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

Splenic marginal zone lymphoma (sMZL) has usually a very indolent course and affects the elderly people. Therapy consists of splenectomy and mild chemotherapy upon clinical course [1-5]. Secondary affection of the central nervous system by non-Hodgkin’s lymphoma is associated with a poor prognosis [6]. Here 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.A 42-year old male presented with paraplegia, reduced vibration sense and neuropathic pain in the legs aggravating over the last six months. The further medical history was empty. Clinical examination and computed tomography revealed generalized lymphadenopathy and splenomegaly. B-symptoms were absent. Magnetic resonance imaging (MRI) of the CNS detected a signal intense 9 mm sized lesion within the temporal lobe and thickened filaments of the cauda equina with contrast medium enhancement, suggestive for a malignant meningeosis (Figure 1). The LDH has been elevated in serum (284 U/l, upper normal value: 253 U/l). Electrophoresis and immunofixation gave no hint for a monoclonal gammopathy.Peripheral blood showed a lymphocytosis of 34,000/μl with predominance (92.2% in differential count) of small, mature and partially villous lymphocytes (Figure 2). The immunophenotype was compatible with SMZL (CD19+, CD20+, cy79a+, expression of sIgM, FMC7 and lambda light chain and partial expression of CD5 and CD38, but not CD10, CD11c, CD23, CD103 and CD138). Diagnosis of splenic marginal zone lymphoma was confirmed by histological examination of a lymph node. In the cerebrospinal fluid (CSF) lymphoma cells were detected by morphology (Figure 2) and flow cytometry (Figure 3). 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. The IgH rearrangement of lymphoma cells was sequenced to estab