title: Extramedullary disease in Ph'-positive chronic myelogenous leukemia: frequency, clinical features and prognostic significance.
abstract: Of 349 consecutive patients with Philadelphia-positive chronic myelogenous leukemia (Ph' + CML), 14 (4%) developed extramedullary disease (EMD) during their illness. The sites of EMD were: bone (57%), lymph nodes (29%), skin and soft tissues (21%), central nervous system (14%). The median time from diagnosis of CML to the occurrence of EMD was 48 months. At the time of diagnosis of EMD, 7 patients were hematologically in chronic phase, while 7 showed features of accelerated or blastic CML. For patients lacking medullary blastic transformation criteria, the median time from diagnosis of EMD to blast crisis was 4 months. The overall median survival from development of EMD was 5 months. In conclusion, EMD may occur during the course of CML either in the context of a frank blastic transformation, or as an isolated tumoral infiltrate which heralds an impending blast crisis. Its recognition requires a prompt change to acute-phase chemotherapy.
authors.affiliation: Divisione di Ematologia, Istituto Scientifico Policlinico San Matteo, Pavia, Italy.
authors.forename: D
authors.initials: D
authors.lastname: Inverardi
authors.forename: M
authors.initials: M
authors.lastname: Lazzarino
authors.forename: E
authors.initials: E
authors.lastname: Morra
authors.forename: P
authors.initials: P
authors.lastname: Bernasconi
authors.forename: S
authors.initials: S
authors.lastname: Merante
authors.forename: A
authors.initials: A
authors.lastname: Canevari
authors.forename: G
authors.initials: G
authors.lastname: Pagnucco
authors.forename: C
authors.initials: C
authors.lastname: Bernasconi
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000150:complications* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis