title: Hemophagocytic syndrome associated with retinoic acid syndrome in acute promyelocytic leukemia.
abstract: A 56-year-old woman with an acute promyelocytic leukemia (APL) developed a severe all-trans-retinoic (ATRA) syndrome on day 17 of treatment. Shortly after, she presented a picture of pancytopenia, hepatosplenomegaly, increased triglycerides, ferritin, and liver enzymes. A bone marrow biopsy showed abundant macrophages and no evidence of leukemia. Tests for secondary hemophagocytic syndrome (HPS) were negative. A diagnosis of HPS was made. Treatment with dexamethasone and high-dose immunoglobulins was unsuccessful. Consolidation chemotherapy with idarubicin and ATRA rapidly reversed the HPS. The HPS in this patient could be related to the release of macrophage-stimulating cytokines by APL cells during ATRA syndrome.
authors.affiliation: Service of Hematology, Príncipe de Asturias University Hospital, Alcalá de Henares, Madrid, Spain. jgarciasu.hupa@salud.madrid.org
authors.forename: J
authors.initials: J
authors.lastname: García-Suárez
authors.forename: H
authors.initials: H
authors.lastname: Bañas
authors.forename: I
authors.initials: I
authors.lastname: Krsnik
authors.forename: D
authors.initials: D
authors.lastname: De Miguel
authors.forename: E
authors.initials: E
authors.lastname: Reyes
authors.forename: C
authors.initials: C
authors.lastname: Burgaleta
mesh_terms: D000970:Antineoplastic Agents / Q000009:adverse effects; D005260:Female; D015614:Histiocytosis / Q000139:chemically induced*; D006801:Humans; D015473:Leukemia, Promyelocytic, Acute / Q000188:drug therapy*; D008875:Middle Aged; D013577:Syndrome; D016896:Treatment Outcome; D014212:Tretinoin / Q000009:adverse effects*
chemical_list: D000970:Antineoplastic Agents; D014212:Tretinoin