Clinical Course And Prognosis

Transformation indicates an unfavorable prognosis in most patients with median survival from transformation of 2-27 months versus 53% for patients with nontrans-formed MF (2-4). Median time from diagnosis of MF to transformation was 6.5 years in one study (6). In this same study, mean survival from transformation to death was 22 months. CD30 expression has no prognostic impact in transformed cases, whereas age (at least 60 years) and extracutaneous spread are associated with a poor prognosis with a 5-year actuarial survival of 8% versus 32% in transformed cases (6). Most patients experience disease progression with fatal outcome (8).

Remarkably, tumors can undergo spontaneous regression as it is seen in 25% of CD30+ ALCL, but systemic spread with involvement of all visceral organs can subsequently follow and result in a fatal outcome.

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