferative small intestinal disease presented. The radiologic changes in 1090 cases of lymphoma of the small bowel were analyzed and correlated with the pathologic findings. Immunoproliferative small intestinal disease ipsid, a term adopted by who in 1978, is a variant of extranodal marginal zone lymphoma of mucosa associated lymphoid tissue malt in which defective heavy chains are secreted. Sep 21, 2016 purpose the clinicopathologic findings in 45 adult chinese patients with primary small intestinal lymphoma psil are described and compared with those in western countries and in underdeveloped nations. Relation to alpha chain protein, cancer on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. Immunoproliferative small intestinal disease ipsid is composed of a special subgroup of small intestinal malt lymphomas arising in the upper small intestine that is restricted in its incidence to certain geographic areas, occurring almost exclusively in the middle east. Immunoproliferative disorders, are disorders of the immune system that are characterized by the abnormal proliferation of the primary cells of the immune system, which includes b cells, t cells and natural killer nk cells, or by the excessive production of immunoglobulins also known as antibodies. Immunoproliferative small intestinal disease and bcell. Pdf on jan 1, 2012, abm shakil gani and others published immunoproliferative small intestinal disease. Following failure of the tissues to respond to routine periodontal therapy, a gingival biopsy was. Immunoproliferative smallintestinal disease ipsid is composed of a special subgroup of smallintestinal malt lymphomas arising in the upper small intestine that is restricted in its incidence to certain geographic areas, occurring almost exclusively in the middle east. Archives contained a total of 27 cases of ipsid diagnosed and treated over an 18year period.
Definition of immunoproliferative small intestinal disease in the dictionary. A rare mucosal lymphoma of the small intestine, associated with infection with campylobacter jejuni. Immunoproliferative small intestinal disease american journal of. This study was performed to confirm the existence of immunoproliferative small intestinal disease ipsid in pakistan. Immunoproliferative small intestinal disease ipsid is prevalent in the mediterranean region and in many third world countries but is rare in southeast asia. Nassar vh, salem pa, shahid mj, alami sy, balikian jb, sa lem aa, nasrallah sm. Immunoproliferative small intestinal disease with 40years of follow. A clonal disorder, also known as immunoproliferative small intestinal disease or mediterranean lymphoma, characterised by the secretion of a defective alpha heavy chain.
Ipsid is a variant of the bcell lymphoma of mucosaassociated lymphoid tissue malt, which involves mainly the proximal small intestine resulting in. Immunoproliferative small intestinal disease springerlink. On immunohistochemistry, lymphoid cells were positive for leukocyte. Lymphoma and immunoproliferative small intestinal disease. It has distinct epidemiological and histological features and commonly develops into a frank lymphoma that extends throughout the small intestinal subepithelial tissues from the lamina propria to the muscular. The material was collected in iran, a high incidence area for immunoproliferative small bowel disease ipsid. The efficacy of combination chemotherapy is also assessed. Mediterranean abdominal lym phoma or immunoproliferative small intestinal disease. Immunoproliferative small intestinal disease ipsid represents a.
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