{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T06:05:10Z","timestamp":1771221910583,"version":"3.50.1"},"reference-count":22,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T00:00:00Z","timestamp":1771200000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Acute erythroid leukemia (AEL) complicated by hemophagocytic lymphohistiocytosis (HLH) is an exceedingly rare hematologic malignancy. Its diagnosis relies on a comprehensive assessment that includes bone marrow cytomorphology, immunophenotyping, cytogenetics, molecular profiling, and serum ferritin levels. Its management poses substantial clinical challenges, and the prognosis is generally guarded.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Main symptoms and\/or important clinical findings<\/jats:title>\n                    <jats:p>\n                      The patient was admitted due to persistent fatigue for 20 days and recurrent fever. A complete blood count showed pancytopenia: white blood cells (2.99 \u00d710\n                      <jats:sup>9<\/jats:sup>\n                      \/L), red blood cells (2\u00d710\u00b9\u00b2\/L), hemoglobin (70g\/L), and platelets (15\u00d710\n                      <jats:sup>9<\/jats:sup>\n                      \/L). Ferritin levels exceeded 2000\u00b5g\/L, lactate dehydrogenase (LDH) was elevated to 1414 U\/L and triglyceride was normal. The coagulation profile indicated normal fibrinogen levels; however, its degradation product was elevated (7.70\u03bcg\/mL), along with increased plasma D-dimer (1.48\u03bcg\/mL). Elevated inflammatory markers included C-reactive protein (33.90mg\/L) and procalcitonin (1.400ng\/mL). A non-contrast computed tomography (CT) scan revealed bilateral pulmonary inflammatory exudation, atelectasis, and splenomegaly.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>The main diagnoses, therapeutic interventions, and outcomes<\/jats:title>\n                    <jats:p>Comprehensive bone marrow evaluation confirmed a diagnosis of AEL complicated by secondary HLH. Initial therapy with a decitabine-CAG (aclacinomycin, cytarabine, G-CSF)-venetoclax regimen failed to induce remission. Morphological complete remission was achieved after switching to a DAE (daunorubicin, cytarabine, etoposide) regimen. Despite plans for allogeneic hematopoietic stem cell transplantation, the patient succumbed within 3 months of diagnosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This case highlights the diagnostic and therapeutic complexities associated with the co-occurrence of AEL and HLH. Early identification of HLH as a potential complication in AEL is crucial, though outcomes remain dismal, emphasizing an urgent need for novel therapeutic strategies.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fonc.2026.1768823","type":"journal-article","created":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T05:18:29Z","timestamp":1771219109000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Diagnosis and management of rare acute erythroid leukemia with hemophagocytic lymphohistiocytosis: a case 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