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 2027, 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.
Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue
An indolent, extranodal type of non-Hodgkin lymphoma composed of small B-lymphocytes (centrocyte-like cells). The gastrointestinal tract is the most common site of involvement. Other common sites of involvement include lung, head and neck, ocular adnexae, skin, thyroid, and breast. Gastric involvement is associated with the presence of H. pylori infection. (WHO, 2001)
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
These cancers include malignant immunoproliferative diseases and certain B-cell lymphomas. They sit within a broader group of cancers of lymph-related and blood-forming tissue.
The subdivisions separate Waldenstrom macroglobulinemia (C88.0), heavy chain disease (C88.2), and immunoproliferative small intestinal disease (C88.3). They also distinguish extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (C88.4), other malignant immunoproliferative diseases (C88.8), and unspecified malignant immunoproliferative disease (C88.9). Each subdivision then separates disease that has not achieved remission from disease in remission.
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, 2026 through September 30, 2027. 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 2027 ICD-10-CM Tabular List and order file, effective October 1, 2026 through September 30, 2027.
