2020 ICD-10-CM Diagnosis Code C88.3

Immunoproliferative small intestinal disease

    2016 2017 2018 2019 2020 Billable/Specific Code
  • C88.3 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 C88.3 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of C88.3 - other international versions of ICD-10 C88.3 may differ.
Applicable To
  • Alpha heavy chain disease
  • Mediterranean lymphoma
The following code(s) above C88.3 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 C88.3:
  • 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
Approximate Synonyms
  • Immunoproliferative small intestinal disease (clinical)
  • Immunoproliferative small intestine disease
Clinical Information
  • A clonal disorder, also known as immunoproliferative small intestinal disease or mediterranean lymphoma, characterised by the secretion of a defective alpha heavy chain. It predominantly affects young people in the mediterranean region. It involves the small intestine, and patients usually present with malabsorption syndrome, abdominal pain, weight loss, and fever. There is extensive villous atrophy of the small intestinal mucosa, which is heavily infiltrated by small lymphocytes and plasma cells. The small intestinal morphologic changes are consistent with a mucosa-associated lymphoid tissue lymphoma (malt lymphoma). Alpha heavy chain disease frequently progresses to large b-cell lymphoma, and normally has a poor prognosis.
  • 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.
ICD-10-CM C88.3 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 C88.3 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 C88.3:
  • Disease, diseased - see also Syndrome
    • heavy chain NEC C88.2
      ICD-10-CM Diagnosis Code C88.2

      Heavy chain disease

        2016 2017 2018 2019 2020 Billable/Specific Code
      Applicable To
      • Franklin disease
      • Gamma heavy chain disease
      • Mu heavy chain disease
      • alpha C88.3
    • immunoproliferative (malignant) C88.9
      ICD-10-CM Diagnosis Code C88.9

      Malignant immunoproliferative disease, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code
      Applicable To
      • Immunoproliferative disease NOS
      • small intestinal C88.3
    • alpha heavy chain C88.3
  • Lymphoma (of) (malignant) C85.90
    ICD-10-CM Diagnosis Code C85.90

    Non-Hodgkin lymphoma, unspecified, unspecified site

      2016 2017 2018 2019 2020 Billable/Specific Code
    • Mediterranean C88.3

ICD-10-CM Codes Adjacent To C88.3
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
C90.10 …… not having achieved remission

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