Article
Histologic features of sinus histiocytosis with massive lymphadenopathy in patients with autoimmune lymphoproliferative syndrome.
The American journal of surgical pathology - 1 Jul 2005
Maric Irina, Pittaluga Stefania, Dale Janet K, Niemela Julie E, Delsol Georges, Diment Judith, Rosai Juan, Raffeld Mark, Puck Jennifer M, Straus Stephen E, Jaffe Elaine S
Abstract excerpt
Autoimmune lymphoproliferative syndrome (ALPS) is an inherited disorder associated with defects in Fas-mediated apoptosis, characterized most often by childhood onset of lymphadenopathy, splenomegaly, hypergammaglobulinemia, and autoimmune phenomena. Children with sinus histiocytosis with massive lymphadenopathy (SHML) have a somewhat similar clinical phenotype in which prominent adenopathy also is associated...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.
