title: The leukemic presentation of mantle-cell lymphoma: disease features and prognostic factors in 58 patients.
abstract: Mantle-cell lymphoma (MCL) is a B-cell malignancy with distinct molecular genetics and pathological features. Peripheral blood involvement has been reported with variable frequency, but information on the natural history of cases presenting with leukemia is lacking. This study aimed to determine the clinical and prognostic features of such cases. We studied clinical features, tumor characteristics, prognostic factors and outcome in 58 patients with leukemic presentation of MCL. Diagnosis was based on morphology, immunophenotype, presence of t(11;14), histology and cyclin D1 expression. The median age was 62 years and male:female 2.4:1. Presenting features included splenomegaly (74%), lymphadenopathy (45%), hepatomegaly (17%) and, in a minority, gastro-intestinal involvement or involvement of Waldeyer's ring; 10% had lymphocytosis alone. Six patients developed central nervous system disease. Median lymphocyte count was 58 x 10(9)/l, 55% had anemia and 17% had thrombocytopenia. Morphology of peripheral blood showed small-cell MCL in 15% of cases, typical MCL in 46% and blastoid MCL in 39%. Immunological markers showed a typical phenotype (CD5+ CD23 -) in 68%, and atypical phenotypes, CD5- CD23- in 17% or CD5+ CD23+ in 15%. CLL scores were 0, 1 or 2 in 96%. Median overall survival was 36 months. Good response to first-line treatment (P = 0.0008) and splenomegaly (P = 0.03) were favorable prognostic factors, while other features including morphology and CD38 expression had no impact on survival or treatment response. This analysis demonstrates that except for splenomegaly, survival of MCL patients presenting with leukemia is not significantly influenced by clinical or tumor characteristics. Splenectomy is a useful treatment option in this group of patients.
authors.affiliation: Academic Department of Haematology and Cytogenetics, The Royal Marsden Hospital NHS Trust, Fulham Road, London SW3 6JJ, UK. estella.matutes@icr.ac.uk
authors.forename: E
authors.initials: E
authors.lastname: Matutes
authors.forename: N
authors.initials: N
authors.lastname: Parry-Jones
authors.forename: V
authors.initials: V
authors.lastname: Brito-Babapulle
authors.forename: A
authors.initials: A
authors.lastname: Wotherspoon
authors.forename: R
authors.initials: R
authors.lastname: Morilla
authors.forename: S
authors.initials: S
authors.lastname: Atkinson
authors.forename: M O
authors.initials: MO
authors.lastname: Elnenaei
authors.forename: P
authors.initials: P
authors.lastname: Jain
authors.forename: G M
authors.initials: GM
authors.lastname: Giustolisi
authors.forename: R P
authors.initials: RP
authors.lastname: A'Hern
authors.forename: D
authors.initials: D
authors.lastname: Catovsky
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D019938:Cyclin D1 / Q000032:analysis; D020732:Cytogenetic Analysis; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D007938:Leukemia / Q000175:diagnosis*; D020522:Lymphoma, Mantle-Cell / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D013156:Splenectomy; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D019938:Cyclin D1