The plasma cell infiltrate of the small intestine in alpha. Summary immunoproliferative small intestinal disease ipsid is a rare disorder, which can progress to malignancy and invasion. 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. Pdf immunoproliferative small intestinal disease in. Herein, a male patient is presented with hypoalbuminemic ascites and a. 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. Delineation of the lymph nodes and intestinal wall involvement was less precise than with sonography.
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 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. Campylobacter has been associated with immunoproliferative small intestinal disease ipsid, on the basis of 16s rdna sequencing, in situ hybridization, and immunohistochemistry. This type of lymphoma, which is also sometimes called mediterranean abdominal lymphoma, typically occurs in young adults in eastern mediterranean countries.
Lymphoma and immunoproliferative small intestinal disease. Monomorphic epitheliotropic intestinal tcell lymphoma. Immunoproliferative small intestinal disease ipsid was recently added to the growing list of infectious pathogenassociated human. Here, for the first time, we have cultured campylobacter from the stools of a patient with ipsid. Hla aw19, b12 in immunoproliferative small intestinal disease. Immunoproliferative small intestinal disease in south india. Pdf on jan 1, 2012, abm shakil gani and others published immunoproliferative small intestinal disease.
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. Information and translations of immunoproliferative small intestinal disease in the most comprehensive dictionary definitions resource on the web. Nassar vh, salem pa, shahid mj, alami sy, balikian jb, sa lem aa, nasrallah sm. Immunoproliferative small intestinal disease medical. Immunoproliferative small intestinal disease and primary. Campylobacter jejuni is a specific pathogen, found to be related to. 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. Mediterranean abdominal lym phoma or immunoproliferative small intestinal disease.
Immunoproliferative small intestinal disease in an american patient with lymphoma and. Immunoglobulin gene rearrangement in immunoproliferative. 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 description of immunoproliferative small intestinal disease. Pdf immunoproliferative small intestinal disease ipsid. 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 ipsid is a rare disorder, which can progress to malignancy and invasion. Immunoproliferative small intestinal disease ipsid represents a.
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. Immunoproliferative small intestinal disease is a distinctive lymphoproliferative disorder. 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. In the west, this disease is less frequent, accounting for 20% of all extranodal, and 30% of gastrointestinal lymphomas 2. Longterm infection with the hepatitis c virus hcv seems to be a risk factor for. Immunoproliferative small intestinal disease with 40years of follow. Despite the obvious long standing gingival inflammation, minimal destruction of alveolar bone had occurred. Archives contained a total of 27 cases of ipsid diagnosed and treated over an 18year period. Immunoproliferative small intestinal disease ipsid was recently added to the growing list of infectious pathogenassociated human lymphomas. Immunoproliferative small intestinal disease american journal of. Defined as enteropathyassociated tcell lymphoma type in world health organization who classification of tumors of haematopoietic and lymphoid tissues in 2008.
The results were compared with 35 disease controls and 120 normal controls. Immunoproliferative small intestinal disease ipsid is a special form of mucosa. It involves the small intestine, and patients usually present. Medical definition of immunoproliferative small intestinal disease. Immunoproliferative small intestinal disease an overview. Ipsid is a variant of the bcell lymphoma of mucosaassociated lymphoid tissue malt, which involves mainly the proximal small intestine resulting in. 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. A clonal disorder, also known as immunoproliferative small intestinal disease or mediterranean lymphoma, characterised by the secretion of a defective alpha heavy chain.
A significant increase of aw19 and b12 antigens was noted among the patients compared with the control subjects. Immunoproliferative small intestinal disease presented. The material was collected in iran, a high incidence area for immunoproliferative small bowel disease ipsid. 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 small intestine disease definition of. Of 100 cases of primary intestinal lymphoma associated with ipsid, 48 involved the duodenum and upper jejunum. In early to mid 1960s it was referred to as mediterranean lymphomas, during the late 1960s the term. Potentially preventable cancer from the third world. Patients and method six patients had immunoproliferative small intestinal disease ipsid indicated by the presence of alphaheavy. On immunohistochemistry, lymphoid cells were positive for leukocyte.
Twentytwo cases of immunoproliferative small intestinal disease ipsid were studied with an im munoperoxidase technique. 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. Immunoproliferative small intestinal disease in algerians. The most common type of heavy chain disease is the iga type, known as. Ipsid is a variant of the bcell lymphoma of mucosaassociated lymphoid tissue malt, which involves mainly the proximal small. 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 small intestinal disease ipsid. The efficacy of combination chemotherapy is also assessed. What does immunoproliferative small intestinal disease mean. Mar 15, 2005 immunoproliferative small intestinal disease ipsid was recently added to the growing list of infectious pathogenassociated human lymphomas. 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.
Summary agroup of 21 patients with immunoproliferative small intestinal disease ipsid, 10 with alpha heavy chain paraproteinaemia and 11 without it, were hlatyped. Immunoproliferative small intestinal disease ipsid is a rare lowgrade bcell lymphoma arising from mucosa associated lymphoid tissue malt, representing approximately onethird of intestinal lymphoma. Immunoproliferative small intestinal disease associated. Immunoproliferative small intestinal disease ipsd is a prevalent, debilitating illness in many developing countries particularly middle east and the. 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. Immunoproliferative small intestinal disease medigoo. 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. Phenotypic analysis and whole genome sequencing identified campylobacter coli. Immunoproliferative small intestinal disease springerlink. Patients and method six patients had immunoproliferative smallintestinal disease ipsid. It predominantly affects young people in the mediterranean region. The radiologic changes in 1090 cases of lymphoma of the small bowel were analyzed and correlated with the pathologic findings. Asignificant increase ofawl9 and b12 antigens was noted amongthe patients compared with the control. Aw19, b12in immunoproliferative small intestinal disease.
Immunoproliferative small intestinal disease associated with. Malt lymphoma called immunoproliferative small intestinal disease. Immunoproliferative small intestinal disease in an american. This study was performed to confirm the existence of immunoproliferative small intestinal disease ipsid in pakistan. Read immunoproliferative small intestinal disease in algerians.
Immunoproliferative small intestinal disease and primary small. 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. A patient with a previous medical history of immunoproliferative small intestinal disease ipsid presented with persistent red and ulcerated lesions on her attached gingiva. Immunoproliferative small intestinal disease ipsid, a term adopted by who in 1978, is a variant of extranodal marginal zone lymphoma of. Nonhodgkin lymphoma causes, risk factors, and prevention. 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. Immunoproliferative small intestinal disease ipsid is a special variant of, extranodal marginal zone bcell lymphoma, which affects the small intestine. Immunoproliferative small intestinal disease and bcell.
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. 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. The infiltrates produces an aberrant immunoglobulin iga, a. Following failure of the tissues to respond to routine periodontal therapy, a gingival biopsy was.
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. Oral manifestation of immunoproliferative small intestinal. Immunoproliferative small intestinal disease associated with overwhelming polymicrobial gastrointestinal infection with transformation to diffuse large b cell. Campylobacter coli cultured from the stools of a patient. Immunoproliferative small intestinal disease without achain. Immunoproliferative small intestinal disease with duodenojejunal. 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.
A group of 21 patients with immunoproliferative small intestinal disease ipsid, 10 with alpha heavy chain paraproteinaemia and 11 without it, were hla typed. 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. Immunoproliferative small intestinal disease ipsid is a prelymphomatous process that is unusual among persons native to the united states. Nonsecretory variant of immunoproliferative small intestinal. Immunoproliferative small intestinal disease ipsid is prevalent in the mediterranean region and in many third world countries but is rare in southeast asia. 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.
A rare mucosal lymphoma of the small intestine, associated with infection with campylobacter jejuni. Clinicopathological features of 12 patients with histologically confirmed disease were analysed. 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. Molecular and immunohistochemical studies demonstrated an association with campylobacter jejuni. Immunoproliferative small intestinal disease and bcell malt.
Immunoproliferative small intestinal diseasea rare extranodal. Immunoproliferative small intestinal diseasea rare. Read immunoproliferative small intestinal disease and primary small intestinal lymphoma. Meaning of immunoproliferative small intestinal disease. Immunoproliferative small intestinal disease definition. Definition of immunoproliferative small intestinal disease in the dictionary. Subsequently most cases are characterized by a loss of ability to synthesize light chains. Immunoproliferative small intestinal disease also known as alpha chain disease is a form of lymphoma that arises in small intestinal mucosaassociated lymphoid tissue malt and is associated. Immunoproliferative small intestine disease definition.
Patients were mostly young males with median age of 24. 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. Khojasteh and colleagues described immunoproliferative small intestinal disease ipsid as a thirdworld lesion june 9 issue. Histopathologically it is characterized by infiltration of small bowel wall with lymphocytes and plasma cells. Immunoproliferative small intestinal disease in an. Pdf on sep 8, 2015, bivas biswas and others published immunoproliferative small intestinal disease. Campylobacter coli cultured from the stools of a patient with. In the recent who classification of hematopoietic and lymphoid tissue, ipsid is considered as a variant of extranodal mucosaassociated lymphoid tissue malt lymphoma.
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