All people with<unk>  have signs of lymphoproliferation, which makes it the most common clinical manifestation of the disease. The increased proliferation of lymphoid cells can cause the size of lymphoid organs such as the lymph nodes and spleen to increase (lymphadenopathy and splenomegaly, present in respectively over 90% and over 80% of patients). The liver is enlarged (hepatomegaly) in 30 - 40% of patients.<unk>  disease is the second most common clinical manifestation and one that most often requires treatment. The most common<unk>  presentations include<unk>  cytopenias, which can be mild to very severe and intermittent or chronic. These include<unk>  hemolytic anemia,<unk>  neutropenia, and<unk>  thrombocytopenia. Other<unk>  manifestations can be similar to systemic lupus erythematosus (least common, affecting <5% of patients). Manifestations within the nervous system can include<unk>  cerebellar ataxia, Guillain–Barré syndrome, and transverse myelitis. Manifestations in the gastrointestinal system can include atrophic gastritis, and<unk>  hepatitis, esophagitis, colitis, and pancreatitis. Other manifestations can affect the skin (hives), lungs (bronchiolitis obliterans), or kidneys<unk>  glomerulonephritis and nephrotic syndrome). Another sign are cancers such as Hodgkin and non-Hodgkin lymphomas, which appear to be increased, possibly due to Epstein–Barr virus-encoded RNA-positivity. Some carcinomas may occur. Unaffected family members with genetic mutations are also at an increased risk of developing cancer.
Autoimmune lymphoproliferative syndrome
Diffuse infiltrative lymphocytosis syndrome
Thymoma with immunodeficiency
Bare lymphocyte syndrome
X-linked lymphoproliferative disease
Thymoma-associated multiorgan autoimmunity
Adult-onset immunodeficiency syndrome
Epstein–Barr virus–associated lymphoproliferative diseases