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Comprehensive DNA Methylation Analysis Using a Methyl-CpG-binding Domain Capture-based Method in Chronic Lymphocytic Leukemia Patients
Published on: June 16, 2017
Familial occurrence of chronic lymphocytic leukaemia in Norway
Geir E Tjønnfjord1, Viggo Jønsson, Bernt E Ly
1Department of Haematology, Oslo University Hospital, Norway. geir.tjonnfjord@oslo-universitetssykehus.no
Insights
Malignant hematological diseases, including chronic lymphocytic leukemia (CLL), are common in relatives of CLL patients. Familial occurrence significantly increases the risk of developing CLL and other lymphoproliferative diseases.
Area of Science:
- Hematology
- Oncology
- Genetics
Background:
- Chronic lymphocytic leukemia (CLL) is a cancer of the blood and bone marrow.
- The only established risk factor for CLL is a family history of the disease.
Purpose of the Study:
- To investigate the frequency of familial chronic lymphocytic leukemia.
- To quantify the risk of developing CLL among relatives of affected individuals.
Main Methods:
- Patients diagnosed with CLL between October 2007 and December 2009 were surveyed.
- Family members reported occurrences of malignant hematological diseases.
- Data were verified using the Cancer Registry.
Main Results:
- 18% of CLL patients (42 of 236) had relatives with malignant hematological disease.
- The relative risk of developing CLL was six times higher for individuals with a family history.
- Other lymphoproliferative diseases were also more common in affected families.
Conclusions:
- Malignant hematological diseases are frequently observed in families of CLL patients.
- CLL is the most common familial malignancy, but other related diseases also occur.
- Evidence suggests a potential genetic predisposition and pleiotropy in familial CLL.
Background:
The only known risk factor for chronic lymphocytic leukaemia (CLL) is occurrence of the disease in close relatives. The aim of this study was to determine the frequency of familial chronic lymphocytic leukaemia.
Material And Method:
All patients with chronic lymphocytic leukaemia notified to the Cancer Registry in the period 1.10.2007-31.12.2009 were asked to report occurrences of malignant disease in siblings, parents, grandparents and children. The information about malignant haematological disease was verified with the Cancer Registry.
Results:
We found malignant haematological disease in close relatives of 42 of the 236 included patients (18%). CLL and lymphoma were the most common diagnoses. On average, 16 family members were identified in each family. The relative risk of developing CLL was six times higher in those who had close relatives with the disease (16 of a total of 3,776 family members) than among those who did not have close relatives who were affected (76 cases among 107,223 family members of 38,159 control subjects). The increased risk of disease was also associated with other lymphoproliferative diseases. With patrilinear, but not matrilinear inheritance, we found a birth order effect, with affection of younger men in a group of siblings, while the eldest escaped.
Interpretation:
Malignant haematological disease is common in the family members of patients with CLL. CLL is the most common disease, but there is extensive pleiotropy.
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