2025 ICD-10-CM Diagnosis Code C88.3

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Immunoproliferative small intestinal disease

  • C88.3 should not be used for reimbursement purposes as there are multiple codes below it that contain a greater level of detail.
  • ICD-10-CM C88.3 is a new 2025 ICD-10-CM code that became effective on October 1, 2024.
  • This is the American ICD-10-CM version of C88.3 – other international versions of ICD-10 C88.3 may differ.

Applicable To

  • Alpha heavy chain disease
  • Mediterranean lymphoma

The following code(s) above C88.3 contain annotation back-references

 that may be applicable to C88.3:

  • C00-D49  Neoplasms
  • C81-C96  Malignant neoplasms of lymphoid, hematopoietic and related tissue
  • C88  Malignant immunoproliferative diseases and certain other B-cell lymphomas

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.

Code History

  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
  • 2021 (effective 10/1/2020): No change
  • 2022 (effective 10/1/2021): No change
  • 2023 (effective 10/1/2022): No change
  • 2024 (effective 10/1/2023): No change
  • 2025 (effective 10/1/2024): Deleted code
  • 2025 (effective 10/1/2024): New code

Diagnosis Index entries containing back-references to C88.3:

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