Secondary cases of Evans syndrome, n = 34
| Secondary cases . | n . |
|---|---|
| Autoimmune diseases | |
| Systemic lupus erythematosus | 7 |
| “Incomplete” lupus | 3 |
| Primary antiphospholipid syndrome | 2 |
| Sjögren syndrome | 2 |
| Immunodeficiencies | |
| Common variable immunodeficiency | 4 |
| IgA deficiency | 2 |
| Lymphomas | |
| B-cell non-Hodgkin malignant lymphoma | 2 |
| Chronic lymphocytic leukemia | 3 |
| T-cell non-Hodgkin lymphoma | 1 |
| Miscellaneous | |
| Chronic myelomonocytic leukemia | 1 |
| Unclassified lymphoproliferative disorder* | 3 |
| MGUS | 1 |
| Hepatitis C | 1 |
| Congenital asplenia | 1 |
| Idiopathic CD4 lymphocytopenia | 1 |
| Secondary cases . | n . |
|---|---|
| Autoimmune diseases | |
| Systemic lupus erythematosus | 7 |
| “Incomplete” lupus | 3 |
| Primary antiphospholipid syndrome | 2 |
| Sjögren syndrome | 2 |
| Immunodeficiencies | |
| Common variable immunodeficiency | 4 |
| IgA deficiency | 2 |
| Lymphomas | |
| B-cell non-Hodgkin malignant lymphoma | 2 |
| Chronic lymphocytic leukemia | 3 |
| T-cell non-Hodgkin lymphoma | 1 |
| Miscellaneous | |
| Chronic myelomonocytic leukemia | 1 |
| Unclassified lymphoproliferative disorder* | 3 |
| MGUS | 1 |
| Hepatitis C | 1 |
| Congenital asplenia | 1 |
| Idiopathic CD4 lymphocytopenia | 1 |
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.