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Generalized Lymphadenopathy with Cytopenias

      Autoimmune lymphoproliferative syndrome (ALPS) is a rare disorder characterized by dysregulation of lymphocyte homeostasis. The true incidence and prevalence of ALPS remain unclear because most patients are misdiagnosed or underdiagnosed.
      • Shah S
      • Wu E
      • Rao VK
      • Tarrant TK.
      Autoimmune lymphoproliferative syndrome: an update and review of the literature.
      Early recognition of ALPS is critical because effective treatment can significantly reduce the morbidity and mortality in most patients.
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      References

        • Shah S
        • Wu E
        • Rao VK
        • Tarrant TK.
        Autoimmune lymphoproliferative syndrome: an update and review of the literature.
        Curr Allergy Asthma Rep. 2014; 14: 462https://doi.org/10.1007/s11882-014-0462-4
        • Oliveira JB
        • Bleesing JJ
        • Dianzani U
        • et al.
        Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome (ALPS): report from the 2009 NIH International Workshop.
        Blood. 2010; 116: e35-e40https://doi.org/10.1182/blood-2010-04-280347
        • Bajwa R
        • Savelli S
        • Gross T.
        Pentostatin for treatment of refractory autoimmune lymphoproliferative syndrome.
        Pediatr Blood Cancer. 2011; 57: 336-337https://doi.org/10.1002/pbc.23144
        • Teachey DT
        • Greiner R
        • Seif A
        • et al.
        Treatment with sirolimus results in complete responses in patients with autoimmune lymphoproliferative syndrome.
        Br J Haematol. 2009; 145: 101-106https://doi.org/10.1111/j.1365-2141.2009.07595.x
        • Rao VK
        • Oliveira JB.
        How I treat autoimmune lymphoproliferative syndrome.
        Blood. 2011; 118: 5741-5751https://doi.org/10.1182/blood-2011-07-325217