title: Hemophagocytic Lymphohistiocytosis in A Child With Very Severe Aplastic Anemia: Double Jeopardy Resulting in Fatality.
abstract: A 7-year-old male child with very severe aplastic anemia developed refractory disease, which was attributed to febrile hemolytic transfusion reactions and was treated with immunosuppressants, which lead to a transient improvement. However, the child worsened subsequently and succumbed to an underlying hemophagocytic lymphohistiocytosis that was recognized late due to an overlap of clinical and biochemical parameters in both the conditions. Hemophagocytic lymphohistiocytosis should be an early suspicion in children with refractory very severe aplastic anemia and the detection of underlying gene mutations can predict disease severity.
authors.affiliation: Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India.
authors.forename: Rumesh
authors.initials: R
authors.lastname: Chandar
authors.forename: Venkatesh
authors.initials: V
authors.lastname: Chandrasekaran
authors.forename: Barath
authors.initials: B
authors.lastname: Jagadisan
authors.forename: Delhi
authors.initials: D
authors.lastname: Kumar
authors.forename: Niranjan
authors.initials: N
authors.lastname: Biswal
mesh_terms: D000741:Anemia, Aplastic / Q000150:complications* / Q000188:drug therapy / Q000628:therapy; D000961:Antilymphocyte Serum / Q000627:therapeutic use; D001803:Blood Transfusion; D002648:Child; D003613:Danazol / Q000627:therapeutic use; D057210:Delayed Diagnosis; D003951:Diagnostic Errors; D018450:Disease Progression; D004351:Drug Resistance; D017809:Fatal Outcome; D064147:Febrile Neutropenia / Q000209:etiology; D006529:Hepatomegaly / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D019190:Iron Overload / Q000209:etiology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood / Q000150:complications / Q000175:diagnosis*; D008297:Male; D013163:Splenomegaly / Q000209:etiology
chemical_list: D000961:Antilymphocyte Serum; D007166:Immunosuppressive Agents; D003613:Danazol