Table 2

Secondary cases of Evans syndrome, n = 34

Secondary casesn
Autoimmune diseases
    Systemic lupus erythematosus7
    “Incomplete” lupus3
    Primary antiphospholipid syndrome2
    Sjögren syndrome2
    Common variable immunodeficiency4
    IgA deficiency2
    B-cell non-Hodgkin malignant lymphoma2
    Chronic lymphocytic leukemia3
    T-cell non-Hodgkin lymphoma1
    Chronic myelomonocytic leukemia1
    Unclassified lymphoproliferative disorder*3
    Hepatitis C1
    Congenital asplenia1
    Idiopathic CD4 lymphocytopenia1
  • MGUS indicates monoclonal gammopathy of unknown significance.

  • * Lymphadenopathy, splenomegaly + polyclonal hypergammaglobulinemia (n = 3) with an excess of large granular lymphocytes in 1 case. No features suggesting an ALPS (no excess of CD3+ C α/β+ CD4/CD8 T cells in peripheral blood, no Fas, Fas-L, or caspase 8 genes mutations) has been found.