Mantle cell lymphoma, in leukaemic phase with prominent splenomegaly. A report of eight cases with similar clinical presentation and aggressive outcome

Mantle cell lymphoma (MCL) is a well-defined peripheral B-cell lymphoma usu ally diagnosed upon peripheral lymph node biopsy. We report eight cases of peripheral B-cell leukaemia that demonstrate presumptive evidence of mantle cell characteristics. The patients had a median age of 68.5 years, and fiv e were male. All presented with an enlarged spleen without any peripheral l ymphadenopathies, and they were leukaemic at presentation (median lymphocyt osis, 38x10(9)/1). Morphological diagnosis of MCL was very difficult in fiv e cases but easier in three because we were able to analyse either pre- or post-mortem lymph nodes and spleen. The immunophenotype of blood lymphocyto sis using flow cytometry, the presence of a t(11;14)(q13;q32) and a cyclin D1 expression by leukaemic cells all fit with the diagnosis of MCL. All pat ients progressed and died with a median overall survival of 8 months. Multi focal areas of transformation in blastoid or large cell variants were obser ved in the three autopsied patients. In summary, one should consider the di agnosis of MCL at presentation in leukaemic phase even in the absence of pe ripheral adenopathies.