2025 ICD-10-CM code C96.6
(Active) Effective Date: N/A Neoplasms - Unifocal Langerhans-cell histiocytosis 2 Feed
Unifocal Langerhans-cell histiocytosis (LCH) is a rare disorder characterized by the uncontrolled proliferation of Langerhans cells, forming tumors (granulomas) at a single site.
Medical necessity for treatment is established by confirming the diagnosis through biopsy and demonstrating the impact on the patient's health. This could include pain, functional impairment, or risk of complications like pathological fractures or respiratory compromise.
Physicians diagnosing and managing LCH might include specialists like hematologists, oncologists, dermatologists, or orthopedists, depending on the site involved. They are responsible for ordering appropriate investigations like blood tests (CBC, ESR, liver and kidney function tests), imaging studies (X-rays, CT, MRI, PET, ultrasound), and biopsies to confirm the diagnosis and assess the extent of the disease. Treatment planning, which could involve surgical removal, topical steroids, or other systemic therapies, also falls under their purview, along with monitoring the patient's response and managing potential complications.
In simple words: Unifocal LCH is a rare condition where too many Langerhans cells (a type of immune cell) grow in one spot in the body, forming a small tumor.This usually happens in a bone, lung, or skin area.While it can affect both children and adults, older children and adults are more likely to develop it.It’s important to differentiate this from other forms of LCH that affect multiple areas of the body.
Unifocal Langerhans-cell histiocytosis (LCH), also known as eosinophilic granuloma or unifocal Histiocytosis X, involves the abnormal growth of Langerhans cells, immune system cells, leading to the formation of granulomas localized to a single site.It primarily affects a single bone, lung, or skin area and is more often seen in adults and older children. This condition is distinguished from multifocal and multisystemic LCH (Letterer-Siwe disease) and multifocal unisystemic LCH.
Example 1: A 10-year-old child presents with persistent pain and swelling in the right femur. Imaging reveals a lytic lesion, and biopsy confirms the diagnosis of unifocal LCH affecting the bone., A 30-year-old adult experiences localized skin lesions resembling a rash. Biopsy of the affected area reveals the characteristic Langerhans cell proliferation consistent with unifocal LCH limited to the skin., A 60-year-old adult presents with a persistent cough and shortness of breath.A chest X-ray shows a solitary nodule in the lung.Further investigation with biopsy confirms a diagnosis of unifocal LCH isolated to the lung.
Documentation should clearly specify the single site of involvement (bone, skin, lung, etc.), the diagnostic methods used (physical exam, imaging, biopsy results), and the treatment plan. Detailed clinical findings, such as pain, swelling, or respiratory symptoms, should also be recorded.
** It's important to note that as of today, December 2nd, 2024, this information is current. However, medical codes and guidelines are subject to change, so it is crucial to consult the latest official resources for the most up-to-date information.
- Payment Status: Active
- Specialties:Hematology, Oncology, Dermatology, Orthopedic Surgery, Pulmonology
- Place of Service:Inpatient Hospital, Outpatient Hospital, Physician's Office, Independent Clinic