C96.0

Applicable To

• Histiocytosis X, multisystemic

• Letterer-Siwe disease

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.

Details

• 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 2023 edition of ICD-10-CM C96.0 became effective on October 1, 2022.

• This is the American ICD-10-CM version of C96.0 – other international versions of ICD-10 C96.0 may differ.