BING NEEL SYNDROME, ABOUT A CASE REPORT AND REVIEW OF LITT
(Abstract release date: 05/21/15)
EHA Library. Bougherira S. 06/12/15; 102685; PB1869
Disclosure(s): faculty of medecine, university hospital of Annabahematology
Soraya Bougherira
Contributions
Contributions
Abstract
Abstract: PB1869
Type: Publication Only
Background
Waldenstrom’s Macroglobulinaemia (WM) is is defined by the World Health Organization as an IgM secreting 'lymphoplasmacytic lymphoma, characterized by BM infiltration, and occasionally lymph nodes and spleen.
Aims
We describe a patient with a long history of WM presenting Bing and Neel syndrome revealed by convulsion.
Methods
68-year-old man was followed up in our department since September 2002 for a typical WM. A first complete remission was obtained with CHOP. In March 2007, he relapsed, and a second complete remission was obtained with chlorambucil. FCR therapy regimen (fludarabine, cyclophosphamide and rituximab) was used on July 2011. In March 2014, the patient presented with persistant headaches, episods of convulsion, blurry vision and disorders of memory. Computerized tomography of the brain showed a tumor mass multifocal supratentorial with edema. Brain magnetic resonance imaging displayed pachymeningeal regions of enhancement with associated FLAIR hypertensity on T2-weighted sequences. Examination of the cerebrospinal fluid (CSF) showed a lymphocytic meningitis with an increase of the CSF protein, and a normal CSF glucose. The patient did not undergo biopsy. The staging including full body scan, bone marrow aspiration, and bone marrow biopsy was normal, no organomegaly, meaning stable WM. Our patient underwent successful treatment with MPV-A regimen chemotherapy of Methotrexate 3,5 g/m² D1/ Vincristine 1,4 mg/m² max 2,8 D1/Procarbazine 100 mg/m² D1to D7(cycles 1, 3, & 5) - 5 cycles repeated every 15 days, at final a closing course with AraC 3g/m² D1 and D2 without radiation therapy.
Results
The evolution was characterized by disparition of clinical symptoms, a marked regression of lesions on MRI and normalization of CSF analysis. The patient is still alive with free-symptoms in December 2014, last scheduled visit.
Summary
BN syndrome is a very rare complication of WM that should be considered in patients with neurologic symptoms and a history of WM. A brain MRI and histologic analysis may be a good supportive tool to diagnose Bing-Neel syndrome.There is still no consensus on the treatment strategies to use in BNS.
Type: Publication Only
Background
Waldenstrom’s Macroglobulinaemia (WM) is is defined by the World Health Organization as an IgM secreting 'lymphoplasmacytic lymphoma, characterized by BM infiltration, and occasionally lymph nodes and spleen.
The most frequent neurological complication of Waldenström’s macroglobulinemia is IgM-mediated polyneuropathy. Direct tumor cell infiltration of the nervous system is extremely rare and better known as the “Bing and Neel Syndrome” (BNS), that was first described in 1936.
Aims
We describe a patient with a long history of WM presenting Bing and Neel syndrome revealed by convulsion.
Methods
68-year-old man was followed up in our department since September 2002 for a typical WM. A first complete remission was obtained with CHOP. In March 2007, he relapsed, and a second complete remission was obtained with chlorambucil. FCR therapy regimen (fludarabine, cyclophosphamide and rituximab) was used on July 2011. In March 2014, the patient presented with persistant headaches, episods of convulsion, blurry vision and disorders of memory. Computerized tomography of the brain showed a tumor mass multifocal supratentorial with edema. Brain magnetic resonance imaging displayed pachymeningeal regions of enhancement with associated FLAIR hypertensity on T2-weighted sequences. Examination of the cerebrospinal fluid (CSF) showed a lymphocytic meningitis with an increase of the CSF protein, and a normal CSF glucose. The patient did not undergo biopsy. The staging including full body scan, bone marrow aspiration, and bone marrow biopsy was normal, no organomegaly, meaning stable WM. Our patient underwent successful treatment with MPV-A regimen chemotherapy of Methotrexate 3,5 g/m² D1/ Vincristine 1,4 mg/m² max 2,8 D1/Procarbazine 100 mg/m² D1to D7(cycles 1, 3, & 5) - 5 cycles repeated every 15 days, at final a closing course with AraC 3g/m² D1 and D2 without radiation therapy.
Results
The evolution was characterized by disparition of clinical symptoms, a marked regression of lesions on MRI and normalization of CSF analysis. The patient is still alive with free-symptoms in December 2014, last scheduled visit.
Summary
BN syndrome is a very rare complication of WM that should be considered in patients with neurologic symptoms and a history of WM. A brain MRI and histologic analysis may be a good supportive tool to diagnose Bing-Neel syndrome.There is still no consensus on the treatment strategies to use in BNS.
Abstract: PB1869
Type: Publication Only
Background
Waldenstrom’s Macroglobulinaemia (WM) is is defined by the World Health Organization as an IgM secreting 'lymphoplasmacytic lymphoma, characterized by BM infiltration, and occasionally lymph nodes and spleen.
Aims
We describe a patient with a long history of WM presenting Bing and Neel syndrome revealed by convulsion.
Methods
68-year-old man was followed up in our department since September 2002 for a typical WM. A first complete remission was obtained with CHOP. In March 2007, he relapsed, and a second complete remission was obtained with chlorambucil. FCR therapy regimen (fludarabine, cyclophosphamide and rituximab) was used on July 2011. In March 2014, the patient presented with persistant headaches, episods of convulsion, blurry vision and disorders of memory. Computerized tomography of the brain showed a tumor mass multifocal supratentorial with edema. Brain magnetic resonance imaging displayed pachymeningeal regions of enhancement with associated FLAIR hypertensity on T2-weighted sequences. Examination of the cerebrospinal fluid (CSF) showed a lymphocytic meningitis with an increase of the CSF protein, and a normal CSF glucose. The patient did not undergo biopsy. The staging including full body scan, bone marrow aspiration, and bone marrow biopsy was normal, no organomegaly, meaning stable WM. Our patient underwent successful treatment with MPV-A regimen chemotherapy of Methotrexate 3,5 g/m² D1/ Vincristine 1,4 mg/m² max 2,8 D1/Procarbazine 100 mg/m² D1to D7(cycles 1, 3, & 5) - 5 cycles repeated every 15 days, at final a closing course with AraC 3g/m² D1 and D2 without radiation therapy.
Results
The evolution was characterized by disparition of clinical symptoms, a marked regression of lesions on MRI and normalization of CSF analysis. The patient is still alive with free-symptoms in December 2014, last scheduled visit.
Summary
BN syndrome is a very rare complication of WM that should be considered in patients with neurologic symptoms and a history of WM. A brain MRI and histologic analysis may be a good supportive tool to diagnose Bing-Neel syndrome.There is still no consensus on the treatment strategies to use in BNS.
Type: Publication Only
Background
Waldenstrom’s Macroglobulinaemia (WM) is is defined by the World Health Organization as an IgM secreting 'lymphoplasmacytic lymphoma, characterized by BM infiltration, and occasionally lymph nodes and spleen.
The most frequent neurological complication of Waldenström’s macroglobulinemia is IgM-mediated polyneuropathy. Direct tumor cell infiltration of the nervous system is extremely rare and better known as the “Bing and Neel Syndrome” (BNS), that was first described in 1936.
Aims
We describe a patient with a long history of WM presenting Bing and Neel syndrome revealed by convulsion.
Methods
68-year-old man was followed up in our department since September 2002 for a typical WM. A first complete remission was obtained with CHOP. In March 2007, he relapsed, and a second complete remission was obtained with chlorambucil. FCR therapy regimen (fludarabine, cyclophosphamide and rituximab) was used on July 2011. In March 2014, the patient presented with persistant headaches, episods of convulsion, blurry vision and disorders of memory. Computerized tomography of the brain showed a tumor mass multifocal supratentorial with edema. Brain magnetic resonance imaging displayed pachymeningeal regions of enhancement with associated FLAIR hypertensity on T2-weighted sequences. Examination of the cerebrospinal fluid (CSF) showed a lymphocytic meningitis with an increase of the CSF protein, and a normal CSF glucose. The patient did not undergo biopsy. The staging including full body scan, bone marrow aspiration, and bone marrow biopsy was normal, no organomegaly, meaning stable WM. Our patient underwent successful treatment with MPV-A regimen chemotherapy of Methotrexate 3,5 g/m² D1/ Vincristine 1,4 mg/m² max 2,8 D1/Procarbazine 100 mg/m² D1to D7(cycles 1, 3, & 5) - 5 cycles repeated every 15 days, at final a closing course with AraC 3g/m² D1 and D2 without radiation therapy.
Results
The evolution was characterized by disparition of clinical symptoms, a marked regression of lesions on MRI and normalization of CSF analysis. The patient is still alive with free-symptoms in December 2014, last scheduled visit.
Summary
BN syndrome is a very rare complication of WM that should be considered in patients with neurologic symptoms and a history of WM. A brain MRI and histologic analysis may be a good supportive tool to diagnose Bing-Neel syndrome.There is still no consensus on the treatment strategies to use in BNS.
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