Autoimmune lymphoproliferative syndrome
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Autoimmune lymphoproliferative syndrome (ALPS) is a rare lymphoproliferative condition.
Clinical presentation
It presents with chronic lymphadenopathy, splenomegaly, and symptomatic multilineage cytopenias in an otherwise healthy child.
Pathology
It represents a failure of apoptotic mechanisms to maintain lymphocyte homeostasis and is a rare inherited disorder most commonly occurring due to mutations in the FAS (TNFRSF6) gene. Other mutations of the genes such as Fas-ligand (FASLG), Caspase 10 (CASP10) and Caspase 8 (CASP8), NRAS and KRAS have also been observed in small numbers of patients and approximately 20-30% of patients may have an unidentified defect 6.
Complications
Although initial lymphadenopathy is non-malignant, patients have an increased risk of developing:
-<p><strong>Autoimmune lymphoproliferative syndrome (ALPS)</strong> is a rare <a href="/articles/lymphoproliferative-condition">lymphoproliferative condition</a>.</p><h4>Clinical presentation</h4><p>It presents with chronic lymphadenopathy, <a href="/articles/splenomegaly">splenomegaly</a>, and symptomatic multilineage cytopenias in an otherwise healthy child.</p><h4>Pathology</h4><p>It represents a failure of apoptotic mechanisms to maintain lymphocyte homeostasis and is a rare inherited disorder most commonly occurring due to mutations in the <em>FAS</em> (<em>TNFRSF6</em>) gene. Other mutations of the genes such as Fas-ligand (FASLG), Caspase 10 (CASP10) and Caspase 8 (CASP8), NRAS and KRAS have also been observed in small numbers of patients and approximately 20-30% of patients may have an unidentified defect<sup> 6</sup>.</p><h5>Complications</h5><p>Although initial lymphadenopathy is non-malignant, patients have an increased risk of developing</p><ul>- +<p><strong>Autoimmune lymphoproliferative syndrome (ALPS)</strong> is a rare <a href="/articles/lymphoproliferative-condition">lymphoproliferative condition</a>.</p><h4>Clinical presentation</h4><p>It presents with chronic lymphadenopathy, <a href="/articles/splenomegaly">splenomegaly</a>, and symptomatic multilineage cytopenias in an otherwise healthy child.</p><h4>Pathology</h4><p>It represents a failure of apoptotic mechanisms to maintain lymphocyte homeostasis and is a rare inherited disorder most commonly occurring due to mutations in the <em>FAS</em> (<em>TNFRSF6</em>) gene. Other mutations of the genes such as Fas-ligand (FASLG), Caspase 10 (CASP10) and Caspase 8 (CASP8), NRAS and KRAS have also been observed in small numbers of patients and approximately 20-30% of patients may have an unidentified defect<sup> 6</sup>.</p><h5>Complications</h5><p>Although initial lymphadenopathy is non-malignant, patients have an increased risk of developing:</p><ul>
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