title: [Successful treatment with bortezomib for a patient with plasma cell leukemia accompanied by severe hyperbilirubinemia].
abstract: A 59-year-old woman was admitted to our hospital with jaundice, renal dysfunction, anemia and hypercalcemia. Primary plasma cell leukemia (PCL) was diagnosed based on findings of IgA-λ type M-protein, 22% plasma cells in the bone marrow and 23.1% plasma cells of WBC in the peripheral blood. Because the total bilirubin (T.Bil) level increased even after the administration of prednisolone (PSL), dexamethasone and methylprednisolone, the patient was started on bortezomib (0.7 mg/m(2) on days 1, 4, 8 and 11 for 3 weeks) combined with PSL (40 mg/day). The level of T.Bil decreased and the patient's condition remarkably improved. We then increased the dose of bortezomib to 1.0 mg/m(2) in the second course, but discontinued treatment just after starting the third course because NCI-CTCAE Grade 3 peripheral neuropathy developed. According to the criteria of the International Myeloma Working Group, the response category was VGPR (=very good partial response) at 1 month after pausing treatment. We recommend these novel agents for PCL, which is an aggressive form of extramedullary plasma cell cancer.
authors.affiliation: Department of Hematology & Oncology, RIRBM, Hiroshima University.
authors.forename: Tatsuji
authors.initials: T
authors.lastname: Mino
authors.forename: Akira
authors.initials: A
authors.lastname: Sakai
authors.forename: Miki
authors.initials: M
authors.lastname: Kinoshita
authors.forename: Tetsumi
authors.initials: T
authors.lastname: Yoshida
authors.forename: Keiichiro
authors.initials: K
authors.lastname: Mihara
authors.forename: Jun
authors.initials: J
authors.lastname: Imagawa
authors.forename: Akiro
authors.initials: A
authors.lastname: Kimura
mesh_terms: D000970:Antineoplastic Agents / Q000008:administration & dosage* / Q000009:adverse effects; D001897:Boronic Acids / Q000008:administration & dosage* / Q000009:adverse effects; D000069286:Bortezomib; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D006932:Hyperbilirubinemia / Q000150:complications*; D007952:Leukemia, Plasma Cell / Q000150:complications* / Q000188:drug therapy*; D011239:Prednisolone / Q000008:administration & dosage; D011719:Pyrazines / Q000008:administration & dosage* / Q000009:adverse effects; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D001897:Boronic Acids; D011719:Pyrazines; D000069286:Bortezomib; D011239:Prednisolone