ALPS type III

Patient had lifelong lymphadenopathy and splenomegaly, marked leukocytosis, large non-Hodgkin's B cell lymphoma, positive autoantibodies: direct antiglobulin test, ANA, ACA IgG and IgM, rheumatoid factor.

Therapeutic options

  • Immune suppression. In patients with massive lymphoproliferation, chemotherapy with prednisone, cyclophosphamide and vincristine has been unsuccessful. In case of hypersplenism, splenectomy is often performed. Severe autoimmune manifestation can be treated with steroids and cyclophosphamide.
  • Lymphoproliferative disorders, eMedicine