title: [Successful treatment of acute promyelocytic leukemia complicated with autoimmune hepatitis-induced portal hypertension with all-trans retinoic acid].
abstract: A 35-year-old man admitted to the hospital for oral hemorrhage was diagnosed with acute promyelocytic leukemia (APL). Remission from APL was achieved by induction therapy with all-trans retinoic acid (ATRA); the PML/RARA fusion gene was not detected on PCR analysis. Despite complete molecular remission, severe persistent pancytopenia, massive ascites, and renal failure were observed. The liver surface appeared rough and irregular on computed tomographic images. On the basis of the liver biopsy results, we diagnosed his condition as portal hypertension due to autoimmune hepatitis. Indocyanine green test showed good residual function of the liver, and therefore, 2 courses of consolidation therapy were administered; chemotherapy was stopped because of severe pancytopenia due to portal hypertension. Instead of continuing the consolidation therapy, maintenance therapy involving 8 rounds of ATRA monotherapy (45 mg/m(2), days1∼14) was initiated. Portal hypertension did not progress further with this maintenance therapy and therefore it was continued. The patient has been in remission from APL ever since, and no relapses have occurred since the past 5 years. These results suggest that ATRA can be used for long-term therapy in such cases.
authors.affiliation: Department of Hematology, Niigata University Graduate School of Medical and Dental Sciences.
authors.forename: Takashi
authors.initials: T
authors.lastname: Ushiki
authors.forename: Koji
authors.initials: K
authors.lastname: Nikkuni
authors.forename: Chie
authors.initials: C
authors.lastname: Yoshida
authors.forename: Yasuhiko
authors.initials: Y
authors.lastname: Shibasaki
authors.forename: Toru
authors.initials: T
authors.lastname: Ishikawa
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Masuko
authors.forename: Kazue
authors.initials: K
authors.lastname: Takai
mesh_terms: D000328:Adult; D000970:Antineoplastic Agents / Q000008:administration & dosage*; D019693:Hepatitis, Autoimmune / Q000150:complications*; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology*; D015473:Leukemia, Promyelocytic, Acute / Q000150:complications / Q000188:drug therapy* / Q000235:genetics; D008297:Male; D009687:Nuclear Proteins; D000071156:Promyelocytic Leukemia Protein; D018168:Receptors, Retinoic Acid; D012074:Remission Induction; D000072482:Retinoic Acid Receptor alpha; D014157:Transcription Factors; D014212:Tretinoin / Q000008:administration & dosage*; D025521:Tumor Suppressor Proteins
chemical_list: D000970:Antineoplastic Agents; D009687:Nuclear Proteins; D000071156:Promyelocytic Leukemia Protein; C000607071:RARA protein, human; D018168:Receptors, Retinoic Acid; D000072482:Retinoic Acid Receptor alpha; D014157:Transcription Factors; D025521:Tumor Suppressor Proteins; C070576:PML protein, human; D014212:Tretinoin