title: Restoration of cytogenetically normal marrow cells after remission of lymphoblastic crisis in a case of Ph positive CML treated with alpha-interferon.
abstract: A patient with Philadelphia (Ph) chromosome-positive chronic myelogenous leukemia (CML) who was treated with alpha-interferon (alpha-IFN) is reported. After the treatment, the number of Ph+ bone marrow (BM) cells decreased gradually and the intensity of the rearranged major breakpoint cluster region (M-BCR) gene became faint; however, a lymphoblastic crisis developed about 1 year later. At the time of the blast crisis, the rearranged M-BCR band was detected, indicating that the blast crisis clone was derived from CML cells. The patient was treated with a combination of vincristine, prednisolone, daunorubicin, and L-asparaginase, and a hematologic remission was obtained. During the remission status, no rearranged M-BCR fragment was detected by conventional Southern analysis. Thus, the hematologic and genetic alteration in this case appeared to be identical to Ph+ acute leukemia with M-BCR rearrangement. The current case therefore indicates that alpha-IFN can reduce the proportion of Ph+ cells, but is unable to prevent blast crisis. Furthermore, the quantitative reduction of the cell population with a Ph chromosome may have some effects in modifying the genetic manifestations and clinical features of Ph+ CML, e.g., the delay in the appearance of the blast crisis.
authors.affiliation: First Department of Internal Medicine, Tokyo Medical College, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Kawakubo
authors.forename: J H
authors.initials: JH
authors.lastname: Ohyashiki
authors.forename: K
authors.initials: K
authors.lastname: Ohyashiki
authors.forename: T
authors.initials: T
authors.lastname: Tauchi
authors.forename: T
authors.initials: T
authors.lastname: Ohto
authors.forename: K
authors.initials: K
authors.lastname: Toyama
mesh_terms: D015703:Antigens, CD / Q000032:analysis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001215:Asparaginase / Q000008:administration & dosage; D001752:Blast Crisis / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D001853:Bone Marrow / Q000473:pathology*; D002869:Chromosome Aberrations; D002871:Chromosome Banding; D003630:Daunorubicin / Q000008:administration & dosage; D015321:Gene Rearrangement; D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use*; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000008:administration & dosage; D012074:Remission Induction; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D015703:Antigens, CD; D016898:Interferon-alpha; D014750:Vincristine; D011239:Prednisolone; D001215:Asparaginase; D003630:Daunorubicin