title: Extreme hyperferritinemia in the setting of acute myeloid leukaemia: a case report of hemophagocytic lymphohistiocytosis.
abstract: BACKGROUND
  Major hyperferritinemia is a rare feature in clinical laboratories associated with a wide variety of disorders, including hemophagocytic lymphohistiocytosis (HLH). The diagnosis of HLH is based on clinical and biological criteria, such as those proposed by the Histiocyte Society. However, several of these criteria are not relevant in the specific setting of hematologic malignancies.
  
  
  METHODS
  A 69-year-old male was treated for an acute myeloid leukaemia. On day 15 after the start of chemotherapy, he developed severe sepsis with high fever, low blood pressure and hepatosplenomegaly.
  
  
  RESULTS
  Blood tests were marked by extreme hyperferritinemia (191,000 µg/L, reference range: 26-388 µg/L) with increased C-reactive protein (87.0 mg/L) and procalcitonin (1.94 µg/L) and aspartate aminotransferase (499 U/L 37 °C) in the setting of chemotherapy-induced aplasia. This unusual extreme ferritinemia led to suspect HLH triggered by an invasive infection. Under intensive treatment, the clinical status improved and ferritin levels significantly decreased.
  
  
  CONCLUSIONS
  The diagnosis of HLH is usually based on clinical and biological criteria, mainly fever, splenomegaly, cytopenias, hypertriglyceridemia, hypofibrinogenemia, hemophagocytosis and hyperferritinemia. In this patient, the diagnosis of HLH was challenging because several criteria, such as hypertriglyceridemia, hemophagocytosis and hypofibrinogenemia, were absent. In addition, some criteria of HLH are not relevant in the setting of hematologic malignancy, in which fever, splenomegaly, cytopenias and elevated lactate dehydrogenase are commonly observed independently of HLH. This unusual case of extremely high ferritinemia emphasizes the important weight of the ferritin level for the diagnosis of HLH in adult patients in the setting of hematologic malignancies.
authors.affiliation: Department of Biochemistry, University Hospital Centre Dijon-Burgundy, France.
authors.forename: Damien
authors.initials: D
authors.lastname: Denimal
authors.affiliation: Department of Biochemistry, University Hospital Centre Dijon-Burgundy, France.
authors.forename: Louise
authors.initials: L
authors.lastname: Ménégaut
authors.affiliation: Department of Clinical Hematology, University Hospital Centre Dijon-Burgundy, France.
authors.forename: Cédric
authors.initials: C
authors.lastname: Rossi
authors.affiliation: Department of Biochemistry, University Hospital Centre Dijon-Burgundy, France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Duvillard
authors.affiliation: Department of Biochemistry, University Hospital Centre Dijon-Burgundy, France.
authors.forename: David
authors.initials: D
authors.lastname: Masson
mesh_terms: D000368:Aged; D005293:Ferritins / Q000097:blood*; D006801:Humans; D015470:Leukemia, Myeloid, Acute / Q000097:blood* / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood* / Q000209:etiology / Q000473:pathology; D008297:Male; D018805:Sepsis / Q000097:blood / Q000139:chemically induced / Q000503:physiopathology*
chemical_list: D005293:Ferritins
keywords: hemophagocytic lymphohistiocytosis; hyperferritinemia; hyperferritinemic syndrome