autoimmune lymphoproliferative syndrome, type 2

autoimmune lymphoproliferative syndrome, type 2

A rare autoimmune disorder characterised by a loss of lymphocyte homeostasis; B-cell lymphocytosis; expansion of an unusual population of CD4/CD8-deficient T cells, which express the alpha/beta T-cell receptor; haemolytic anaemia; and variable ITP.

Clinical findings
Prominent lymphadenopathy, hepatosplenomegaly and Coombs-positive haemolytic anaemia; loss of immunologic self-tolerance resulting in hypergammaglobulinaemia with multiple autoantibodies, such as anti-erythrocyte, anti-RNP, anti-SM, anti-SSB, rheumatoid factor and anti-factor-VIII antibodies, causing severe coagulopathy.

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