title: A multicenter retrospective clinical study of CD5/CD10-negative chronic B cell leukemias.
abstract: CD5-negative chronic B cell lymphoproliferative disorders in leukemic phase (B-CLPD) are heterogeneous and relatively uncommon pathologies that often lack a histopathological definition because of the absence of accessible pathological tissue. We describe the clinical features and evolution-related variables of 156 patients with CD5/CD10-negative B-CLPD (median age 66 years, range 25-86). The median follow-up was 51 months (range 6-216), and overall 3- and 5-year survival was respectively 87 and 76%; 50 patients needed therapy at diagnosis, 56 during follow-up, and 50 remained untreated until the last control. A combined clinical, histological, cytomorphological, immunophenotypical, and cytogenetic diagnostic approach allowed the complete classification of only a minority of patients as being affected by splenic marginal zone or lymphoplasmacytic lymphoma; the majority of cases remained unclassifiable. Multivariate analysis showed that the clinicohematological variables adversely related to overall survival were serum LDH levels and age, whereas high serum LDH levels, hemoglobin levels of <11 g/dl, and splenomegaly related to treatment-free time (in "wait and see" cases); only splenomegaly related to time to progression (in treated patients). In conclusion, our retrospective study describes the clinical features and variables related to evolution in a large group of patients with CD5/CD10-negative chronic B-cell lymphoid leukemias and underlines the fact that a probable lymphoplasmacytic or marginal zone normal cell origin can be supposed in such leukemic forms, but never surely demonstrated.
authors.affiliation: UO Ematologia e Centro Trapianti di Midollo, Ospedale Maggiore, IRCCS, University of Milan, Milan, Italy. mariacecilia.goldaniga@virgilio.it
authors.forename: Maria
authors.initials: M
authors.lastname: Goldaniga
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrario
authors.forename: Sergio
authors.initials: S
authors.lastname: Cortelazzo
authors.forename: Andrea
authors.initials: A
authors.lastname: Guffanti
authors.forename: Enzo
authors.initials: E
authors.lastname: Pavone
authors.forename: Achille
authors.initials: A
authors.lastname: Ambrosetti
authors.forename: Luigi
authors.initials: L
authors.lastname: Marcheselli
authors.forename: Francesca
authors.initials: F
authors.lastname: Rossi
authors.forename: Stefano
authors.initials: S
authors.lastname: Luminari
authors.forename: Andrea
authors.initials: A
authors.lastname: Rossi
authors.forename: Lilla
authors.initials: L
authors.lastname: Cro
authors.forename: Massimo
authors.initials: M
authors.lastname: Federico
authors.forename: Giorgio
authors.initials: G
authors.lastname: Lambertenghi Deliliers
authors.forename: Luca
authors.initials: L
authors.lastname: Baldini
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000032:analysis*; D000951:Antigens, Neoplasm / Q000032:analysis*; D016175:B-Lymphocyte Subsets / Q000737:chemistry* / Q000473:pathology; D018450:Disease Progression; D005260:Female; D005434:Flow Cytometry; D005500:Follow-Up Studies; D006801:Humans; D016130:Immunophenotyping; D007621:Karyotyping; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000145:classification; D008297:Male; D008875:Middle Aged; D014411:Neoplastic Stem Cells / Q000737:chemistry* / Q000473:pathology; D012189:Retrospective Studies; D013160:Splenic Neoplasms / Q000175:diagnosis / Q000473:pathology; D016019:Survival Analysis
chemical_list: D015703:Antigens, CD; D000951:Antigens, Neoplasm