C88.3

Applicable To

• Alpha heavy chain disease

• Mediterranean lymphoma

Approximate Synonyms

• Immunoproliferative small intestinal disease clinical)

• Immunoproliferative small intestine disease

Clinical Information

• 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.

• 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.

Details

• C88.3 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.

• The 2023 edition of ICD-10-CM C88.3 became effective on October 1, 2022.

• This is the American ICD-10-CM version of C88.3 – other international versions of ICD-10 C88.3 may differ.