What is the prognosis of anaplastic large cell lymphoma (ALCL)?

Updated: May 08, 2020
  • Author: Delong Liu, MD, PhD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Answer

Generally, cutaneous CD30+ anaplastic large-cell lymphoma (cutaneous CD30+ ALCL) has a favorable clinical course (5-y survival rate of 90%), with occasional spontaneous regression (up to 25% of cases) of the skin lesions. The systemic form has a worse prognosis compared with the primary cutaneous form. However, primary cutaneous CD30- large-cell lymphoma also has worse prognosis (5-y survival rate of 15%).

Primary cutaneous disease, spontaneous regression, absence of extracutaneous involvement, and younger age at onset (< 60 y) have been suggested to be associated with better prognosis.

The expression of either NPM-ALK transcripts or ALK protein is not correlated with prognosis or age in the primary cutaneous form of CD30+ cutaneous lymphoproliferations. However, expression of either NPM-ALK transcripts or ALK protein indicates a better prognosis in the systemic form of ALCL.

Prior studies have suggested that the stage of disease may be more important than the cytologic subtype.

CD30 ligand expression is detected in regressing lesions only and indicates a better prognosis. Cutaneous CD30+ ALCLs developing from preexistent Mycosis Fungoides are often associated with a poor prognosis (5-y survival rate of 10-30%). Regional lymph node involvement is not necessarily associated with unfavorable prognosis.

P53 expression by immunohistochemistry is not associated with spontaneous regression, extracutaneous spreading, or survival.


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