TY - JOUR
T1 - Familial chronic lymphocytic leukemia
T2 - what does it mean to me?
AU - Slager, Susan L.
AU - Kay, Neil E.
N1 - Funding Information:
This project was funded in part by a grant from NIH-NCI CA 118444 (SLS) and philanthropic support from the Donner Family Foundation and Mr. Edson Spencer.
PY - 2009
Y1 - 2009
N2 - Though B-chronic lymphocytic leukemia (CLL) is known to be a heterogeneous disease, only recently has the familial component of CLL been more thoroughly investigated. This entity is seen in approximately 5%-10% of all patients with CLL and can be associated with earlier age of diagnosis, higher female prevalence, and increased incidence of other lymphoproliferative disorders (LPDs), such as non-Hodgkin lymphoma and the more recently described monoclonal B-cell lymphocytosis CLL in family members. The prognostic parameters and clinical course of familial CLL is not clearly distinguishable from that of sporadic disease. In addition, it is not clear that the treatment responses for progressive disease has any discernible difference in familial versus sporadic CLL. The genetic etiology of CLL is unknown, and early work on familial CLL has not yet uncovered any obvious gene or group of genes that can be clearly related to the pathophysiology of CLL. However, the detailed genetic study of familial CLL is likely to be critical in uncovering relevant genes. At present it is best to indicate to concerned CLL patients that their relatives are at relatively low risk of developing CLL or other LPDs.
AB - Though B-chronic lymphocytic leukemia (CLL) is known to be a heterogeneous disease, only recently has the familial component of CLL been more thoroughly investigated. This entity is seen in approximately 5%-10% of all patients with CLL and can be associated with earlier age of diagnosis, higher female prevalence, and increased incidence of other lymphoproliferative disorders (LPDs), such as non-Hodgkin lymphoma and the more recently described monoclonal B-cell lymphocytosis CLL in family members. The prognostic parameters and clinical course of familial CLL is not clearly distinguishable from that of sporadic disease. In addition, it is not clear that the treatment responses for progressive disease has any discernible difference in familial versus sporadic CLL. The genetic etiology of CLL is unknown, and early work on familial CLL has not yet uncovered any obvious gene or group of genes that can be clearly related to the pathophysiology of CLL. However, the detailed genetic study of familial CLL is likely to be critical in uncovering relevant genes. At present it is best to indicate to concerned CLL patients that their relatives are at relatively low risk of developing CLL or other LPDs.
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U2 - 10.3816/clm.2009.s.011
DO - 10.3816/clm.2009.s.011
M3 - Review article
C2 - 19778840
AN - SCOPUS:74049156895
SN - 2152-2669
VL - 9 Suppl 3
SP - S194-197
JO - Clinical lymphoma & myeloma
JF - Clinical lymphoma & myeloma
ER -