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We report a 17\u2010year\u2010old male with a heterozygous\n                    <jats:italic>PIK3CD<\/jats:italic>\n                    c.3061G\u2009&gt;\u2009A (p.E1021K) variant who presented with progressive edema, extensive hypermetabolic lymphadenopathy, splenomegaly, and Epstein\u2013Barr virus (EBV) DNAemia. A core needle biopsy of the right inguinal lymph node demonstrated an immunodeficiency\u2010associated EBV\u2010positive B\u2010cell lymphoproliferative disorder with extensive monotypic plasmacytoid differentiation. Because the biopsy contained limited mature B\u2010cell tissue, the pathological findings favored a polymorphic B\u2010LPD although EBV\u2010positive diffuse large B\u2010cell lymphoma with plasmacytic differentiation could not be excluded. The patient received anti\u2010B\u2010cell\u2010directed therapy and supportive treatment. He was subsequently readmitted with septic shock and acute respiratory distress syndrome. Blood metagenomic next\u2010generation sequencing detected\n                    <jats:italic>Escherichia coli<\/jats:italic>\n                    and\n                    <jats:italic>Klebsiella pneumoniae<\/jats:italic>\n                    , together with antimicrobial\u2010resistance genes including\n                    <jats:italic>blaNDM<\/jats:italic>\n                    . Despite intensive antimicrobial and organ\u2010supportive treatment, the patient remained critically ill and was discharged at his family\u2019s request for transfer to a local hospital; his subsequent outcome was unavailable. This case highlights the diagnostic difficulty of classifying EBV\u2010positive B\u2010cell proliferations using limited biopsy tissue in APDS and the competing risks of lymphoproliferative disease and severe infection. Adequate tissue sampling and pathological characterization, close microbiological surveillance, and individualized multidisciplinary management are essential in this setting.\n                  <\/jats:p>","DOI":"10.1155\/crh\/3644513","type":"journal-article","created":{"date-parts":[[2026,8,12]],"date-time":"2026-08-12T06:46:13Z","timestamp":1786517173000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Epstein\u2013Barr Virus\u2010Positive B\u2010Cell Lymphoproliferative Disorder Complicated by Septic Shock in Activated PI3K\u03b4 Syndrome: A Pediatric Case Report and Literature 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