LEPTOMENINGEAL MYELOMATOSIS ? A RARE COMPLICATION IN MULTIPLE MYELOMA
(Abstract release date: 05/19/16)
EHA Library. Bommer M. 06/09/16; 134877; PB1977
Dr. Martin Bommer
Contributions
Contributions
Abstract
Abstract: PB1977
Type: Publication Only
Background
Involvement of the central nervous (CNS) system in patients with Multiple Myeloma (MM) is a rare event, occuring mostly during late stage of disease. However, there are reports about an increasing incidence of extramedullary myeloma manifestations since the introduction of so called novel agents.
Aims
We therefore tried to optimize the diagnostic workup in patients with suspected neoplastic meningitis of Multiple Myeloma - so called leptomenigeal myelomatosis (LMM) – by incorporating different techniques.
Methods
Between 04/2005 and 10/2013 we identified 15 cases with CNS-MM. The involvement was confirmed by magnetic resonance imaging (MRI), cerebrospinal fluid cytology as well as by flow cytometry. Additionally, cIg-FISH and DNA probes mapping to chromosome bands 1q21.2, 9q34, 13q14, 14q32, 17p13, and 22q11 were applied to 4 of the 15 cases. In one case, high-resolution genome-wide screening for genetic alterations using SNP-array analysis (Affymetrix Microarray Human Genome SNP 6.0) was performed on the CNS involvement as well as on the corresponding bone marrow (BM) aspirate.
Results
The median time from initial diagnosis until the occurrence of LMM was 463 days. Only two patients presented with CNS manifestation within 180 days after initial diagnosis. Seven patients were diagnosed at late stage of disease e.g. after high dose melphalan treatment. At diagnosis of LMM, the median age was 59 years. The median cell count in the cerebrospinal fluid was 21/µl (Range 1/µl -1333/µl). All CSF samples showed malignant pleocytosis, confirmed by flow cytometry in 12/15 patients. CIg-FISH presented cytogenetically defined high risk features in all samples tested: 3 of 4 patients showed a translocation t(4;14), one patient had a 17p13 deletion. Using high-resolution genome-wide screening assays revealed different subclones at the two clinical sites (CSF, BM). Treatment for LMM consisted of intrathecal chemotherapy (9 of 15 cases) and radiation therapy (7 of 15 cases). Despite treatment, the outcome of patients with confirmed LMM was dismal with a median overall survival after diagnosis of LMM of 69 days. Only one patient survived longer than 2 years after diagnosis of CNS involvement.
Conclusion
By combining several technical procedures (MRI, cytology, flow cytometry, cIg-FISH and SNP-array analysis) it is possible to identify the vast majority of patients with LMM. However, management of affected patients remains challenging and the survival is generally short after diagnosis of LMM.
Session topic: E-poster
Type: Publication Only
Background
Involvement of the central nervous (CNS) system in patients with Multiple Myeloma (MM) is a rare event, occuring mostly during late stage of disease. However, there are reports about an increasing incidence of extramedullary myeloma manifestations since the introduction of so called novel agents.
Aims
We therefore tried to optimize the diagnostic workup in patients with suspected neoplastic meningitis of Multiple Myeloma - so called leptomenigeal myelomatosis (LMM) – by incorporating different techniques.
Methods
Between 04/2005 and 10/2013 we identified 15 cases with CNS-MM. The involvement was confirmed by magnetic resonance imaging (MRI), cerebrospinal fluid cytology as well as by flow cytometry. Additionally, cIg-FISH and DNA probes mapping to chromosome bands 1q21.2, 9q34, 13q14, 14q32, 17p13, and 22q11 were applied to 4 of the 15 cases. In one case, high-resolution genome-wide screening for genetic alterations using SNP-array analysis (Affymetrix Microarray Human Genome SNP 6.0) was performed on the CNS involvement as well as on the corresponding bone marrow (BM) aspirate.
Results
The median time from initial diagnosis until the occurrence of LMM was 463 days. Only two patients presented with CNS manifestation within 180 days after initial diagnosis. Seven patients were diagnosed at late stage of disease e.g. after high dose melphalan treatment. At diagnosis of LMM, the median age was 59 years. The median cell count in the cerebrospinal fluid was 21/µl (Range 1/µl -1333/µl). All CSF samples showed malignant pleocytosis, confirmed by flow cytometry in 12/15 patients. CIg-FISH presented cytogenetically defined high risk features in all samples tested: 3 of 4 patients showed a translocation t(4;14), one patient had a 17p13 deletion. Using high-resolution genome-wide screening assays revealed different subclones at the two clinical sites (CSF, BM). Treatment for LMM consisted of intrathecal chemotherapy (9 of 15 cases) and radiation therapy (7 of 15 cases). Despite treatment, the outcome of patients with confirmed LMM was dismal with a median overall survival after diagnosis of LMM of 69 days. Only one patient survived longer than 2 years after diagnosis of CNS involvement.
Conclusion
By combining several technical procedures (MRI, cytology, flow cytometry, cIg-FISH and SNP-array analysis) it is possible to identify the vast majority of patients with LMM. However, management of affected patients remains challenging and the survival is generally short after diagnosis of LMM.
Session topic: E-poster
Abstract: PB1977
Type: Publication Only
Background
Involvement of the central nervous (CNS) system in patients with Multiple Myeloma (MM) is a rare event, occuring mostly during late stage of disease. However, there are reports about an increasing incidence of extramedullary myeloma manifestations since the introduction of so called novel agents.
Aims
We therefore tried to optimize the diagnostic workup in patients with suspected neoplastic meningitis of Multiple Myeloma - so called leptomenigeal myelomatosis (LMM) – by incorporating different techniques.
Methods
Between 04/2005 and 10/2013 we identified 15 cases with CNS-MM. The involvement was confirmed by magnetic resonance imaging (MRI), cerebrospinal fluid cytology as well as by flow cytometry. Additionally, cIg-FISH and DNA probes mapping to chromosome bands 1q21.2, 9q34, 13q14, 14q32, 17p13, and 22q11 were applied to 4 of the 15 cases. In one case, high-resolution genome-wide screening for genetic alterations using SNP-array analysis (Affymetrix Microarray Human Genome SNP 6.0) was performed on the CNS involvement as well as on the corresponding bone marrow (BM) aspirate.
Results
The median time from initial diagnosis until the occurrence of LMM was 463 days. Only two patients presented with CNS manifestation within 180 days after initial diagnosis. Seven patients were diagnosed at late stage of disease e.g. after high dose melphalan treatment. At diagnosis of LMM, the median age was 59 years. The median cell count in the cerebrospinal fluid was 21/µl (Range 1/µl -1333/µl). All CSF samples showed malignant pleocytosis, confirmed by flow cytometry in 12/15 patients. CIg-FISH presented cytogenetically defined high risk features in all samples tested: 3 of 4 patients showed a translocation t(4;14), one patient had a 17p13 deletion. Using high-resolution genome-wide screening assays revealed different subclones at the two clinical sites (CSF, BM). Treatment for LMM consisted of intrathecal chemotherapy (9 of 15 cases) and radiation therapy (7 of 15 cases). Despite treatment, the outcome of patients with confirmed LMM was dismal with a median overall survival after diagnosis of LMM of 69 days. Only one patient survived longer than 2 years after diagnosis of CNS involvement.
Conclusion
By combining several technical procedures (MRI, cytology, flow cytometry, cIg-FISH and SNP-array analysis) it is possible to identify the vast majority of patients with LMM. However, management of affected patients remains challenging and the survival is generally short after diagnosis of LMM.
Session topic: E-poster
Type: Publication Only
Background
Involvement of the central nervous (CNS) system in patients with Multiple Myeloma (MM) is a rare event, occuring mostly during late stage of disease. However, there are reports about an increasing incidence of extramedullary myeloma manifestations since the introduction of so called novel agents.
Aims
We therefore tried to optimize the diagnostic workup in patients with suspected neoplastic meningitis of Multiple Myeloma - so called leptomenigeal myelomatosis (LMM) – by incorporating different techniques.
Methods
Between 04/2005 and 10/2013 we identified 15 cases with CNS-MM. The involvement was confirmed by magnetic resonance imaging (MRI), cerebrospinal fluid cytology as well as by flow cytometry. Additionally, cIg-FISH and DNA probes mapping to chromosome bands 1q21.2, 9q34, 13q14, 14q32, 17p13, and 22q11 were applied to 4 of the 15 cases. In one case, high-resolution genome-wide screening for genetic alterations using SNP-array analysis (Affymetrix Microarray Human Genome SNP 6.0) was performed on the CNS involvement as well as on the corresponding bone marrow (BM) aspirate.
Results
The median time from initial diagnosis until the occurrence of LMM was 463 days. Only two patients presented with CNS manifestation within 180 days after initial diagnosis. Seven patients were diagnosed at late stage of disease e.g. after high dose melphalan treatment. At diagnosis of LMM, the median age was 59 years. The median cell count in the cerebrospinal fluid was 21/µl (Range 1/µl -1333/µl). All CSF samples showed malignant pleocytosis, confirmed by flow cytometry in 12/15 patients. CIg-FISH presented cytogenetically defined high risk features in all samples tested: 3 of 4 patients showed a translocation t(4;14), one patient had a 17p13 deletion. Using high-resolution genome-wide screening assays revealed different subclones at the two clinical sites (CSF, BM). Treatment for LMM consisted of intrathecal chemotherapy (9 of 15 cases) and radiation therapy (7 of 15 cases). Despite treatment, the outcome of patients with confirmed LMM was dismal with a median overall survival after diagnosis of LMM of 69 days. Only one patient survived longer than 2 years after diagnosis of CNS involvement.
Conclusion
By combining several technical procedures (MRI, cytology, flow cytometry, cIg-FISH and SNP-array analysis) it is possible to identify the vast majority of patients with LMM. However, management of affected patients remains challenging and the survival is generally short after diagnosis of LMM.
Session topic: E-poster
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