2020 ICD-10-CM Diagnosis Code C88.2

Heavy chain disease

    2016 2017 2018 2019 2020 Billable/Specific Code
  • C88.2 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2020 edition of ICD-10-CM C88.2 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of C88.2 - other international versions of ICD-10 C88.2 may differ.
Applicable To
  • Franklin disease
  • Gamma heavy chain disease
  • Mu heavy chain disease
The following code(s) above C88.2 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to C88.2:
  • C00-D49
    2020 ICD-10-CM Range C00-D49

    Neoplasms

    Note
    • Functional activity
    • All neoplasms are classified in this chapter, whether they are functionally active or not. An additional code from Chapter 4 may be used, to identify functional activity associated with any neoplasm.
    • Morphology [Histology]
    • Chapter 2 classifies neoplasms primarily by site (topography), with broad groupings for behavior, malignant, in situ, benign, etc. The Table of Neoplasms should be used to identify the correct topography code. In a few cases, such as for malignant melanoma and certain neuroendocrine tumors, the morphology (histologic type) is included in the category and codes.
    • Primary malignant neoplasms overlapping site boundaries
    • A primary malignant neoplasm that overlaps two or more contiguous (next to each other) sites should be classified to the subcategory/code .8 ('overlapping lesion'), unless the combination is specifically indexed elsewhere. For multiple neoplasms of the same site that are not contiguous, such as tumors in different quadrants of the same breast, codes for each site should be assigned.
    • Malignant neoplasm of ectopic tissue
    • Malignant neoplasms of ectopic tissue are to be coded to the site mentioned, e.g., ectopic pancreatic malignant neoplasms are coded to pancreas, unspecified (C25.9).
    Neoplasms
  • C81-C96
    2020 ICD-10-CM Range C81-C96

    Malignant neoplasms of lymphoid, hematopoietic and related tissue

    Type 2 Excludes
    • Kaposi's sarcoma of lymph nodes (C46.3)
    • secondary and unspecified neoplasm of lymph nodes (C77.-)
    • secondary neoplasm of bone marrow (C79.52)
    • secondary neoplasm of spleen (C78.89)
    Malignant neoplasms of lymphoid, hematopoietic and related tissue
  • C88
    ICD-10-CM Diagnosis Code C88

    Malignant immunoproliferative diseases and certain other B-cell lymphomas

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • B-cell lymphoma, unspecified (C85.1-)
    • personal history of other malignant neoplasms of lymphoid, hematopoietic and related tissues (Z85.79)
    Malignant immunoproliferative diseases and certain other B-cell lymphomas
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.
ICD-10-CM C88.2 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 820 Lymphoma and leukemia with major o.r. Procedure with mcc
  • 821 Lymphoma and leukemia with major o.r. Procedure with cc
  • 822 Lymphoma and leukemia with major o.r. Procedure without cc/mcc
  • 823 Lymphoma and non-acute leukemia with other procedure with mcc
  • 824 Lymphoma and non-acute leukemia with other procedure with cc
  • 825 Lymphoma and non-acute leukemia with other procedure without cc/mcc
  • 840 Lymphoma and non-acute leukemia with mcc
  • 841 Lymphoma and non-acute leukemia with cc
  • 842 Lymphoma and non-acute leukemia without cc/mcc

Convert C88.2 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change

Diagnosis Index entries containing back-references to C88.2:

ICD-10-CM Codes Adjacent To C88.2
C86 Other specified types of T/NK-cell lymphoma
C86.0 Extranodal NK/T-cell lymphoma, nasal type
C86.1 Hepatosplenic T-cell lymphoma
C86.2 Enteropathy-type (intestinal) T-cell lymphoma
C86.3 Subcutaneous panniculitis-like T-cell lymphoma
C86.4 Blastic NK-cell lymphoma
C86.5 Angioimmunoblastic T-cell lymphoma
C86.6 Primary cutaneous CD30-positive T-cell proliferations
C88 Malignant immunoproliferative diseases and certain other B-cell lymphomas
C88.0 Waldenström macroglobulinemia
C88.2 Heavy chain disease
C88.3 Immunoproliferative small intestinal disease
C88.4 Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue [MALT-lymphoma]
C88.8 Other malignant immunoproliferative diseases
C88.9 Malignant immunoproliferative disease, unspecified
C90 Multiple myeloma and malignant plasma cell neoplasms
C90.0 Multiple myeloma
C90.00 …… not having achieved remission
C90.01 …… in remission
C90.02 …… in relapse
C90.1 Plasma cell leukemia

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.