C88.2

Applicable To

• Franklin disease

• Gamma heavy chain disease

• Mu heavy chain disease

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.

Details

• C88.2 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 C88.2 became effective on October 1, 2022.

• This is the American ICD-10-CM version of C88.2 – other international versions of ICD-10 C88.2 may differ.