2025 ICD-10-CM Diagnosis Code C88.2

by

Heavy chain disease

  • C88.2 should not be used for reimbursement purposes as there are multiple codes below it that contain a greater level of detail.
  • ICD-10-CM C88.2 is a new 2025 ICD-10-CM code that became effective on October 1, 2024.
  • This is the American ICD-10-CM version of C88.2 – other international versions of ICD-10 C88.2 may differ.

Applicable To

  • Franklin disease
  • Gamma heavy chain disease
  • Mu heavy chain disease

The following code(s) above C88.2 contain annotation back-references

 that may be applicable to C88.2:

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

Clinical Information

  • A clonal disorder characterized by the secretion of a mu heavy chain that lacks a variable region. Most patients present with slowly progressive chronic lymphocytic leukemia (cll). In contrast to most cases of cll, mu heavy chain disease is associated with hepatosplenomegaly and absence of lymphadenopathy.
  • A clonal disorder characterized by the secretion of a truncated gamma chain. In most cases, it is associated with morphologic changes also seen in lymphoplasmacytic lymphomas, but the clinical course is typically more aggressive than in lymphoplasmacytic lymphoma/waldenstrom’s macroglobulinemia.
  • A disorder of immunoglobulin synthesis in which large quantities of abnormal heavy chains are excreted in the urine. The amino acid sequences of the n-(amino-) terminal regions of these chains are normal, but they have a deletion extending from part of the variable domain through the first domain of the constant region, so that they cannot form cross-links to the light chains. The defect arises through faulty coupling of the variable (v) and constant (c) region genes.
  • Disorder of immunoglobulin synthesis in which large quantities of abnormal heavy chains are excreted in the urine; amino acid sequences of the n-(amino-) terminal regions of these chains are normal, but they have a deletion extending from part of the variable domain through the first domain of the constant region, so that they cannot form cross-links to the light chains; the defect arises through faulty coupling of the variable (v) and constant (c) region genes.
  • One of a group of rare disorders of immunoglobulin synthesis associated with b-cell proliferative disorders and diagnosed by the finding of heavy chains in the serum, urine, or both. Heavy chains are immunologically related to the fc fragment of the immunoglobulin chain. Three types of heavy chain disease (hcd) have been recognized: gamma heavy chain disease (associated with waldenstrom’s macroglobulinemia), alpha heavy chain disease (also known as immunoproliferative small intestinal disease or mediterranean lymphoma), and mu heavy chain disease (associated with chronic lymphocytic leukemia). Complications of hcd vary depending upon type.

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 C88.2:

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