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  5. Malignant immunoproliferative diseases and certain other B-cell lymphomas (C88)

Malignant immunoproliferative diseases and certain other B-cell lymphomas (C88)

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.

  • Neoplasms (C00–D49)

    • Malignant neoplasms of lymphoid, hematopoietic and related tissue (C81-C96)

        • Malignant immunoproliferative diseases and certain other B-cell lymphomas (C88)

        • C88 Malignant immunoproliferative diseases and certain other B-cell lymphomas
        • C88.0 Waldenstrom macroglobulinemia DELETED CODE
        • C88.00 Waldenstrom macroglobulinemia not having achieved remission NEW CODE
        • C88.01 Waldenstrom macroglobulinemia, in remission NEW CODE
        • C88.2 Heavy chain disease DELETED CODE
        • C88.20 Heavy chain disease not having achieved remission NEW CODE
        • C88.21 Heavy chain disease, in remission NEW CODE
        • C88.3 Immunoproliferative small intestinal disease DELETED CODE
        • C88.30 Immunoproliferative small intestinal disease not having achieved remission NEW CODE
        • C88.31 Immunoproliferative small intestinal disease, in remission NEW CODE
        • C88.4 Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue [MALT-lymphoma] DELETED CODE
        • C88.40 Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue [MALT-lymphoma] not having achieved remission NEW CODE
        • C88.41 Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue [MALT-lymphoma], in remission NEW CODE
        • C88.8 Other malignant immunoproliferative diseases DELETED CODE
        • C88.80 Other malignant immunoproliferative diseases not having achieved remission NEW CODE
        • C88.81 Other malignant immunoproliferative diseases, in remission NEW CODE
        • C88.9 Malignant immunoproliferative disease, unspecified DELETED CODE
        • C88.90 Malignant immunoproliferative disease, unspecified not having achieved remission NEW CODE
        • C88.91 Malignant immunoproliferative disease, unspecified, in remission NEW CODE

Instructional Notations

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.

  • B-cell lymphoma, unspecified C85.1
  • personal history of other malignant neoplasms of lymphoid, hematopoietic and related tissues Z85.79

Clinical Terms

The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.

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.