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2025 ICD-10-CM code C84.6

Anaplastic large cell lymphoma, ALK-positive.

Use additional codes to document the specific site(s) of involvement.Chapter 4 codes may be used to identify any associated functional activity.

Medical necessity for treatment is determined by the confirmed diagnosis of ALK-positive ALCL, the stage of the disease, and the patient's overall health.Treatment is medically necessary to eradicate the malignant cells and prevent disease progression.

Diagnosis involves physical examination, imaging studies (CT, MRI, PET), and biopsy with histopathological and immunohistochemical analysis. Treatment includes chemotherapy regimens like CHOP, and potentially radiation therapy and stem cell transplantation for refractory or relapsed cases. ALK inhibitor drugs may be used. Regular follow-up is crucial for ongoing monitoring and management.

In simple words: ALK-positive anaplastic large cell lymphoma (ALCL) is a rare, fast-growing cancer of the immune system. It affects a type of white blood cell called T-cells. These abnormal T-cells carry a specific protein called ALK on their surface.It can form tumors in lymph nodes and other organs throughout the body. While aggressive, it often responds well to treatment, especially in children and young adults.

Anaplastic Large Cell Lymphoma (ALCL) is a rare type of Non-Hodgkin Lymphoma (NHL) characterized by the presence of CD30-positive large pleomorphic lymphoid cells.This code (C84.6) specifies ALCL that is positive for anaplastic lymphoma kinase (ALK) protein expression, a marker often associated with a more favorable prognosis.ALCL is a systemic lymphoma that can form tumors throughout the body involving the lymph nodes, spleen, lung, liver, other organs, blood, and bone marrow. ALK-positive ALCL is typically more common in children and young adults.

Example 1: A 15-year-old presents with painless swollen lymph nodes in the neck and armpits, fatigue, and intermittent fever. Biopsy and immunohistochemistry confirm ALK-positive ALCL., A 5-year-old child experiences persistent cough and shortness of breath.Imaging reveals a mediastinal mass. Biopsy confirms ALK-positive ALCL with lung involvement., A young adult develops a solitary skin lesion that does not heal. Biopsy shows ALK-positive ALCL with primary cutaneous involvement, requiring localized treatment such as radiation therapy or surgery.

Diagnosis requires histopathological examination of biopsied tissue demonstrating CD30 expression and positive ALK staining through immunohistochemistry. Imaging reports, blood tests (CBC, LDH, liver and kidney function tests), and bone marrow biopsy results may be necessary for staging and assessing disease extent.

** Distinguishing between ALK-positive and ALK-negative ALCL is essential for prognosis and treatment planning. While both subtypes are aggressive, ALK-positive ALCL typically has a better response to standard chemotherapy.

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