CASE REPORT article
Front. Oncol.
Sec. Hematologic Malignancies
Diagnosis and management of rare acute erythroid leukemia with hemophagocytic lymphohistiocytosis: a case report
Provisionally accepted
- Department of Hematology, Changhai Hospital, Naval Medical University, Shanghai, China, Shanghai, China
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Introduction: 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. Main symptoms and/or important clinical findings: 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 ×10⁹/L), red blood cells (2×10¹²/L), hemoglobin (70g/L), and platelets (15×10⁹/L). Ferritin levels exceeded 2000µg/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 (7.70μg/mL), along with increased plasma D-dimer (1.48μg/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. The main diagnoses, therapeutic interventions, and outcomes: 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. Conclusion: 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.
Keywords: Acute erythroid leukemia, diagnosis, hemophagocytic lymphohistiocytosis, prognosis, therapy
Received: 16 Dec 2025; Accepted: 30 Jan 2026.
Copyright: © 2026 Zhang, Tang and Cheng. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Hui Cheng
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