Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia and Newly Diagnosed Lymphoma
نویسندگان
چکیده
منابع مشابه
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.
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Hodgkin lymphoma (HL) accounts for about 10% of all childhood cancers. Five-year survival rates with modern therapies are now approaching >90-95% as a consequence of its significant sensitivity to both chemotherapy and radiation. The current challenge is to determine how much therapy is needed to improve survival and how to adapt treatment to the patient to prevent these long term toxicities. T...
متن کاملtreatment of newly diagnosed and relapsed hodgkin lymphoma in children
hodgkin lymphoma (hl) accounts for about 10% of all childhood cancers. five-year survival rates with modern therapies are now approaching >90-95% as a consequence of its significant sensitivity to both chemotherapy and radiation. the current challenge is to determine how much therapy is needed to improve survival and how to adapt treatment to the patient to prevent these long term toxicities. t...
متن کاملAn autoimmune lymphoproliferative syndrome initially diagnosed as Evans syndrome.
Autoimmune lymphoproliferative syndrome (ALPS) is a rare childhood disorder characterized by chronic non-malignant lymphoproliferation and autoimmunity. Patients with ALPS frequently exhibit episodic and intermittent, severe autoimmune- induced hemolytic anemia, thrombocytopenia or combined cytopenias. The co-occurrence of immune-mediated cytopenias, autoimmune thrombocytopenia and autoimmune h...
متن کاملImmune function in newly diagnosed children with malignancy.
BACKGROUND Tumor genesis of many pediatric malignancies remains unclear. Data of immune function are lacking at diagnosis. We prospectively analyzed 109 pediatric patients with malignancy at diagnosis. METHODS Lymphocyte subpopulations were characterized by FACS, TREC-assay, and Immunoscope, cytokines by FACS and ELISA. OBSERVATIONS We detected higher values of CD4(+) T cells and consecutiv...
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ژورنال
عنوان ژورنال: Turkish Journal of Hematology
سال: 2020
ISSN: 1300-7777
DOI: 10.4274/tjh.galenos.2020.2020.0618