Incidence and risk factors for central nervous system relapse in patients with primary mediastinal large B-cell lymphoma in the rituximab era.

Central nervous system (CNS) involvement is rare in primary mediastinal large B-cell lymphoma (PMLBCL). We aimed to evaluate the incidence of CNS relapse as first treatment failure event and the effect of the induction chemotherapy regimen, central nervous system - international prognostic index (CNS-IPI) and other clinical and laboratory variables on the risk of CNS relapse in 564 PMLBCL patients treated with immunochemotherapy. Only 17 patients (3.0%) received CNS prophylaxis. During a 55-month median follow-up only 8 patients experienced CNS relapse as first event, always isolated. The 2-year cumulative incidence of CNS relapse (CI-CNSR) was 1.47% and remained unchanged thereafter. The CI-CNSR was not affected by the chemotherapy regimen (R-CHOP or R-da-EPOCH). None of the established International Prognostic Index factors for aggressive lymphomas predicted CNS relapse in PMLBCL. The 2-year CI-CNSR in patients with versus without kidney involvement was 13.3% versus 0.96% (p < 0.001); 14.3% versus 1.13% with versus without adrenal involvement (p < 0.001); and 10.2% versus 0.97% with versus without either kidney or adrenal involvement. CNS-IPI was also predictive (2-year CI-CNSR in high-risk vs. intermediate/low-risk: 10.37% vs. 0.84%, p < 0.001). However, this association may be driven mainly by kidney and/or adrenal involvement. In conclusion, in PMLBCL, CNS relapse is rare and appears to be strongly associated with kidney and/or adrenal involvement.

Primary bone lymphoma: a retrospective analysis of 22 patients treated in a single tertiary center.

BACKGROUND: Primary bone lymphoma is a rare disease, representing less than 5% of all extra-nodal non-Hodgkin lymphomas. MATERIALS AND METHODS: We retrospectively searched the database of the lymphoma unit, Hematology/Lymphoma Department, Athens General Hospital 'Evangelismos' for primary bone lymphoma patients. Demographic and clinicopathologic data were collected and overall survival was analyzed. A log-rank test was used in a univariate analysis to identify factors affecting overall survival. RESULTS: We identified 24 and analyzed data from 22 patients. 12 were male (54.5%) and 10 female (45.4%) and their median age was 55 years (range: 19-83). Most patients had localized disease at the time of diagnosis (n = 19, 86.3%), the most common site was the spine (n = 11, 50%) and the most common histology was diffuse large B-cell lymphoma. 21 patients received chemotherapy as initial therapy and 16 received combined chemoradiation. 81.8% of the patients (n = 18) achieved complete remission. 5-year survival rate was 86.3% and overall survival was found to be affected by the patients' initial response to treatment. CONCLUSIONS: Primary bone lymphoma is usually associated with a good prognosis. Prospective studies are needed in order to clarify the effect of immunochemotherapy in overall survival.

Primary renal MALT lymphoma presenting with cryoglobulinaemia.

Primary renal lymphoma is a rare clinicopathologic entity that typically presents as renal mass or renal impairment with enlarged kidneys. We describe the case of a 66-year-old woman who presented with type II mixed cryoglobulinaemic vasculitis as the first manifestation of underlying low-grade primary renal lymphoma.

Primary abdominal muscle lymphoma.

Primary skeletal muscle lymphoma accounts for <1% of all lymphomas; a fraction of these arise within the abdominal muscles. Here, we describe the case of an 84-year-old woman who presented with multiple painful abdominal masses that proved to be diffuse large B-cell lymphoma and we discuss the main features of the disease. Clinical differential diagnosis from soft tissue sarcoma can be extremely difficult. Therefore, accurate pathological diagnosis including optimal tissue processing and immunohistochemical staining is vital to ensuring proper treatment.

A case of acute myelogenous leukaemia characterised by the BCR-FGFR1 translocation.

The 8p11 myeloproliferative syndrome is a rare atypical disorder defined by the presence of rearrangements between the fibroblast growth factor receptor 1 (FGFR1) and 1 of 13 partner genes described to date, including the BCR gene on chromosome 22. The disease characterised by the BCR-FGFR1 fusion gene has distinct biological and clinical features, with significant diversity among the published cases. We report a case of BCR-FGFR1 disease which was presented as acute myeloid leukaemia with an aggressive clinical course and we review all the adult cases published in the literature.