[New concept for ALK negative anaplastic large cell lymphoma]

Area of Science:

• Hematology
• Oncology
• Pathology

Context:

• Anaplastic large cell lymphoma (ALCL) is categorized into ALK-positive (ALK(+) ALCL) and ALK-negative (ALK(-) ALCL) subtypes.
• While ALK(-) ALCL shares CD30 expression and morphology with ALK(+) ALCL, it presents unique molecular and genetic characteristics.
• ALK(-) ALCL is associated with poorer treatment response, advanced stage, and inferior survival rates compared to ALK(+) ALCL.

Purpose:

• To review recent advances in the understanding of ALK-negative anaplastic large cell lymphoma.
• To consolidate information on the basis, diagnosis, and treatment of ALK(-) ALCL.
• To highlight the key differences and challenges associated with ALK(-) ALCL.

Summary:

• ALK(-) ALCL lacks ALK protein expression but shares CD30 positivity and morphology with ALK(+) ALCL.
• Molecular and genetic analyses reveal significant differences between ALK(-) and ALK(+) ALCL subtypes.
• This subtype is linked to older patients, B symptoms, advanced stage, high IPI scores, and a dismal prognosis with less than 49% 5-year survival.

Impact:

• Provides a comprehensive overview of ALK(-) ALCL for researchers and clinicians.
• Facilitates a better understanding of the underlying biology of ALK(-) ALCL.
• Aims to guide improved diagnostic and therapeutic strategies for patients with ALK(-) ALCL, potentially improving outcomes.