2019 ICD-10-CM Diagnosis Code C96.0

Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis

    2016 2017 2018 2019 Billable/Specific Code
  • C96.0 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • Short description: Multifocal and multisystemic Langerhans-cell histiocytosis
  • The 2019 edition of ICD-10-CM C96.0 became effective on October 1, 2018.
  • This is the American ICD-10-CM version of C96.0 - other international versions of ICD-10 C96.0 may differ.
Applicable To
  • Histiocytosis X, multisystemic
  • Letterer-Siwe disease
Type 1 Excludes
Type 1 Excludes Help
A type 1 excludes note is a pure excludes. It means "not coded here". A type 1 excludes note indicates that the code excluded should never be used at the same time as C96.0. A type 1 excludes note is for used for when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
  • adult pulmonary Langerhans cell histiocytosis (
    ICD-10-CM Diagnosis Code J84.82

    Adult pulmonary Langerhans cell histiocytosis

      2016 2017 2018 2019 Billable/Specific Code Adult Dx (15-124 years)
    Applicable To
    • Adult PLCH
    J84.82
    )
  • multifocal and unisystemic Langerhans-cell histiocytosis (
    ICD-10-CM Diagnosis Code C96.5

    Multifocal and unisystemic Langerhans-cell histiocytosis

      2016 2017 2018 2019 Billable/Specific Code
    Applicable To
    • Hand-Schüller-Christian disease
    • Histiocytosis X, multifocal
    Type 1 Excludes
    • multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis (C96.0)
    • unifocal Langerhans-cell histiocytosis (C96.6)
    C96.5
    )
  • unifocal Langerhans-cell histiocytosis (
    ICD-10-CM Diagnosis Code C96.6

    Unifocal Langerhans-cell histiocytosis

      2016 2017 2018 2019 Billable/Specific Code
    Applicable To
    • Eosinophilic granuloma
    • Histiocytosis X, unifocal
    • Histiocytosis X NOS
    • Langerhans-cell histiocytosis NOS
    Type 1 Excludes
    • multifocal and multisysemic (disseminated) Langerhans-cell histiocytosis (C96.0)
    • multifocal and unisystemic Langerhans-cell histiocytosis (C96.5)
    C96.6
    )
The following code(s) above C96.0 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 C96.0:
  • C00-D49
    2019 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
    2019 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
  • C96
    ICD-10-CM Diagnosis Code C96

    Other and unspecified malignant neoplasms of lymphoid, hematopoietic and related tissue

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • personal history of other malignant neoplasms of lymphoid, hematopoietic and related tissues (Z85.79)
    Other and unspecified malignant neoplasms of lymphoid, hematopoietic and related tissue
Approximate Synonyms
  • Langerhans cell histiocytosis, disseminated (clinical)
  • Letterer-siwe disease of intra-abdominal lymph nodes
  • Letterer-siwe disease of intrapelvic lymph nodes
  • Letterer-siwe disease of intrathoracic lymph nodes
  • Letterer-siwe disease of lymph nodes of axilla
  • Letterer-siwe disease of lymph nodes of head
  • Letterer-siwe disease of lymph nodes of inguinal region
  • Letterer-siwe disease of lymph nodes of lower limb
  • Letterer-siwe disease of lymph nodes of multiple sites
  • Letterer-siwe disease of lymph nodes of neck
  • Letterer-siwe disease of lymph nodes of upper limb
  • Letterer-siwe disease of spleen
  • Letterer-siwe disease, arm ln
  • Letterer-siwe disease, axillary ln
  • Letterer-siwe disease, head ln
  • Letterer-siwe disease, inguinal ln
  • Letterer-siwe disease, leg ln
  • Letterer-siwe disease, neck ln
  • Lymphoma disseminated langerhans cell histiocytosi
  • Lymphoma intra abdominal lymph node, letterer siwe
  • Lymphoma intrapelvic lymph node, letterer siwe
  • Lymphoma intrathoracic lymph node, letterer siwe
  • Lymphoma multiple sites lymph node, letterer siwe
  • Lymphoma spleen, letterer siwe
Clinical Information
  • A group of disorders resulting from the abnormal proliferation of and tissue infiltration by langerhans cells which can be detected by their characteristic birbeck granules (x bodies), or by monoclonal antibody staining for their surface cd1 antigens. Langerhans-cell granulomatosis can involve a single organ, or can be a systemic disorder.
  • A group of rare disorders in which too many langerhans cells (a type of white blood cell) grow in certain tissues and organs including the bones, skin, and lungs, and damage them. Langerhans cell histiocytosis may also affect the pituitary gland (which makes hormones that control other glands and many body functions, especially growth). Langerhans cell histiocytosis is most common in children and young adults.
  • A multifocal, multisystem form of langerhans-cell histiocytosis. There is involvement of multiple organ systems including the bones, skin, liver, spleen, and lymph nodes. Patients are usually infants presenting with fever, hepatosplenomegaly, lymphadenopathy, bone and skin lesions, and pancytopenia.
  • A multifocal, unisystem form of langerhans-cell histiocytosis. There is involvement of multiple sites in one organ system, most frequently the bone. Patients are usually young children presenting with multiple destructive bone lesions.
  • A neoplastic proliferation of langerhans cells which contain birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognized: eosinophilic granuloma, letterer-siwe disease, and hand-schuller-christian disease. The clinical course is generally related to the number of organs affected at presentation. (who, 2001)
  • Group of disorders of histiocyte proliferation which includes letterer-siwe disease; hand-schueller-christian syndrome; and eosinophilic granuloma; langerhans cells are components of the lesions.
ICD-10-CM C96.0 is grouped within Diagnostic Related Group(s) (MS-DRG v36.0):
  • 011 Tracheostomy for face,mouth & neck diagnoses or laryngectomy with mcc
  • 012 Tracheostomy for face,mouth & neck diagnoses or laryngectomy with cc
  • 013 Tracheostomy for face,mouth & neck diagnoses or laryngectomy without cc/mcc
  • 826 Myeloproliferative disorders or poorly differentiated neoplasms with major o.r. Procedure with mcc
  • 827 Myeloproliferative disorders or poorly differentiated neoplasms with major o.r. Procedure with cc
  • 828 Myeloproliferative disorders or poorly differentiated neoplasms with major o.r. Procedure without cc/mcc
  • 829 Myeloproliferative disorders or poorly differentiated neoplasms with other procedure with cc/mcc
  • 830 Myeloproliferative disorders or poorly differentiated neoplasms with other procedure without cc/mcc
  • 843 Other myeloproliferative disorders or poorly differentiated neoplastic diagnoses with mcc
  • 844 Other myeloproliferative disorders or poorly differentiated neoplastic diagnoses with cc
  • 845 Other myeloproliferative disorders or poorly differentiated neoplastic diagnoses without cc/mcc

Convert C96.0 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
Code annotations containing back-references to C96.0:
  • Code First: N16
    ICD-10-CM Diagnosis Code N16

    Renal tubulo-interstitial disorders in diseases classified elsewhere

      2016 2017 2018 2019 Billable/Specific Code Manifestation Code
    Applicable To
    • Pyelonephritis
    • Tubulo-interstitial nephritis
    Code First
    Type 1 Excludes
    • diphtheritic pyelonephritis and tubulo-interstitial nephritis (A36.84)
    • pyelonephritis and tubulo-interstitial nephritis in candidiasis (B37.49)
    • pyelonephritis and tubulo-interstitial nephritis in cystinosis (E72.04)
    • pyelonephritis and tubulo-interstitial nephritis in salmonella infection (A02.25)
    • pyelonephritis and tubulo-interstitial nephritis in sarcoidosis (D86.84)
    • pyelonephritis and tubulo-interstitial nephritis in sicca syndrome [Sjogren's] (M35.04)
    • pyelonephritis and tubulo-interstitial nephritis in systemic lupus erythematosus (M32.15)
    • pyelonephritis and tubulo-interstitial nephritis in toxoplasmosis (B58.83)
    • renal tubular degeneration in diabetes (E08-E13 with .29)
    • syphilitic pyelonephritis and tubulo-interstitial nephritis (A52.75)
  • Type 1 Excludes: C96.5
    , C96.6
    , D76
    , D76
    ICD-10-CM Diagnosis Code C96.5

    Multifocal and unisystemic Langerhans-cell histiocytosis

      2016 2017 2018 2019 Billable/Specific Code
    Applicable To
    • Hand-Schüller-Christian disease
    • Histiocytosis X, multifocal
    Type 1 Excludes
    • multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis (C96.0)
    • unifocal Langerhans-cell histiocytosis (C96.6)
    ICD-10-CM Diagnosis Code C96.6

    Unifocal Langerhans-cell histiocytosis

      2016 2017 2018 2019 Billable/Specific Code
    Applicable To
    • Eosinophilic granuloma
    • Histiocytosis X, unifocal
    • Histiocytosis X NOS
    • Langerhans-cell histiocytosis NOS
    Type 1 Excludes
    • multifocal and multisysemic (disseminated) Langerhans-cell histiocytosis (C96.0)
    • multifocal and unisystemic Langerhans-cell histiocytosis (C96.5)
    ICD-10-CM Diagnosis Code D76

    Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • (Abt-) Letterer-Siwe disease (C96.0)
    • eosinophilic granuloma (C96.6)
    • Hand-Schüller-Christian disease (C96.5)
    • histiocytic medullary reticulosis (C96.9)
    • histiocytic sarcoma (C96.A)
    • histiocytosis X, multifocal (C96.5)
    • histiocytosis X, unifocal (C96.6)
    • Langerhans-cell histiocytosis, multifocal (C96.5)
    • Langerhans-cell histiocytosis NOS (C96.6)
    • Langerhans-cell histiocytosis, unifocal (C96.6)
    • leukemic reticuloendotheliosis (C91.4-)
    • lipomelanotic reticulosis (I89.8)
    • malignant histiocytosis (C96.A)
    • malignant reticulosis (C86.0)
    • nonlipid reticuloendotheliosis (C96.0)
    ICD-10-CM Diagnosis Code D76

    Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • (Abt-) Letterer-Siwe disease (C96.0)
    • eosinophilic granuloma (C96.6)
    • Hand-Schüller-Christian disease (C96.5)
    • histiocytic medullary reticulosis (C96.9)
    • histiocytic sarcoma (C96.A)
    • histiocytosis X, multifocal (C96.5)
    • histiocytosis X, unifocal (C96.6)
    • Langerhans-cell histiocytosis, multifocal (C96.5)
    • Langerhans-cell histiocytosis NOS (C96.6)
    • Langerhans-cell histiocytosis, unifocal (C96.6)
    • leukemic reticuloendotheliosis (C91.4-)
    • lipomelanotic reticulosis (I89.8)
    • malignant histiocytosis (C96.A)
    • malignant reticulosis (C86.0)
    • nonlipid reticuloendotheliosis (C96.0)

Diagnosis Index entries containing back-references to C96.0:
  • Histiocytosis D76.3
    ICD-10-CM Diagnosis Code D76.3

    Other histiocytosis syndromes

      2016 2017 2018 2019 Billable/Specific Code
    Applicable To
    • Reticulohistiocytoma (giant-cell)
    • Sinus histiocytosis with massive lymphadenopathy
    • Xanthogranuloma
    • Langerhans' cell NEC C96.6
      ICD-10-CM Diagnosis Code C96.6

      Unifocal Langerhans-cell histiocytosis

        2016 2017 2018 2019 Billable/Specific Code
      Applicable To
      • Eosinophilic granuloma
      • Histiocytosis X, unifocal
      • Histiocytosis X NOS
      • Langerhans-cell histiocytosis NOS
      Type 1 Excludes
      • multifocal and multisysemic (disseminated) Langerhans-cell histiocytosis (C96.0)
      • multifocal and unisystemic Langerhans-cell histiocytosis (C96.5)
    • X NEC C96.6
      ICD-10-CM Diagnosis Code C96.6

      Unifocal Langerhans-cell histiocytosis

        2016 2017 2018 2019 Billable/Specific Code
      Applicable To
      • Eosinophilic granuloma
      • Histiocytosis X, unifocal
      • Histiocytosis X NOS
      • Langerhans-cell histiocytosis NOS
      Type 1 Excludes
      • multifocal and multisysemic (disseminated) Langerhans-cell histiocytosis (C96.0)
      • multifocal and unisystemic Langerhans-cell histiocytosis (C96.5)
      • acute C96.0 (progressive)
      • multisystemic C96.0
    • acute differentiated progressive C96.0
  • Letterer-Siwe's disease C96.0
  • Reticuloendotheliosis
    • acute infantile C96.0
    • nonlipid C96.0
  • Reticulosis (skin)
    • acute of infancy C96.0

ICD-10-CM Codes Adjacent To C96.0
C95.02 …… in relapse
C95.1 Chronic leukemia of unspecified cell type
C95.10 …… not having achieved remission
C95.11 …… in remission
C95.12 …… in relapse
C95.9 Leukemia, unspecified
C95.90 …… not having achieved remission
C95.91 …… in remission
C95.92 …… in relapse
C96 Other and unspecified malignant neoplasms of lymphoid, hematopoietic and related tissue
C96.0 Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis
C96.2 Malignant mast cell neoplasm
C96.20 …… unspecified
C96.21 Aggressive systemic mastocytosis
C96.22 Mast cell sarcoma
C96.29 Other malignant mast cell neoplasm
C96.4 Sarcoma of dendritic cells (accessory cells)
C96.5 Multifocal and unisystemic Langerhans-cell histiocytosis
C96.6 Unifocal Langerhans-cell histiocytosis
C96.A Histiocytic sarcoma
C96.Z Other specified malignant neoplasms of lymphoid, hematopoietic and related tissue

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