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Alpha-heavy chain disease

MedGen UID:
7039
•Concept ID:
C0021071
•
Neoplastic Process
Synonyms: alpha Chain Disease; alpha-Chain Disease; alpha-Chain Diseases; Disease, alpha-Chain; Diseases, alpha-Chain; Heavy Chain Disease, IgA Type; Immunoproliferative Small Intestinal Disease; IPSID; Lymphoma, Mediterranean; Mediterranean Lymphoma
SNOMED CT: Alpha heavy chain disease (6381009); IgA heavy chain disease (6381009); Alpha heavy chain disease (clinical) (109982002); Immunoproliferative small intestinal disease (clinical) (109985000); Mediterranean lymphoma (clinical) (109985000); Alpha heavy chain disease, enteric form (123313007); Immunoproliferative small intestinal disease (109985000); Alpha heavy chain disease (109982002); Immunoproliferative small intestinal disease (27461004); Mediterranean lymphoma (27461004); Alpha heavy chain disease, enteric form (27461004)
 
Monarch Initiative: MONDO:0015045
Orphanet: ORPHA100025

Definition

A type of HCD characterized by the production of incomplete monoclonal alpha-heavy chains without associated light chains. Alpha-HCD is considered to be a subtype of immunoproliferative small intestinal disease (IPSID). The clinical presentation includes chronic diarrhea with evidence of malabsorption. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAlpha-heavy chain disease

Professional guidelines

PubMed

Alpha-heavy chain disease, Mediterranean lymphoma, and immunoproliferative small intestinal disease: a review of clinicopathological features, pathogenesis, and differential diagnosis.
Fine KD, Stone MJ
Am J Gastroenterol 1999 May;94(5):1139-52. doi: 10.1111/j.1572-0241.1999.01057.x. PMID: 10235185

Recent clinical studies

Etiology

Alpha-heavy chain disease, Mediterranean lymphoma, and immunoproliferative small intestinal disease: a review of clinicopathological features, pathogenesis, and differential diagnosis.
Fine KD, Stone MJ
Am J Gastroenterol 1999 May;94(5):1139-52. doi: 10.1111/j.1572-0241.1999.01057.x. PMID: 10235185
Immunoproliferative small intestinal disease: Mediterranean lymphoma and alpha heavy chain disease.
Martin IG, Aldoori MI
Br J Surg 1994 Jan;81(1):20-4. doi: 10.1002/bjs.1800810107. PMID: 8313111
Primary small intestinal lymphomas and alpha-heavy-chain disease. A study of 43 cases from a pathology department in Israel.
Selzer G, Sherman G, Callihan TR, Schwartz Y
Isr J Med Sci 1979 Feb;15(2):111-23. PMID: 112083
Alpha heavy-chain disease in southern Iran.
Haghshenass M, Haghighi P, Abadi P, Kharazmi A, Gerami C, Nasr K
Am J Dig Dis 1977 Oct;22(10):866-73. doi: 10.1007/BF01076161. PMID: 411371
Middle Eastern intestinal lymphoma: report of a case and review of the literature.
Zarrabi MH, Rosner F
Am J Med Sci 1976 Jul-Aug;272(1):101-19. doi: 10.1097/00000441-197607000-00013. PMID: 786012

Diagnosis

The Evolution of Our Understanding of Immunoproliferative Small Intestinal Disease (IPSID) over Time.
AlYamany R, Kharfan-Dabaja MA, Hamadani M, Alshaibani A, Aljurf M
Curr Oncol 2022 May 23;29(5):3759-3769. doi: 10.3390/curroncol29050301. PMID: 35621691Free PMC Article
Alpha-heavy chain disease, Mediterranean lymphoma, and immunoproliferative small intestinal disease: a review of clinicopathological features, pathogenesis, and differential diagnosis.
Fine KD, Stone MJ
Am J Gastroenterol 1999 May;94(5):1139-52. doi: 10.1111/j.1572-0241.1999.01057.x. PMID: 10235185
Immunoproliferative small intestinal disease: Mediterranean lymphoma and alpha heavy chain disease.
Martin IG, Aldoori MI
Br J Surg 1994 Jan;81(1):20-4. doi: 10.1002/bjs.1800810107. PMID: 8313111
Gastric alpha heavy chain disease.
Tungekar MF, Omar YT, Behbehani K
Oncology 1987;44(6):360-6. doi: 10.1159/000226512. PMID: 3120071
Alpha heavy chain disease (report of 18 cases from Iraq).
Al-Bahrani Z, Al-Saleem T, Al-Mondhiry H, Bakir F, Yahia H, Taha I, King J
Gut 1978 Jul;19(7):627-31. doi: 10.1136/gut.19.7.627. PMID: 98395Free PMC Article

Therapy

Heavy Chain Disease of the Small Bowel.
Bianchi G, Sohani AR
Curr Gastroenterol Rep 2018 Jan 25;20(1):3. doi: 10.1007/s11894-018-0608-y. PMID: 29372346
Severe enteropathy caused by α-heavy chain disease lacking detectable M-proteins.
Kurimoto M, Sonoki T, Nakamura Y, Yamamoto Y, Maeda Y, Kuriyama K, Mushino T, Hosoi H, Murata S, Shimanuki M, Hatanaka K, Hanaoka N, Kato J, Nakakuma H
Intern Med 2014;53(6):581-5. doi: 10.2169/internalmedicine.53.1625. PMID: 24633028
Severe malnutrition associated with alpha-heavy chain disease: response to tetracycline and intensive nutritional support.
O'Keefe SJ, Winter TA, Newton KA, Ogden JM, Young GO, Price SK
Am J Gastroenterol 1988 Sep;83(9):995-1001. PMID: 3414653
Alpha heavy chain disease.
Seligmann M
Blut 1975 Jul;31(1):1-4. doi: 10.1007/BF01635434. PMID: 1148399
Abdominal lymphoma with alpha-heavy chain disease.
Plesnicar S, Sumi-Kriznik T, Golouh R
Isr J Med Sci 1975 Aug;11(8):832-7. PMID: 809383

Prognosis

Heavy Chain Disease of the Small Bowel.
Bianchi G, Sohani AR
Curr Gastroenterol Rep 2018 Jan 25;20(1):3. doi: 10.1007/s11894-018-0608-y. PMID: 29372346
Immunoproliferative small intestinal disease: Mediterranean lymphoma and alpha heavy chain disease.
Martin IG, Aldoori MI
Br J Surg 1994 Jan;81(1):20-4. doi: 10.1002/bjs.1800810107. PMID: 8313111
New presentation of alpha heavy chain disease: North American polypoid gastrointestinal lymphoma. Clinical and cellular studies.
Cohen HJ, Gonzalvo A, Krook J, Thompson TT, Kremer WB
Cancer 1978 Mar;41(3):1161-9. doi: 10.1002/1097-0142(197803)41:3<1161::aid-cncr2820410354>3.0.co;2-j. PMID: 416900
Alpha heavy chain disease (report of 18 cases from Iraq).
Al-Bahrani Z, Al-Saleem T, Al-Mondhiry H, Bakir F, Yahia H, Taha I, King J
Gut 1978 Jul;19(7):627-31. doi: 10.1136/gut.19.7.627. PMID: 98395Free PMC Article
Alpha heavy chain disease.
Seligmann M
Blut 1975 Jul;31(1):1-4. doi: 10.1007/BF01635434. PMID: 1148399

Clinical prediction guides

Alpha heavy chain disease alpha mRNA contain nucleotide sequences of unknown origins.
Fakhfakh F, Dellagi K, Ayadi H, Bouguerra A, Fourati R, Ben Ayed F, Brouet JC, Tsapis A
Eur J Immunol 1992 Nov;22(11):3037-40. doi: 10.1002/eji.1830221141. PMID: 1425927
Immunoproliferative small intestinal disease: a study of 13 cases with alpha heavy-chain disease.
Price SK
Histopathology 1990 Jul;17(1):7-17. doi: 10.1111/j.1365-2559.1990.tb00658.x. PMID: 2227833
Alpha heavy chain disease lacking secretory alpha chain, with cobblestone appearance of the small intestine and duodenal ulcer demonstrated by endoscopy.
Hibi T, Asakura H, Kobayashi K, Munakata Y, Kano S, Tsuchiya M, Teramoto T, Uematsu Y
Gut 1982 May;23(5):422-7. doi: 10.1136/gut.23.5.422. PMID: 6804314Free PMC Article
Follicular centre cell lymphoma with alpha heavy chain disease. A histopathological and immunohistological study.
Nemes Z, Thomázy V, Szeifert G
Virchows Arch A Pathol Anat Histol 1981;394(1-2):119-32. doi: 10.1007/BF00431670. PMID: 6801847
Alpha heavy chain disease (report of 18 cases from Iraq).
Al-Bahrani Z, Al-Saleem T, Al-Mondhiry H, Bakir F, Yahia H, Taha I, King J
Gut 1978 Jul;19(7):627-31. doi: 10.1136/gut.19.7.627. PMID: 98395Free PMC Article

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