[Leukaemic phase of Mantle zone (intermediate) lymphoma--case report--]

Leukaemic phase of non-Hodgkin lymphoma (NHL) is characterised by penetration of lymphoma cells from the originating tissues (lymph nodes, less commonly the spleen) into the peripheral blood and bone marrow. The diagnosis of leukaemic phase of Mantle zone lymphoma is established on the basis of histological findings of lymph node biopsy and, possibly, the spleen, peripheral blood smear, and characteristic membranous phenotype. A patient, aged 60, is reported with Mantle zone (intermediate lymphoma) in leukaemic phase. Physical examination revealed pallor of the skin, generalized lymphadenopathy, and hepatomegaly. WBC count in the peripheral blood was 22.5 x 109/l, and the smear revealed the presence of pleomorphic lymphoid cells, mainly medium sized, with irregular nucleus or nuclear notches. Immunophenotype studies of mononuclear cells of the peripheral blood showed characteristic membranous phenotype for Mantle zone lymphoma in leukaemic phase: Smlg+ (lambda light chain); HLA-DR+; CD19+; CD22+; CD5+; CD10-; CD25-. Pro-MACE-Cyta-bom protocol was applied resulting in a 13-month-lasting remission. The total survival was 20 months, suggesting poor prognosis of leukaemic phase of Mantle zone lymphoma.