title: Extramedullary blast crisis in chronic myeloid leukemia.
abstract: Among 235 patients with CML we reviewed 91 patients with BC diagnosed between 1980 and 1995; 15 of the 91 (16%) developed extramedullary disease (EMD). The sites involved were the lymph nodes (13/15), CNS (1/15) and suborbital mass (1/15). The appearance of EMD was associated with chronic phase (CP) features in the bone marrow in 3/15 cases, with accelerated phase (AP) in 3/15 and with BC in 9/15. 11/15 (73%) cases of EMD were classified as myeloid (My-EMD) and 4/15 as lymphoid-type (Ly-EMD): three B-phenotype and one T-phenotype. All Ly-EMD cases were treated with vincristine, daunorubicin and prednisone and obtained complete remission (CR). Cases of My-EMD were treated with daunorubicin and cytosine arabinoside, of which only 1/11 achieved CR. We suggest that in EMD also, the type, lymphoid or myeloid, of BC has a bearing on treatment response and prognosis: Ly-EMD is more responsive to treatment and has longer survival than My-EMD.
authors.affiliation: Department of Hematology, University of Bari, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Specchia
authors.forename: G
authors.initials: G
authors.lastname: Palumbo
authors.forename: D
authors.initials: D
authors.lastname: Pastore
authors.forename: D
authors.initials: D
authors.lastname: Mininni
authors.forename: A
authors.initials: A
authors.lastname: Mestice
authors.forename: V
authors.initials: V
authors.lastname: Liso
mesh_terms: D000328:Adult; D000368:Aged; D018906:Antineoplastic Agents, Alkylating / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001752:Blast Crisis / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology* / Q000532:radiotherapy; D016026:Bone Marrow Transplantation; D002066:Busulfan / Q000627:therapeutic use; D002490:Central Nervous System / Q000473:pathology*; D003131:Combined Modality Therapy; D003561:Cytarabine / Q000008:administration & dosage; D003630:Daunorubicin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000627:therapeutic use; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000453:epidemiology / Q000473:pathology* / Q000628:therapy; D015465:Leukemia, Myeloid, Accelerated Phase / Q000188:drug therapy / Q000473:pathology; D015466:Leukemia, Myeloid, Chronic-Phase / Q000188:drug therapy / Q000473:pathology; D008198:Lymph Nodes / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009915:Orbit / Q000473:pathology*; D011241:Prednisone / Q000008:administration & dosage; D011994:Recombinant Proteins; D012074:Remission Induction; D012189:Retrospective Studies; D015996:Survival Rate; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D018906:Antineoplastic Agents, Alkylating; D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins; D003561:Cytarabine; D014750:Vincristine; D002066:Busulfan; D011241:Prednisone; D006918:Hydroxyurea; D003630:Daunorubicin