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Open Access Case report

Treatment of splenic marginal zone lymphoma of the CNS with high-dose therapy and allogeneic stem cell transplantation

Christoph Busemann1, Andrej Gudzuhn1, Carsten Hirt1, Michael Kirsch2, Silke Vogelgesang3, Christian A Schmidt1, Gottfried Dölken1 and William H Krüger14*

Author Affiliations

1 Internal Medicine C (Hematology and Oncology, Palliative Care, Transplant Centre), University Hospital Greifswald, Ernst-Moritz-Arndt-University, Greifswald, Germany

2 Institute for Radiology, University Hospital Greifswald, Ernst-Moritz-Arndt-University, Greifswald, Germany

3 Department of Neuropathology, Institute for Pathology, University Hospital Greifswald, Ernst-Moritz-Arndt-University, Greifswald, Germany

4 Medizinische Klinik C (Hämatologie und Onkologie, Transplantationszentrum), Ernst-Moritz-Arndt-Universität Greifswald, Ferdinand-Sauerbruch-Straße, Greifswald, 17475, Germany

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Experimental Hematology & Oncology 2012, 1:32  doi:10.1186/2162-3619-1-32

Published: 16 October 2012

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.

Keywords:
Non-Hodgkin’s lymphoma; Allogeneic stem cell transplantation; Graft-versus-lymphoma effect; Marginal zone lymphoma