Hemophagocytic Syndrome Caused by All Trans-Retinoic Acid Following Induction Chemotherapy in Two Patients with Acute Promyelocytic Leukemia (M3)

• Published in: The Japanese Journal of Pediatric Hematology Vol. 20; no. 1; pp. 39 - 44
• Main Authors: KOBAYASHI, Ryoji, IGUCHI, Akihiro, SATO, Tomonobu, KANEDA, Makoto
• Format: Journal Article
• Language: Japanese
• Published: THE JAPANESE SOCIETY OF PEDIATRIC HEMATOLOGY/ONCOLOGY 2006; 特定非営利活動法人 日本小児血液・がん学会
• Subjects: acute promyelocytic leukemia (M3); all trans-retinoic acid (ATRA); hemophagocytic syndrome (HPS); induction chemotherapy
• ISSN: 0913-8706; 1884-4723
• DOI: 10.11412/jjph1987.20.39

We present two patients who developed hemophagocytic syndrome (HPS) associated with all trans-retinoic acid (ATRA) after induction chemotherapy for acute promyelocytic leukemia (M3). Case 1 : A 4-year-old girl was diagnosed as having M3 and was treated with chemotherapy including ATRA. Thirty days after starting chemotherapy, she developed HPS. Case 2 : A 17-year-old boy was diagnosed as having M3 and was also treated with chemotherapy including ATRA. Thirty-seven days after starting chemotherapy, he developed HPS. A drug-lymphocyte stimulation test against ATRA was positive in case 2. In both cases, the patients recovered immediately after treatment with prednisolone, with no recurrence of HPS, although the patients received ATRA during subsequent consolidation or maintenance chemotherapy phases. We speculate that the HPS-associated ATRA administration following induction chemotherapy was due to enhancement of hypercytokinemia during the hematological recovery period or was one of the late onset symptoms of ATRA syndrome.