BRAF GENE MUTATIONS AND PROGNOSTIC SIGNIFICANCE OF CLINICAL FINDINGS IN CASTLEMAN DISEASE
(Abstract release date: 05/19/16)
EHA Library. Türker M. 06/09/16; 134781; PB1881
Dr. Mehmet Türker
Contributions
Contributions
Abstract
Abstract: PB1881
Type: Publication Only
Background
Castleman's Disease is a quite rare lymphoproliferative disorder. The association with IL-6 secretion, HIV / HHV-8 is well described, but the pathogenesis is not fully understood. BRAF is a serine/threonine kinase that has an important role in the mitogen activated protein kinase (MAPK) pathway. Dysregulation of this pathway and gene mutations lead to development of malignancy. BRAF mutations are present in malignant melanoma, colorectal cancer, ovarian carcinoma, papillary thyroid carcinoma and hairy cell leukemia.
Aims
We aimed to determine the presence of BRAF gene mutation in Castleman’s Disease and to assess clinical data of disease and the relationship with prognosis.
Methods
This retrospective study included 34 patients who were diagnosed as Castleman's Disease between 2007-2014 from 8 different medical centers in Turkey. Clinical and laboratory findings of the patients have been evaluated from medical records. All tissue samples were characterized for the presence of the BRAF mutation by pyrosequencing. Statistical analysis was performed to determine any associations among histological variants,clinical type and clinical features of the patients including age, gender, survival, and laboratory findings.
Results
There were 17 men and 17 women. Hyaline vascular variant was the most common subtype (75.6%). The study included 21 (61.8%) cases with unicentric Castleman’s Disease (UCD) and 13 (38.2%) cases with multicentric Castleman’s Disease (MCD). Hyaline vascular variant was more common in the UCD group (p<0.05). Cases were divided in 3 sites according to involvement; thoracic, abdominal and cervical. The majority of patients (61.7%) had cervical involvement. 10 of patients (29.4%) had more than one region involvement. The plasma cell variant was significantly higher in men (p=0.006). The mean age was significantly higher in the plasma cell group and MCD (p<0.05). CRP levels were found significantly higher in plasma cell variant compared to hyaline vascular variant (p<0.05). The BRAF gene mutation was not detected in all tissue samples. Hepatomegaly and/or splenomegaly were presented significantly common in patients with multiple site involvement (p<0.05). Splenomegaly was presented significantly common in MCD (p=0.001). 3-year overall survival was significantly higher in patients treated with only radiotherapy or chemotherapy in addition to radiotherapy when compared to only chemotherapy (p=0.001). Mean survival was significantly higher in patients with hyaline vascular variant and UCD (p <0.05).
Conclusion
This study is the most comprehensive study in our country about Castleman's Disease and we obtained important information about clinical, histopathological and prognostic features of the disease. We suggest combined therapy with radiotherapy is superior to chemotherapy alone, but as we know the efficiency of radiotherapy in the treatment of unicentric Castleman's Disease and we may have got this outcome due to some of the unicentric patients in our study were treated by radiotherapy alone.
Session topic: E-poster
Keyword(s): Castleman's disease, Herpesvirus, Mutation analysis
Type: Publication Only
Background
Castleman's Disease is a quite rare lymphoproliferative disorder. The association with IL-6 secretion, HIV / HHV-8 is well described, but the pathogenesis is not fully understood. BRAF is a serine/threonine kinase that has an important role in the mitogen activated protein kinase (MAPK) pathway. Dysregulation of this pathway and gene mutations lead to development of malignancy. BRAF mutations are present in malignant melanoma, colorectal cancer, ovarian carcinoma, papillary thyroid carcinoma and hairy cell leukemia.
Aims
We aimed to determine the presence of BRAF gene mutation in Castleman’s Disease and to assess clinical data of disease and the relationship with prognosis.
Methods
This retrospective study included 34 patients who were diagnosed as Castleman's Disease between 2007-2014 from 8 different medical centers in Turkey. Clinical and laboratory findings of the patients have been evaluated from medical records. All tissue samples were characterized for the presence of the BRAF mutation by pyrosequencing. Statistical analysis was performed to determine any associations among histological variants,clinical type and clinical features of the patients including age, gender, survival, and laboratory findings.
Results
There were 17 men and 17 women. Hyaline vascular variant was the most common subtype (75.6%). The study included 21 (61.8%) cases with unicentric Castleman’s Disease (UCD) and 13 (38.2%) cases with multicentric Castleman’s Disease (MCD). Hyaline vascular variant was more common in the UCD group (p<0.05). Cases were divided in 3 sites according to involvement; thoracic, abdominal and cervical. The majority of patients (61.7%) had cervical involvement. 10 of patients (29.4%) had more than one region involvement. The plasma cell variant was significantly higher in men (p=0.006). The mean age was significantly higher in the plasma cell group and MCD (p<0.05). CRP levels were found significantly higher in plasma cell variant compared to hyaline vascular variant (p<0.05). The BRAF gene mutation was not detected in all tissue samples. Hepatomegaly and/or splenomegaly were presented significantly common in patients with multiple site involvement (p<0.05). Splenomegaly was presented significantly common in MCD (p=0.001). 3-year overall survival was significantly higher in patients treated with only radiotherapy or chemotherapy in addition to radiotherapy when compared to only chemotherapy (p=0.001). Mean survival was significantly higher in patients with hyaline vascular variant and UCD (p <0.05).
Conclusion
This study is the most comprehensive study in our country about Castleman's Disease and we obtained important information about clinical, histopathological and prognostic features of the disease. We suggest combined therapy with radiotherapy is superior to chemotherapy alone, but as we know the efficiency of radiotherapy in the treatment of unicentric Castleman's Disease and we may have got this outcome due to some of the unicentric patients in our study were treated by radiotherapy alone.
Session topic: E-poster
Keyword(s): Castleman's disease, Herpesvirus, Mutation analysis
Abstract: PB1881
Type: Publication Only
Background
Castleman's Disease is a quite rare lymphoproliferative disorder. The association with IL-6 secretion, HIV / HHV-8 is well described, but the pathogenesis is not fully understood. BRAF is a serine/threonine kinase that has an important role in the mitogen activated protein kinase (MAPK) pathway. Dysregulation of this pathway and gene mutations lead to development of malignancy. BRAF mutations are present in malignant melanoma, colorectal cancer, ovarian carcinoma, papillary thyroid carcinoma and hairy cell leukemia.
Aims
We aimed to determine the presence of BRAF gene mutation in Castleman’s Disease and to assess clinical data of disease and the relationship with prognosis.
Methods
This retrospective study included 34 patients who were diagnosed as Castleman's Disease between 2007-2014 from 8 different medical centers in Turkey. Clinical and laboratory findings of the patients have been evaluated from medical records. All tissue samples were characterized for the presence of the BRAF mutation by pyrosequencing. Statistical analysis was performed to determine any associations among histological variants,clinical type and clinical features of the patients including age, gender, survival, and laboratory findings.
Results
There were 17 men and 17 women. Hyaline vascular variant was the most common subtype (75.6%). The study included 21 (61.8%) cases with unicentric Castleman’s Disease (UCD) and 13 (38.2%) cases with multicentric Castleman’s Disease (MCD). Hyaline vascular variant was more common in the UCD group (p<0.05). Cases were divided in 3 sites according to involvement; thoracic, abdominal and cervical. The majority of patients (61.7%) had cervical involvement. 10 of patients (29.4%) had more than one region involvement. The plasma cell variant was significantly higher in men (p=0.006). The mean age was significantly higher in the plasma cell group and MCD (p<0.05). CRP levels were found significantly higher in plasma cell variant compared to hyaline vascular variant (p<0.05). The BRAF gene mutation was not detected in all tissue samples. Hepatomegaly and/or splenomegaly were presented significantly common in patients with multiple site involvement (p<0.05). Splenomegaly was presented significantly common in MCD (p=0.001). 3-year overall survival was significantly higher in patients treated with only radiotherapy or chemotherapy in addition to radiotherapy when compared to only chemotherapy (p=0.001). Mean survival was significantly higher in patients with hyaline vascular variant and UCD (p <0.05).
Conclusion
This study is the most comprehensive study in our country about Castleman's Disease and we obtained important information about clinical, histopathological and prognostic features of the disease. We suggest combined therapy with radiotherapy is superior to chemotherapy alone, but as we know the efficiency of radiotherapy in the treatment of unicentric Castleman's Disease and we may have got this outcome due to some of the unicentric patients in our study were treated by radiotherapy alone.
Session topic: E-poster
Keyword(s): Castleman's disease, Herpesvirus, Mutation analysis
Type: Publication Only
Background
Castleman's Disease is a quite rare lymphoproliferative disorder. The association with IL-6 secretion, HIV / HHV-8 is well described, but the pathogenesis is not fully understood. BRAF is a serine/threonine kinase that has an important role in the mitogen activated protein kinase (MAPK) pathway. Dysregulation of this pathway and gene mutations lead to development of malignancy. BRAF mutations are present in malignant melanoma, colorectal cancer, ovarian carcinoma, papillary thyroid carcinoma and hairy cell leukemia.
Aims
We aimed to determine the presence of BRAF gene mutation in Castleman’s Disease and to assess clinical data of disease and the relationship with prognosis.
Methods
This retrospective study included 34 patients who were diagnosed as Castleman's Disease between 2007-2014 from 8 different medical centers in Turkey. Clinical and laboratory findings of the patients have been evaluated from medical records. All tissue samples were characterized for the presence of the BRAF mutation by pyrosequencing. Statistical analysis was performed to determine any associations among histological variants,clinical type and clinical features of the patients including age, gender, survival, and laboratory findings.
Results
There were 17 men and 17 women. Hyaline vascular variant was the most common subtype (75.6%). The study included 21 (61.8%) cases with unicentric Castleman’s Disease (UCD) and 13 (38.2%) cases with multicentric Castleman’s Disease (MCD). Hyaline vascular variant was more common in the UCD group (p<0.05). Cases were divided in 3 sites according to involvement; thoracic, abdominal and cervical. The majority of patients (61.7%) had cervical involvement. 10 of patients (29.4%) had more than one region involvement. The plasma cell variant was significantly higher in men (p=0.006). The mean age was significantly higher in the plasma cell group and MCD (p<0.05). CRP levels were found significantly higher in plasma cell variant compared to hyaline vascular variant (p<0.05). The BRAF gene mutation was not detected in all tissue samples. Hepatomegaly and/or splenomegaly were presented significantly common in patients with multiple site involvement (p<0.05). Splenomegaly was presented significantly common in MCD (p=0.001). 3-year overall survival was significantly higher in patients treated with only radiotherapy or chemotherapy in addition to radiotherapy when compared to only chemotherapy (p=0.001). Mean survival was significantly higher in patients with hyaline vascular variant and UCD (p <0.05).
Conclusion
This study is the most comprehensive study in our country about Castleman's Disease and we obtained important information about clinical, histopathological and prognostic features of the disease. We suggest combined therapy with radiotherapy is superior to chemotherapy alone, but as we know the efficiency of radiotherapy in the treatment of unicentric Castleman's Disease and we may have got this outcome due to some of the unicentric patients in our study were treated by radiotherapy alone.
Session topic: E-poster
Keyword(s): Castleman's disease, Herpesvirus, Mutation analysis
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