• Hemophagocytic Lymphohistiocytosis as the initial presentation of pediatric Acute Lymphoblastic Leukemia
  • Atousa Moghadam Fard,1,* Gholamreza Bahoush,2
    1. Student Research Committee, School of Medicine, Iran University of Medical Sciences, Tehran, Iran
    2. Associate Professor, Pediatric Hematologist and Oncologist, Ali-Asghar Children Hospital, Department of Pediatrics, Faculty of Medicine, Iran University of Medical Sciences, Tehran, Iran


  • Introduction: Hemophagocytic Lymphohistiocytosis (HLH) is a multisystem inflammatory syndrome that is rare and life-threatening. It causes immune dysregulation, prolonged hyper-inflammatory conditions, and cytokine storm. Acute lymphoblastic leukemia (ALL) is one of the malignancies which can have an initial presentation of HLH.
  • Methods: A case report study
  • Results: We presented a 7-year-old child who at first had manifestations of HLH based on pathology results and HLH-2004 criteria. She received chemotherapy treatment based on the HLH-2004 chemotherapy regime; after the chemotherapy treatment, she achieved complete remission and was successfully treated. About 48 weeks after starting chemotherapy, cervical lymphadenopathies and consistent fever were found. Based on the results of lymphadenopathy biopsy and bone marrow aspiration, the patient was diagnosed with precursor B-cell ALL.
  • Conclusion: We found that HLH could be the initial presentation of B-cell leukemia. It is essential to rule out B-cell leukemia in patients with secondary HLH.
  • Keywords: Hemophagocytic lymphohistiocytosis, Acute lymphoblastic leukemia, HLH, ALL