autoimmune lymphoproliferative syndrome misdiagnosed as hemophagocytic lymphohistiocytosis a case report
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abstract
autoimmune lymphoproliferative syndrome (alps) is a rare inherited disorder of apoptosis. it usually presents with chronic lymphadenopathy, splenomegaly, and symptomatic cytopenia in a child. herein, we report a 14-year-old boy with symptoms misdiagnosed as hemophagocytic lymphohistiocytosis who was treated before alps was diagnosed for the patient. this case should alert pediatricians to consider alps in differential diagnosis of a child with lymphadenopathy, splenomegaly, and cytopenia.
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Autoimmune Lymphoproliferative Syndrome Misdiagnosed as Hemophagocytic Lymphohistiocytosis A Case Report
Autoimmune lymphoproliferative Syndrome (ALPS) is a rare inherited disorder of apoptosis. It usually presents with chronic lymphadenopathy, splenomegaly, and symptomatic cytopenia in a child. Herein, we report a 14-year-old boy with symptoms misdiagnosed as hemophagocytic lymphohistiocytosis who was treated before ALPS was diagnosed for the patient. This case should alert pediatricians to consi...
full textAutoimmune lymphoproliferative syndrome misdiagnosed as hemophagocytic lymphohistiocytosis.
Autoimmune lymphoproliferative syndrome (ALPS) is a rare inherited disorder of apoptosis, most commonly due to mutations in the FAS (TNFRSF6) gene. It presents with chronic lymphadenopathy, splenomegaly, and symptomatic multilineage cytopenias in an otherwise healthy child. Unfortunately, these clinical findings are also noted in other childhood lymphoproliferative conditions, such as leukemia,...
full textautoimmune lymphoproliferative syndrome; a case report
autoimmune lymphoproliferative syndrome is a disorder of lymphoid system regulation characterized by chronic splenomegaly, lymphadenopathy and autoimmune phenomena especially immune-mediated cytopenias. the hallmark of the disease is the presence in peripheral blood and lymphoid tissue of increased numbers of a normally rare t lymphocyte subset, usually referred to as “double-negative” t cells....
full text[Autoimmune lymphoproliferative syndrome: a case report].
Autoimmune lymphoproliferative syndrome is a disorder of lymphoid system regulation characterized by chronic splenomegaly, lymphadenopathy and autoimmune phenomena especially immune-mediated cytopenias. The hallmark of the disease is the presence in peripheral blood and lymphoid tissue of increased numbers of a normally rare T lymphocyte subset, usually referred to as "double-negative" T cells....
full textAutoimmune Lymphoproliferative Syndrome; A Case Report
Autoimmune lymphoproliferative syndrome is a disorder of lymphoid system regulation characterized by chronic splenomegaly, lymphadenopathy and autoimmune phenomena especially immune-mediated cytopenias. The hallmark of the disease is the presence in peripheral blood and lymphoid tissue of increased numbers of a normally rare T lymphocyte subset, usually referred to as “double-negative” T cells....
full textNeonatal hemophagocytic lymphohistiocytosis--case report.
Hemophagocytic lymphohystiocytosis (HLH) represents a severe hyperinflammatory condition with the cardinal symptoms prolonged fever, hepatosplenomegaly, and cytopenias. The most prominent histopathological feature of HLH is an accumulation of activated T lymphocytes and macrophages predominantly in lymphoid tissues. Although it can occur in all age groups, neonatal-onset HLH is very rare. We re...
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Journal title:
iranian journal of blood and cancerجلد ۷، شماره ۴، صفحات ۱۹۸-۲۰۰
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