Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0855152 (Primary mediastinal large B-cell lymphoma)
 46 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary mediastinal large B-cell lymphoma (PMLCL) is an aggressive non-Hodgkin lymphoma with distinct clinical and gene expression profiles. Outcomes of salvage chemotherapy and autologous stem cell transplantation (ASCT) for relapsed or refractory disease (RR) have not been well characterised. We retrospectively identified 180 consecutive RR patients (37 PMLCL and a control group of 143 DLBCL) that underwent salvage chemotherapy. The overall response rate (ORR) to salvage chemotherapy (25% vs. 48%, p = 0.01) and 2-year OS after diagnosis of RR disease (15% vs. 34%, p = 0.018) was inferior in PMLCL patients. The 2-year post-ASCT OS (67% PMLCL vs. 53%, p = 0.78) and PFS (57% PMLCL vs. 36%, p = 0.64) were similar. RR PMLCL had an inferior ORR and survival compared with DLBCL but chemosensitive PMLCL and DLBCL patients have similar outcomes post-ASCT. Strategies for PMLCL should focus on identifying poor risk patients to test novel induction and salvage strategies.
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PMID:Salvage chemotherapy and autologous stem cell transplantation are inferior for relapsed or refractory primary mediastinal large B-cell lymphoma compared with diffuse large B-cell lymphoma. 1860 22

Primary mediastinal large B-cell lymphoma (PMLBCL) is a distinct clinical and biological disease from other types of DLBCL. It is more frequent in young female and constitutes 6%-10% of all DLBCL. PMLBCL is characterized by a diffuse proliferation of medium to large B-cells associated with sclerosis. Molecular analysis shows it to be a distinct entity from other DLBCL. Rituximab CHOP/MACOP-B-like regimens followed by mediastinal radiotherapy (RT) were associated with a 5-years PFS of 75%-85%. More intensive regimens, as DA-EPOCH-R without mediastinal RT, have shown very promising results, but this therapeutic advance needs to be confirmed in further prospective trials. The role of consolidative mediastinal RT should be still better assess in prospective comparative studies. PET-CT scan is a powerful tool to define the real quality of response and it is hoped that future prospective trials may allow its role in the de-escalation of mediastinal RT.
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PMID:Primary mediastinal large B-cell lymphoma. 2831 92