Autoimmune lymphoproliferative syndrome (ALPS): a rare cause of immune cytopenia.
Autoimmune Lymphoproliferative syndrome (ALPS) is an inherited disorder manifesting with autoimmune cytopenia, lymphadenopathy and splenomegaly. The differential diagnosis includes infections, autoimmune disorders or malignancies. The disease is characterized by accumulation of double negative (CD3+ CD4- CD8-) T cells (DNT) in the peripheral blood. We describe a case and review the literature.
John
M Joseph
MJ
Department of Hematology, Christian Medical College, Vellore, India.
Rajasekhar
Reena
R
Mathews
Vikram
V
D001323:Autoantibodies / Q000276:immunology*; D017252:CD3 Complex / Q000276:immunology*; D015704:CD4 Antigens / Q000276:immunology*; D016827:CD8 Antigens / Q000276:immunology*; D002648:Child; D015897:Comorbidity; D003937:Diagnosis, Differential; D006801:Humans; D008206:Lymphatic Diseases / Q000150:complications / Q000276:immunology*; D008231:Lymphopenia / Q000150:complications / Q000276:immunology*; D008297:Male; D013163:Splenomegaly / Q000150:complications / Q000276:immunology*; D013577:Syndrome
D001323:Autoantibodies; D017252:CD3 Complex; D015704:CD4 Antigens; D016827:CD8 Antigens