Prognosis

Patient registry data collected between 2000 and 2011 in Sweden reported 5-year survival of around 61% in all patients with AA.[9]

Prognosis for those who do not have severe AA is excellent, with survival >90%.[38] Some spontaneously remit; others may live many years without progression of disease.

The prognosis of patients with severe or very severe AA has markedly improved over the last 30 years. The 5-year survival with immunosuppressive therapy, using horse anti-thymocyte globulin (ATG) plus cyclosporine, is around 75% to 80%, but lower with rabbit ATG.[9][53]However, survival rate may be age-dependent. In one study, younger patients (ages 19 to 39 years) treated with immunosuppressive therapy had a significantly higher 5-year survival than older patients (ages ≥60 years) treated with immunosuppressive therapy (90% vs. 52%, respectively).[9]

With stem cell transplant (SCT), young patients with a matched related donor have a probability of long-term survival >80%; for older patients the prognosis is not quite as good. Outcomes after matched unrelated donor SCT have improved significantly, such that overall survival is similar to matched related donor SCT when using ATG-based conditioning regimens, although with a higher risk of graft versus host disease (GVHD).[73] In contrast, the use of alemtuzumab-based conditioning is associated with a lower risk of GVHD in the setting of unrelated donor SCT.[74]

The impact of best supportive care is extremely important for patients in all stages of the disease. Developments in supportive care have led to a reduction in infection-related deaths and thereby improved the survival of refractory patients.[75]

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