Malignant immunoproliferative diseases and certain other B-cell lymphomas (C88) ICD-10-CM
The C88 code range covers malignant immunoproliferative diseases and certain other b-cell lymphomas with 19 ICD-10-CM diagnosis codes. 12 of them are billable and valid for claim submission in fiscal year 2026, and the category headers group them but cannot themselves be billed.
Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
Codes in the C88 Range 19 codes · 12 billable
- C88 Malignant immunoproliferative diseases and certain other B-cell lymphomasNon-billable
- C88.0 Waldenstrom macroglobulinemiaNon-billable
- C88.00 Waldenstrom macroglobulinemia not having achieved remission
- C88.01 Waldenstrom macroglobulinemia, in remission
- C88.2 Heavy chain diseaseNon-billable
- C88.20 Heavy chain disease not having achieved remission
- C88.21 Heavy chain disease, in remission
- C88.3 Immunoproliferative small intestinal diseaseNon-billable
- C88.30 Immunoproliferative small intestinal disease not having achieved remission
- C88.31 Immunoproliferative small intestinal disease, in remission
- C88.4 Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue [MALT-lymphoma]Non-billable
- C88.40 Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue [MALT-lymphoma] not having achieved remission
- C88.41 Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue [MALT-lymphoma], in remission
- C88.8 Other malignant immunoproliferative diseasesNon-billable
- C88.80 Other malignant immunoproliferative diseases not having achieved remission
- C88.81 Other malignant immunoproliferative diseases, in remission
- C88.9 Malignant immunoproliferative disease, unspecifiedNon-billable
- C88.90 Malignant immunoproliferative disease, unspecified not having achieved remission
- C88.91 Malignant immunoproliferative disease, unspecified, in remission
Clinical Terms in This Code Range
Definitions from the National Library of Medicine for conditions coded in the C88 range.
Heavy Chain Disease
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.
Immunoproliferative Small Intestinal Disease
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.
Waldenstrom Macroglobulinemia
A lymphoproliferative disorder characterized by pleomorphic B-LYMPHOCYTES including PLASMA CELLS, with increased levels of monoclonal serum IMMUNOGLOBULIN M. There is lymphoplasmacytic cells infiltration into bone marrow and often other tissues, also known as lymphoplasmacytic lymphoma. Clinical features include ANEMIA; HEMORRHAGES; and hyperviscosity.
About the C88 Code Range
The ICD-10 code C88 covers malignant immunoproliferative diseases and certain other B-cell lymphomas, conditions characterized by abnormal growth of immune cells, especially B lymphocytes.
This code section specifically includes Waldenstrom macroglobulinemia (C88.0), a disease also known as Macroglobulinemia or lymphoplasmacytic lymphoma, distinguished by excess abnormal antibodies causing symptoms such as neuropathy and kidney issues. It differentiates whether the condition is in remission (C88.01) or not (C88.00). Heavy chain disease (C88.2), with synonyms like Gamma or Mu heavy chain disease, involves abnormal heavy chains of antibodies and can lead to glomerular or nephropathy complications; codes also specify remission status. Immunoproliferative small intestinal disease (C88.3), sometimes called Alpha heavy chain disease, affects the intestines with abnormal heavy chain proteins. Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) (C88.4) refers to a type of non-Hodgkin lymphoma impacting areas like the stomach or orbit, with remission status indicated. Lastly, other malignant immunoproliferative diseases (C88.8) include conditions like light chain disease, again specifying remission. The section also holds codes for unspecified malignant immunoproliferative diseases (C88.9). These codes assist medical professionals in precisely classifying and tracking complex B-cell lymphomas and related immune disorders.
Questions About This Page
How many billable codes are in the C88 range?
Of the 19 codes in this range, 12 are billable and valid for claim submission from October 1, 2025 through September 30, 2026. Category header codes group them but cannot be reported on claims.
What does the C88 range classify?
The range classifies malignant immunoproliferative diseases and certain other b-cell lymphomas. Each code links to its own reference page with billing status, MS-DRG grouping, coding notes, and clinical information.
Related References
Source: CMS FY 2026 ICD-10-CM Tabular List and order file, effective October 1, 2025 through September 30, 2026.
