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Anemia and Neutropenia

  • Kristin ShimanoEmail author
Chapter

Abstract

Clinical features of autoimmune lymphoproliferative syndrome (ALPS) include cytopenias, chronic lymphadenopathy and hepatosplenomegaly

Elevated double negative T cells are the characteristic laboratory abnormality in ALPS

Autosomal dominant mutations in FAS, leading to defective apoptosis of lymphocytes, are causal in the majority of cases of ALPS

Sirolimus is an effective treatment for the lymphadenopathy and cytopenia in many patients with ALPS

Patients with ALPS are at increased risk for development of lymphoma and should be monitored for this complication

Keywords

Autoimmune lymphoproliferative syndrome FAS Double negative T cells 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Hematology/Oncology and Bone Marrow Transplant, Department of PediatricsUCSF Benioff Children’s HospitalSan FranciscoUSA

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