alpha heavy chain disease dup disorder

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alpha heavy chain disease dup disorder

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[term] alpha heavy chain disease dup disorder

[id] C0021071
[semantic type] Neoplastic Process
[semantic id] T191
[definition] (MSH) A condition that is caused by HYPERPLASIA of LYMPHOCYTES in the small intestine (INTESTINE, SMALL) and the mesenteric LYMPH NODES. These lymphocytes produce an anomalous alpha heavy chain protein. Generally, these IPSID patients have either concurrent LYMPHOMA or develop lymphoma within a few years. The disease was first described in the Mediterranean region and is characterized by malabsorption; WEIGHT LOSS; DIARRHEA; and STEATORRHEA.
[definition] (NCI) A clonal disorder, also known as immunoproliferative small intestinal disease or Mediterranean lymphoma, characterised by the secretion of a defective alpha heavy chain. It predominantly affects young people in the Mediterranean region. It involves the small intestine, and patients usually present with malabsorption syndrome, abdominal pain, weight loss, and fever. There is extensive villous atrophy of the small intestinal mucosa, which is heavily infiltrated by small lymphocytes and plasma cells. The small intestinal morphologic changes are consistent with a mucosa-associated lymphoid tissue lymphoma (MALT lymphoma). Alpha heavy chain disease frequently progresses to large B-cell lymphoma, and normally has a poor prognosis.
[synonym] ALPHA CHAIN DIS | MSH | ENG | 0 | D007161 | | M0011165 | D007161 | DEV
[synonym] ALPHA CHAIN DISEASE | DXP | ENG | 0 | NOCODE | | | | SY
[synonym] alpha Chain Disease | MSH | ENG | 0 | D007161 | | M0011165 | D007161 | PM
[synonym] alpha Chain Disease | NDFRT | ENG | 1 | C3518 | | C3518 | | SY
[synonym] ALPHA HCD | DXP | ENG | 0 | NOCODE | | | | SY
[synonym] ALPHA HEAVY CHAIN DISEASE | DXP | ENG | 0 | NOCODE | | | | SY
[synonym] Alpha Heavy Chain Disease | NCI | ENG | 0 | C3132 | | C3132 | | PT
[synonym] Alpha heavy chain disease | SNMI | ENG | 9 | M-97623 | | | | PT
[synonym] Alpha heavy chain disease | SNOMEDCT | ENG | 9 | 109982002 | 2659763011 | 109982002 | | SY
[synonym] Alpha heavy chain disease | SNOMEDCT | ENG | 9 | 123312002 | 2765144014 | 123312002 | | OP
[synonym] Alpha heavy chain disease | SNOMEDCT | ENG | 9 | 190819007 | 293381011 | 190819007 | | OP
[synonym] Alpha heavy chain disease | SNOMEDCT | ENG | 9 | 6381009 | 11578015 | 6381009 | | SY
[synonym] Alpha heavy chain disease (clinical) | SNOMEDCT | ENG | 9 | 109982002 | 174678013 | 109982002 | | PT
[synonym] Alpha heavy chain disease (disorder) | SNOMEDCT | ENG | 9 | 109982002 | 617763011 | 109982002 | | FN
[synonym] Alpha heavy chain disease (disorder) | SNOMEDCT | ENG | 9 | 123312002 | 2712535010 | 123312002 | | OF
[synonym] Alpha heavy chain disease (disorder) | SNOMEDCT | ENG | 9 | 190819007 | 2743134017 | 190819007 | | OF
[synonym] Alpha heavy chain disease -RETIRED- | SNOMEDCT | ENG | 9 | 123312002 | 206052012 | 123312002 | | IS
[synonym] Alpha heavy chain disease -RETIRED- | SNOMEDCT | ENG | 9 | 123312002 | 725662012 | 123312002 | | OF
[synonym] Alpha heavy chain disease [dup] (disorder) | SNOMEDCT | ENG | 9 | 190819007 | 574448017 | 190819007 | | OF
[synonym] Alpha heavy chain disease, enteric form | SNMI | ENG | 9 | M-97643 | | | | SY
[synonym] Alpha heavy chain disease, enteric form | SNOMEDCT | ENG | 9 | 123313007 | 189808015 | 123313007 | | PT
[synonym] Alpha heavy chain disease, enteric form | SNOMEDCT | ENG | 9 | 27461004 | 45915018 | 27461004 | | SY
[synonym] Alpha heavy chain disease, enteric form (disorder) | SNOMEDCT | ENG | 9 | 123313007 | 725663019 | 123313007 | | FN
[synonym] Alpha heavy chain disease, NOS | SNOMEDCT | ENG | 9 | 123312002 | 189806016 | 123312002 | | IS
[synonym] alpha-Chain Disease | MSH | ENG | 0 | D007161 | | M0011165 | D007161 | EP
[synonym] alpha-Chain Disease | NDFRT | ENG | 1 | C3518 | | C3518 | | SY
[synonym] alpha-Chain disease | SNM | ENG | 9 | D-4732 | | | | SY
[synonym] alpha-Chain Diseases | MSH | ENG | 0 | D007161 | | M0011165 | D007161 | PM
[synonym] alpha-Chain Diseases | NDFRT | ENG | 1 | C3518 | | C3518 | | SY
[synonym] ALPHA-HEAVY CHAIN DISEASE | DXP | ENG | 0 | NOCODE | | | | SY
[synonym] Alpha-Schwerkettenkrankheit | DMDICD10 | GER | 1 | C88.1 | | | | PT
[synonym] Disease, alpha-Chain | MSH | ENG | 0 | D007161 | | M0011165 | D007161 | PM
[synonym] Disease, alpha-Chain | NDFRT | ENG | 1 | C3518 | | C3518 | | SY
[synonym] Diseases, alpha-Chain | MSH | ENG | 0 | D007161 | | M0011165 | D007161 | PM
[synonym] Diseases, alpha-Chain | NDFRT | ENG | 1 | C3518 | | C3518 | | SY
[synonym] enfermedad de cadena pesada alfa - RETIRADO - | SCTSPA | SPA | 9 | 123312002 | 1124635012 | 123312002 | | OP
[synonym] enfermedad de cadena pesada alfa - RETIRADO - (concepto no activo) | SCTSPA | SPA | 9 | 123312002 | 1124636013 | 123312002 | | OF
[synonym] enfermedad de cadena pesada alfa, forma enterica | SCTSPA | SPA | 9 | 123313007 | | 123313007 | | MTH_PT
[synonym] enfermedad de cadena pesada alfa, forma entérica | SCTSPA | SPA | 9 | 123313007 | 1124637016 | 123313007 | | PT
[synonym] enfermedad de cadena pesada alfa, forma enterica (trastorno) | SCTSPA | SPA | 9 | 123313007 | | 123313007 | | MTH_FN
[synonym] enfermedad de cadena pesada alfa, forma entérica (trastorno) | SCTSPA | SPA | 9 | 123313007 | 1124638014 | 123313007 | | FN
[synonym] enfermedad de cadenas pesadas alfa (clinico) | SCTSPA | SPA | 9 | 109982002 | | 109982002 | | MTH_PT
[synonym] enfermedad de cadenas pesadas alfa (clínico) | SCTSPA | SPA | 9 | 109982002 | 1097651017 | 109982002 | | PT
[synonym] enfermedad de cadenas pesadas alfa (clinico) (trastorno) | SCTSPA | SPA | 9 | 109982002 | | 109982002 | | MTH_FN
[synonym] enfermedad de cadenas pesadas alfa (clínico) (trastorno) | SCTSPA | SPA | 9 | 109982002 | 1097652012 | 109982002 | | FN
[synonym] enfermedad de cadenas pesadas alfa [dup] | SCTSPA | SPA | 9 | 190819007 | 1291931011 | 190819007 | | OP
[synonym] enfermedad de cadenas pesadas alfa [dup] (trastorno) | SCTSPA | SPA | 9 | 190819007 | 1356240014 | 190819007 | | OF
[synonym] enfermedad de cadenas pesadas alfa, forma enterica | SCTSPA | SPA | 9 | 27461004 | | 27461004 | | MTH_SY
[synonym] enfermedad de cadenas pesadas alfa, forma entérica | SCTSPA | SPA | 9 | 27461004 | 911113017 | 27461004 | | SY
[synonym] enfermedad de cadenas pesadas de IgA | SCTSPA | SPA | 9 | 6381009 | 860318011 | 6381009 | | SY
[synonym] enfermedad inmunoproliferativa del intestino delgado | SCTSPA | SPA | 9 | 109985000 | 2591486013 | 109985000 | | SY
[synonym] enfermedad inmunoproliferativa del intestino delgado | SCTSPA | SPA | 9 | 27461004 | 911111015 | 27461004 | | PT
[synonym] enfermedad inmunoproliferativa del intestino delgado (anomalia morfologica) | SCTSPA | SPA | 9 | 27461004 | | 27461004 | | MTH_FN
[synonym] enfermedad inmunoproliferativa del intestino delgado (anomalía morfológica) | SCTSPA | SPA | 9 | 27461004 | 911114011 | 27461004 | | FN
[synonym] enfermedad inmunoproliferativa del intestino delgado (clinica) | SCTSPA | SPA | 9 | 109985000 | | 109985000 | | MTH_PT
[synonym] enfermedad inmunoproliferativa del intestino delgado (clínica) | SCTSPA | SPA | 9 | 109985000 | 1097657018 | 109985000 | | PT
[synonym] enfermedad inmunoproliferativa del intestino delgado (trastorno) | SCTSPA | SPA | 9 | 109985000 | 1097659015 | 109985000 | | FN
[synonym] HEAVY CHAIN DIS IGA TYPE | MSH | ENG | 0 | D007161 | | M0011165 | D007161 | DEV
[synonym] Heavy Chain Disease, IgA Type | MSH | ENG | 0 | D007161 | | M0011165 | D007161 | EP
[synonym] Heavy Chain Disease, IgA Type | NDFRT | ENG | 1 | C3518 | | C3518 | | SY
[synonym] Heavy chain disease, IgA type | SNM | ENG | 9 | D-4732 | | | | PT
[synonym] IGA HEAVY CHAIN DISEASE | DXP | ENG | 0 | NOCODE | | | | SY
[synonym] IgA heavy chain disease | SNOMEDCT | ENG | 9 | 6381009 | 11580014 | 6381009 | | SY
[synonym] IgA heavy chain disease, NOS | SNMI | ENG | 9 | M-97623 | | | | SY
[synonym] IgA heavy chain disease, NOS | SNOMEDCT | ENG | 9 | 123312002 | 189807013 | 123312002 | | IS
[synonym] IgA heavy chain disease, NOS | SNOMEDCT | ENG | 9 | 6381009 | 11579011 | 6381009 | | IS
[synonym] IMMUNOPROLIFERATIVE SMALL INTESTINAL DIS | MSH | ENG | 0 | D007161 | | M0011165 | D007161 | DEV
[synonym] Immunoproliferative Small Intestinal Disease | MSH | ENG | 0 | D007161 | | M0011165 | D007161 | MH
[synonym] Immunoproliferative Small Intestinal Disease | NCI | ENG | 0 | C3132 | | C3132 | | SY
[synonym] Immunoproliferative Small Intestinal Disease | NDFRT | ENG | 1 | C3518 | | C3518 | | PT
[synonym] Immunoproliferative small intestinal disease | SNMI | ENG | 9 | M-97643 | | | | PT
[synonym] Immunoproliferative small intestinal disease | SNOMEDCT | ENG | 9 | 109985000 | 2659694018 | 109985000 | | SY
[synonym] Immunoproliferative small intestinal disease | SNOMEDCT | ENG | 9 | 123313007 | 189809011 | 123313007 | | IS
[synonym] Immunoproliferative small intestinal disease | SNOMEDCT | ENG | 9 | 27461004 | 45913013 | 27461004 | | PT
[synonym] Immunoproliferative small intestinal disease (clinical) | SNOMEDCT | ENG | 9 | 109985000 | 174683017 | 109985000 | | PT
[synonym] Immunoproliferative small intestinal disease (disorder) | SNOMEDCT | ENG | 9 | 109985000 | 617796013 | 109985000 | | FN
[synonym] Immunoproliferative small intestinal disease (morphologic abnormality) | SNOMEDCT | ENG | 9 | 27461004 | 758031012 | 27461004 | | FN
[synonym] Immunoproliferative Small Intestinal Disease [Disease/Finding] | NDFRT | ENG | 1 | C3518 | | C3518 | | FN
[synonym] IMMUNOPROLIFERATIVE SMALL INTESTINE DISEASE | DXP | ENG | 0 | NOCODE | | | | SY
[synonym] Immunproliferative Duenndarmkrankheit | DMDICD10 | GER | 1 | C88.3 | | | | PT
[synonym] IPSID | DXP | ENG | 0 | NOCODE | | | | SY
[synonym] IPSID | MSH | ENG | 0 | D007161 | | M0011165 | D007161 | EP
[synonym] IPSID | NCI | ENG | 0 | C3132 | | C3132 | | AB
[synonym] IPSID | NDFRT | ENG | 1 | C3518 | | C3518 | | SY
[synonym] linfoma del Mediterraneo | SCTSPA | SPA | 9 | 27461004 | | 27461004 | | MTH_SY
[synonym] linfoma del Mediterráneo | SCTSPA | SPA | 9 | 27461004 | 911112010 | 27461004 | | SY
[synonym] linfoma del Mediterraneo (clinico) | SCTSPA | SPA | 9 | 109985000 | | 109985000 | | MTH_SY
[synonym] linfoma del Mediterráneo (clínico) | SCTSPA | SPA | 9 | 109985000 | 1097658011 | 109985000 | | SY
[synonym] Lymphoma, Mediterranean | MSH | ENG | 0 | D007161 | | M0011165 | D007161 | EP
[synonym] Lymphoma, Mediterranean | NDFRT | ENG | 1 | C3518 | | C3518 | | SY
[synonym] Mediterranean Abdominal Lymphoma | NCI | ENG | 0 | C3132 | | C3132 | | SY
[synonym] Mediterranean Lymphoma | MSH | ENG | 0 | D007161 | | M0011165 | D007161 | PM
[synonym] Mediterranean Lymphoma | NDFRT | ENG | 1 | C3518 | | C3518 | | SY
[synonym] Mediterranean lymphoma | SNMI | ENG | 9 | M-97643 | | | | SY
[synonym] Mediterranean lymphoma | SNOMEDCT | ENG | 9 | 123313007 | 189810018 | 123313007 | | IS
[synonym] Mediterranean lymphoma | SNOMEDCT | ENG | 9 | 27461004 | 45914019 | 27461004 | | SY
[synonym] Mediterranean lymphoma (clinical) | SNOMEDCT | ENG | 9 | 109985000 | 174684011 | 109985000 | | SY
[synonym] Mediterraneanl Lymphoma | NCI | ENG | 0 | C3132 | | C3132 | | SY
[synonym] [M] Alpha heavy chain disease | SNOMEDCT | ENG | 9 | 190009000 | 291960011 | 190009000 | | OP
[synonym] [M] Alpha heavy chain disease (morphologic abnormality) | SNOMEDCT | ENG | 9 | 190009000 | 573534010 | 190009000 | | OF
[synonym] [M] enfermedad de cadenas pesadas alfa | SCTSPA | SPA | 9 | 190009000 | 1314034013 | 190009000 | | OP
[synonym] [M] enfermedad de cadenas pesadas alfa (anomalia morfologica) | SCTSPA | SPA | 9 | 190009000 | | 190009000 | | MTH_OF
[synonym] [M] enfermedad de cadenas pesadas alfa (anomalía morfológica) | SCTSPA | SPA | 9 | 190009000 | 1378197013 | 190009000 | | OF
[synonym] [M] enfermedad inmunoproliferativa del intestino delgado | SCTSPA | SPA | 9 | 134181009 | 1313632018 | 134181009 | | OP
[synonym] [M] enfermedad inmunoproliferativa del intestino delgado (anomalia morfologica) | SCTSPA | SPA | 9 | 134181009 | | 134181009 | | MTH_OF
[synonym] [M] enfermedad inmunoproliferativa del intestino delgado (anomalía morfológica) | SCTSPA | SPA | 9 | 134181009 | 1377795011 | 134181009 | | OF
[synonym] [M] Immunoproliferative small intestinal disease | SNOMEDCT | ENG | 9 | 134181009 | 215918011 | 134181009 | | OP
[synonym] [M] Immunoproliferative small intestinal disease (morphologic abnormality) | SNOMEDCT | ENG | 9 | 134181009 | 513758010 | 134181009 | | OF
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