الفهرس 
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Abstract
Autoimmune lymphoproliferative syndrome (ALPS) is a rare genetic disorder occurring especially in early childhood occurring as a syndrome which characterized by Production of large number of lymphocyte ,Chronic non-malignant lymphadenopathy and/or splenomegaly and Increased number of double negative T cells. ALPS is one of the first human disease whose etiology has been attributed to a primary defect in apoptosis (programmed cell death). Presentation of ALPS patients is generalized chronic lymphadenopathy more than 6 months, splenomegaly and autoimmune cytopenia. Autoimmunity is the second most common clinical manifestation of ALPS that affect over 70% of patients, It may affect erythrocytes causing (autoimmune haemolytic anemia), Platelets causing (autoimmune thrombocytopenia), or neutrophils causing (neutropenia) or a combination of all of these. Patients with autoimmune lymphoproliferative syndrome are more susceptible to lymphoma and many cancers such as leukemia, liver, thyroid glands, breast, lung and colon. The study was conducted on 36 patients (25 males and 11 females) to perform a complete blood count (CBC) to detect autoimmune cytopenia and to determine the percentage of double-negative T cells, which is one of the main criteria required to diagnose ALPS. The study revealed that about (55%) of cases had anemia, (33%) of cases had neutropenia, and one case had thrombocytopenia with association of TCR αβ CD3, double-negative T cells (15.2%) in suspected cases.which should be more than (2.5%). This study summarized that that autoimmune lymphoproliferative syndrome is a complex disease that now includes patients who may not have been diagnosed before. The prevalence of the disease is still unknown and early recognition and diagnosis of ALPS is essential for monitoring and treatment of patients. The study recommends that considering double negative T cell percentage >11.7as a cut off level for further investigations or suspecting ALPS patient in addition to clinical and other lab criteria as an alarming point for further investigations and Further studies should be carried out for early recognition and diagnosis of ALPS to avoid misdiagnosis and to provide optimum care.