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Sivelestat relieves respiratory distress refractory to dexamethasone in all‐trans retinoic acid syndrome: a report of two cases
Author(s) -
Kawasaki Kozo,
Akaike Hiroto,
Miyauchi Ayaka,
Ouchi Kazunobu
Publication year - 2006
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/j.0902-4441.2006.t01-1-ejh2852.x
Subject(s) - medicine , dexamethasone , respiratory distress , refractory (planetary science) , acute promyelocytic leukemia , respiratory failure , surgery , anesthesia , gastroenterology , retinoic acid , chemistry , astrobiology , gene , biochemistry , physics
Treatment with all‐trans retinoic acid (ATRA) improves the prognosis of patients with acute promyelocytic leukemia (APL), but ATRA syndrome may occur as a possible fatal side effect, especially in cases refractory to medication or involving pulmonary hemorrhage. We describe two patients with APL who suffered from intracranial hemorrhage. The first patient was a 16‐yr‐old girl who was treated with ATRA and then developed respiratory distress refractory to treatment with dexamethasone combined with anthracycline‐cytarabine cytoreduction therapy. Treatment with Sivelestat, a small molecule inhibitor of neutrophil elastase, achieved rapid improvement in oxygenation and chest radiograph findings, and the patient has been in complete remission for 24 months. The second patient was a 10‐yr‐old boy in whom pulmonary hemorrhage developed following administration of ATRA, dexamethasone and cytoreduction therapy. Aspiration and administration of Sivelestat improved oxygenation and he remained stable. Hematological improvement was also achieved, but the patient died of brain dysfunction because of cerebral edema accompanied by intracranial bleeding. The two cases suggest that Sivelestat may be effective as an additional agent in the treatment of refractory ATRA syndrome, and, therefore, prospective randomized studies of treatment protocols are warranted.