C88.0Waldenstrom macroglobulinemia

C88.0 is the ICD-10-CM diagnosis code for Waldenstrom macroglobulinemia. It belongs to category C88 (malignant immunoproliferative diseases and certain other B-cell lymphomas), block C81-C96 (malignant neoplasms of lymphoid, hematopoietic and related tissue) and chapter 2 (neoplasms). It is a header code, so it can't be reported by itself; one of its 2 subcodes must be used instead.

Within C88 (malignant immunoproliferative diseases and certain other B-cell lymphomas), C88.0 is specifically for Waldenstrom macroglobulinemia. Related codes cover heavy chain disease (C88.2), immunoproliferative small intestinal disease (C88.3), extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue [MALT-lymphoma] (C88.4), other malignant immunoproliferative diseases (C88.8) and malignant immunoproliferative disease, unspecified (C88.9).

To report this condition, pick the subcode that matches the documentation: C88.00 (not having achieved remission) and C88.01 (in remission).

Don't report C88.0 together with the conditions in its Excludes1 note: small cell B-cell lymphoma (C83.0).

It has been part of ICD-10-CM since the code set took effect on October 1, 2015, and changed in FY2025 (expanded into subcodes).

Before ICD-10, this condition was coded in ICD-9-CM as 273.3 (macroglobulinemia).