Abstract
Therapy of indolent lymphomas with involvement of the central nervous system (CNS) has not been standardized so far. A 42-year old male patient presented with neurological signs because of leukemic splenic marginal zone lymphoma (SMZL) manifested in bone marrow, lymph nodes and CNS. Due to the aggressiveness of the disease and the young age of the patient, an intensive immunochemotherapy followed by high-dose therapy with busulfan, thiotepa and fludarabine and subsequent unrelated allogeneic stem cell transplantation (alloSCT) was performed. The haemopoietic stem cells engrafted in time and the patient is doing well (ECOG 0) without evidence for active lymphoma three years after transplantation. Highly sensitive tests by specific quantitative real-time polymerase chain reaction for presence of lymphoma cells in blood and bone marrow indicated also a molecular remission. The reported case shows the feasibility of high-dose therapy and allogeneic stem cell transplantation in high-risk patients with CNS-involvement of indolent non-Hodgkin’s lymphoma. In addition, the case supports the hypothesis that the graft-versus lymphoma effect after alloSCT is also active within the CNS.
Highlights
Splenic marginal zone lymphoma has usually a very indolent course and affects the elderly people
We present the uncommon case of a young man suffering from a very aggressive Splenic marginal zone lymphoma (sMZL) with primary affection of the central nervous system (CNS) and the treatment with allogeneic stem cell transplantation for consolidation
The synopsis of all diagnostic findings in the presented case resulted in the diagnosis of a splenic marginal zone lymphoma with meningeal lymphomatosis, infiltration of the temporal lobe and infiltration of the cauda equina despite the unavailability of a spleen histology as the diagnostic hallmark of this disease
Summary
Splenic marginal zone lymphoma (sMZL) has usually a very indolent course and affects the elderly people. We present the uncommon case of a young man suffering from a very aggressive sMZL with primary affection of the CNS and the treatment with allogeneic stem cell transplantation for consolidation. Cytogenetics showed an additional isochromosome 3q and a translocation t(2;7)(p11;q21-22) in line with the diagnosis of splenic marginal zone lymphoma.
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