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.

单词	autoimmune lymphoproliferative syndrome, type 2
释义	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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