Hemophagocytic syndrome associated with retinoic acid syndrome in acute promyelocytic leukemia.
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.
García-Suárez
J
J
Service of Hematology, Príncipe de Asturias University Hospital, Alcalá de Henares, Madrid, Spain. jgarciasu.hupa@salud.madrid.org
Bañas
H
H
Krsnik
I
I
De Miguel
D
D
Reyes
E
E
Burgaleta
C
C
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*
D000970:Antineoplastic Agents; D014212:Tretinoin