About: Alpha Heavy Chain Disease     Goto   Sponge   NotDistinct   Permalink

An Entity of Type : owl:Class, within Data Space : linked.opendata.cz associated with source document(s)

AttributesValues
rdf:type
rdfs:label
  • Alpha Heavy Chain Disease
equivalentClass
Semantic_Type
  • Neoplastic Process
Preferred_Name
  • Alpha Heavy Chain Disease
UMLS_CUI
  • C0021071
ICD-O-3_Code
  • 9760/3
  • 9764/3
Neoplastic_Status
  • Malignant
Legacy_Concept_Name
  • Alpha_Heavy_Chain_Disease
FULL_SYN
  • Mediterranean Abdominal LymphomaSYNCI
  • Mediterraneanl LymphomaSYNCI
  • Immunoproliferative Small Intestinal DiseaseSYNCI
  • Alpha Heavy Chain DiseasePTNCI
  • IPSIDABNCI
DEFINITION
  • 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.NCI
code
  • C3132
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