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Year : 2014  |  Volume : 15  |  Issue : 3  |  Page : 144-146

Autoimmune lymphoproliferative syndrome presenting as urticaria

Department of Paediatrics, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India

Correspondence Address:
Parveen Bhardwaj
Department of Pediatrics, Indira Gandhi Medical College, Shimla, Himachal Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2319-7250.143677

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Autoimmune lymphoproliferative syndrome (ALPS) is an inherited disorder characterized by chronic splenomegaly, lymphadenopaphy hypergammaglobulinemia and autoimmune phenomena especially immune-mediated cytopenias. The disease is characterized by presence of "double-negative (CD3 + CD4 CD8 )" T-cells in the peripheral blood and lymphoid tissue. Rarely it can also present as urticaria. We report a child of ALPS who presented to us with fever, generalized lymphadenopathy, chronic urticaria and hepatosplenomegaly.

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