2025 ICD-10-CM Diagnosis Code C96.0

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Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis

  • 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 2025 edition of ICD-10-CM C96.0 became effective on October 1, 2024.
  • 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

  • adult pulmonary Langerhans cell histiocytosis (J84.82)
  • multifocal and unisystemic Langerhans-cell histiocytosis (C96.5)
  • unifocal Langerhans-cell histiocytosis (C96.6)

The following code(s) above C96.0 contain annotation back-references

 that may be applicable to C96.0:

  • C00-D49  Neoplasms
  • C81-C96  Malignant neoplasms of lymphoid, hematopoietic and related tissue
  • C96  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 v42.0):

  • 011 Tracheostomy for face, mouth and neck diagnoses or laryngectomy with mcc
  • 012 Tracheostomy for face, mouth and neck diagnoses or laryngectomy with cc
  • 013 Tracheostomy for face, mouth and neck diagnoses or laryngectomy without cc/mcc
  • 826 Myeloproliferative disorders or poorly differentiated neoplasms with major o.r. Procedures with mcc
  • 827 Myeloproliferative disorders or poorly differentiated neoplasms with major o.r. Procedures with cc
  • 828 Myeloproliferative disorders or poorly differentiated neoplasms with major o.r. Procedures without cc/mcc
  • 829 Myeloproliferative disorders or poorly differentiated neoplasms with other procedures with cc/mcc
  • 830 Myeloproliferative disorders or poorly differentiated neoplasms with other procedures 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-CMCode 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
  • 2021 (effective 10/1/2020): No change
  • 2022 (effective 10/1/2021): No change
  • 2023 (effective 10/1/2022): No change
  • 2024 (effective 10/1/2023): No change
  • 2025 (effective 10/1/2024): No change

Code annotations containing back-references to C96.0:

Diagnosis Index entries containing back-references to C96.0:

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