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B-cell chronic lymphocytic leukemia (B-CLL)

B-cell chronic lymphocytic leukemia (CLL) is caused by the abnormal progressive accumulation of functionally incompetent monoclonal B-lymphocytes in blood, bone marrow, lymph nodes and spleen. It is the most common adult leukemia in Western countries, accounting for about 30% of total leukaemias. Worldwide there are approximately 180,000 new cases every year. A main focus in CLL research involved the evaluation of genetic features related to somatic gene mutation or deletions that disrupt apoptosis and enhance tumor cell proliferation. The best characterized genes are TP53 (also known as p53) and ATM, for which mutations and/or deletions have been described that predict rapid disease progression, resistance to conventional therapies and poor survival.

B-cell chronic lymphocytic leukemia (B-CLL), also known as chronic lymphoid leukemia (CLL), is the most common type of leukemia. Leukemias are cancers of the white blood cells (leukocytes). CLL affects B cell lymphocytes. B cells originate in the bone marrow, develop in the lymph nodes, and normally fight infection by producing antibodies. In CLL, the DNA of a B cell is damaged, so that it cannot produce antibodies. Additionally, B cells grow out of control and accumulate in the bone marrow and blood, where they crowd out healthy blood cells. CLL is a stage of small lymphocytic lymphoma (SLL), a type of B-cell lymphoma, which presents primarily in the lymph nodes. CLL and SLL are considered the same underlying disease, just with different appearances.

CLL is a disease of adults, but, in rare cases, it can occur in teenagers and occasionally in children (inherited). Most (>75%) people newly diagnosed with CLL are over the age of 50, and the majority are men.

Most people are diagnosed without symptoms as the result of a routine blood test that returns a high white blood cell count, but, as it advances, CLL results in swollen lymph nodes, spleen, and liver, and eventually anemia and infections. Early CLL is not treated, and late CLL is treated with chemotherapy and monoclonal antibodies.

KEGG: ds:H00005

USED DRUG(s):
Fludarabine [DR:D01907]
Chlorambucil [DR:D00266]
Prednisone [DR:D00473]
Rituximab [DR:D02994]
Cyclophosphamide [DR:D00287]
Alemtuzumab [DR:D02802]

ABREVIATION(S):
B-CLL
CLL
BCLL

TYPE: blood cell cancer

Related patway(s): DNA damage response (DDR)

DNAtraffic protein(s) related to disease: ATM

OMIM: none

Last modification date: Nov. 17, 2011