Acute Respiratory Distress Syndrome as an Initial Presentation of Hemophagocytic Lymphohistiocytosis After Induction Therapy for Acute Myeloid Leukemia

A 7-month-old girl with acute myeloid leukemia (AML) developed acute respiratory distress syndrome (ARDS) during the pancytopenic period after induction chemotherapy. Respiratory failure did not improve despite intensive treatments. Eventually, hemophagocytic lymphohistiocytosis (HLH) was diagnosed based on hemophagocytosis in bone marrow, and high soluble interleukin-2 receptor (sIL-2R) and ferritin levels. Even after cyclosporin A was started against HLH, she did not recover. Autopsy showed macrophage proliferation in bone marrow and lymph nodes. HLH should be considered, even in the pancytopenic period after chemotherapy, when patients develop ARDS that does not respond to supportive therapies.

[1]  A. Filipovich.  Hemophagocytic lymphohistiocytosis and other hemophagocytic disorders. , 2008, Immunology and allergy clinics of North America.

[2]  T. George,et al.  Precursor B‐cell acute lymphoblastic leukemia presenting with hemophagocytic lymphohistiocytosis , 2008, Pediatric blood & cancer.

[3]  C. Urban,et al.  Hemophagocytic lymphohistiocytosis as severe adverse event of antineoplastic treatment in children , 2008, Haematologica.

[4]  L. Glasser,et al.  Florid histiocytic hemophagocytosis following therapy with long acting G‐CSF (pegfilgrastim) , 2007, American journal of hematology.

[5]  M. Aricò,et al.  HLH‐2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis , 2007, Pediatric blood & cancer.

[6]  H. Hennies,et al.  Mutation spectrum in children with primary hemophagocytic lymphohistiocytosis: molecular and functional analyses of PRF1, UNC13D, STX11, and RAB27A , 2006, Human mutation.

[7]  A. Attarbaschi,et al.  Histiocytosis following T-acute lymphoblastic leukemia: A BFM study , 2005, Leukemia & lymphoma.

[8]  S. Ikeda,et al.  Acute respiratory distress syndrome due to systemic lupus erythematosus with hemophagocytic syndrome: an autopsy report , 2005, Clinical Rheumatology.

[9]  N. Fitzgerald,et al.  Imaging characteristics of hemophagocytic lymphohistiocytosis , 2003, Pediatric Radiology.

[10]  E. Nahum,et al.  Hemophagocytic lymphohistiocytic syndrome: Unrecognized cause of multiple organ failure , 2000, Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.