What is the prognosis of autoimmune lymphoproliferative syndrome (ALPS)?

Updated: Jul 29, 2019
  • Author: Akaluck Thatayatikom, MD, RhMSUS; Chief Editor: Harumi Jyonouchi, MD  more...
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Answer

The mortality and morbidity of ALPS vary widely. The major determinants of prognosis in patients diagnosed with ALPS include the following:

  • The severity of autoimmune disease (particularly autoimmune cytopenias)

  • Hypersplenism

  • Asplenia-related sepsis

  • Development of lymphoma

Addressing these serious conditions with proper surveillance and education is vital for an optimal prognosis. Patients with mutations of the intracellular region of the Fas protein have a significantly increased risk of developing lymphoma and warrant the most diligent long-term surveillance. Despite the numerous potentially serious complications, the overall prognosis for patients with ALPS is good.

Many patients are expected to live a normal lifespan, with few clinical complications. [6]  However, a significant number of patients develop childhood-onset life-threatening cytopenias, which necessitate interventions such as hospitalization, immunosuppressive therapy, blood transfusion, antibiotic therapy, or splenectomy. These cytopenias are often chronic and refractory.

As pediatric ALPS patients develop into adolescents and young adults, the degree of adenopathy (particularly visible adenopathy) tends to decrease. The natural course of the adenopathy should be discussed with the patients and their families, particularly during adolescence, when visible adenopathy can be particularly distressing.


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