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A multicenter retrospective clinical study of CD5/CD10‐negative chronic B cell leukemias
Author(s) -
Goldaniga Maria,
Ferrario Andrea,
Cortelazzo Sergio,
Guffanti Andrea,
Pavone Enzo,
Ambrosetti Achille,
Marcheselli Luigi,
Rossi Francesca,
Luminari Stefano,
Rossi Andrea,
Cro Lilla,
Federico Massimo,
Lambertenghi Deliliers Giorgio,
Baldini Luca
Publication year - 2008
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.21065
Subject(s) - lymphoplasmacytic lymphoma , medicine , cd5 , gastroenterology , pathological , lymphoproliferative disorders , marginal zone , waldenstrom macroglobulinemia , retrospective cohort study , hematology , lymphoma , pathology , b cell , immunology , antibody
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. Am. J. Hematol. 2008. © 2008 Wiley‐Liss, Inc.