Áutoimmune Lymphoproliferative Syndrome (ALPS)
Stefanaki K, Van-Vliet-Constadinidou C, Kolibiris Ch, Rondogianni
D, Theodoridou M, Kanariou M, Karentzou O
Dept of Pathology, Dept of Immunology and Pediatric Clinics
of Athens Medical School, Aghia Sophia Children's Hospital Dept
of Pathology, "Evangelismos" General Hospital, Athens
The Autoimmune Lymphoproliferative Syndrome (ALPS) is generally
caused by defective Fas Imediated apoptosis and is characterized
by lymphoproliferations and autoimmune features. Although pronounced
lymphadenopathy is one of the cardinal symptoms, only a few descriptions
of lymph node histopathology in patients with ALPS have been reported.
Moreover, the differential diagnosis between ALPS lymphadenopathy
and other autoimmune lymphadenopathies or malignant lymphomas
may be difficult. In the present study, the histological and immunophenotypic
features of the lymph node of a 5yr old boy with ALPS Ia are reported.
Key words: Autoimmune Lymphoproliferative Syndrome, ALPS
Ia, Lymph node, Histology, Fas mutation.
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