scholarly journals Bing and Neel Syndrome

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
S. Jennane ◽  
K. Doghmi ◽  
E. M. Mahtat ◽  
N. Messaoudi ◽  
B. Varet ◽  
...  

Introduction. We report the case of a Bing and Neel syndrome revealed by an isolated left ptosis.Case Report. a 57-year-old man was followed up since October 2003 for a typical Waldenström’s macroglobulinemia. A first complete remission was obtained with chlorambucil. In August 2004, he relapsed. A second complete remission was obtained with RFC chemotherapy regimen (rituximab, fludarabine, and cyclophosphamide). In October 2009, the patient presented with an isolated left ptosis revealing a Bing and Neel syndrome. The diagnosis was suspected on MRI and confirmed by the detection in the CSF of a monoclonal IgM similar to the one found in the plasma. A quite good partial remission has been obtained after one course of RDHAP (rituximab, dexamethasone, cytarabine, and cisplatin) and 3 courses of RDHOx (rituximab, dexamethasone, cytarabine, and oxaliplatin), in addition to ten intrahectal chemotherapy injections. The treatment was followed by intensification and autologous stem cell transplantation. At D58, the patient died due to a septic shock.Conclusion. BNS is a rare and potentially treatable complication of WM. It should be considered in patients with neurologic symptoms and a history of WM.

2004 ◽  
Vol 22 (14) ◽  
pp. 2816-2825 ◽  
Author(s):  
Michael G. Kiehl ◽  
Ludwig Kraut ◽  
Rainer Schwerdtfeger ◽  
Bernd Hertenstein ◽  
Mats Remberger ◽  
...  

Purpose The role of unrelated allogeneic stem-cell transplantation in acute lymphoblastic leukemia (ALL) patients is still not clear, and only limited data are available from the literature. We analyzed factors affecting clinical outcome of ALL patients receiving a related or unrelated stem-cell graft from matched donors. Patients and Methods The total study population was 264 adult patients receiving a myeloablative allogeneic stem-cell transplant for ALL at nine bone marrow transplantation centers between 1990 and 2002. Of these, 221 patients receiving a matched related or unrelated graft were analyzed. One hundred forty-eight patients received transplantation in complete remission; 62 patients were in relapse; and 11 patients were refractory to chemotherapy before transplant. Fifty percent of patients received bone marrow, and 50% received peripheral blood stem cell from a human leukocyte antigen–identical related (n = 103), or matched unrelated (n = 118) donor. Results Disease-free survival (DFS) at 5 years was 28%, with 76 patients (34%) still alive (2.2 to 103 months post-transplantation), and 145 deceased (65 relapses, transplant-related mortality, 45%). We observed an advantage regarding DFS in favor of patients receiving transplantation during their first complete remission (CR) in comparison with patients receiving transplantation in or after second CR (P = .014) or who relapsed (P < .001). We observed a clear trend toward improved survival in favor of B-lineage ALL patients compared with T-lineage ALL patients (P = .052), and Philadelphia chromosome–positive patients had no poorer outcome than Philadelphia chromosome–negative patients. Total-body irradiation–based conditioning improved DFS in comparison with busulfan (P = .041). Conclusion Myeloablative matched related or matched unrelated allogeneic hematopoietic stem-cell transplantation in ALL patients should be performed in first CR.


2016 ◽  
Vol 29 (3) ◽  
pp. 205
Author(s):  
Margarida Dantas Brito ◽  
Fernando Campilho ◽  
Rosa Branca ◽  
Carlos Vaz ◽  
Susana Roncon ◽  
...  

<p><strong>Introduction:</strong> Diffuse large B-cell lymphoma can be cured in 60% – 70% of patients. Autologous stem cell transplantation is the standard treatment for relapsed disease. This high-intensity treatment after first complete remission in patients with high International Prognostic Index remains controversial and was performed in our department during some years. <br /><strong>Material and Methods:</strong> Retrospective study, review of clinical records. <br /><strong>Results:</strong> This study evaluates the outcome of 113 patients transplanted between 1992 and 2012. Considering status before transplantation patients were divided in groups: a) first complete remission after 1 line of chemotherapy (n = 64); b) first complete remission after ≥ two chemotherapy lines (n = 15); c) second complete remission (n = 15); d) more advanced diseased (n = 19). Chemotherapy used in first line therapy was mainly R-CHOP (n = 71) and CHOP (n = 28). The median follow-up of patients still alive was 34 months (1 - 221). At five years, overall survival was 73% (± 5) and disease free survival was 75% (± 5).<br /><strong>Conclusion:</strong> Conventional chemotherapy followed by autologous stem cell transplant is a safe and efficient option for selected patients. In our series 70% high-risk patients were free from disease with this strategy.</p>


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