Leukemia, Chronic Lymphocytic

Alternative Names

  • CLL
  • Leukemia, Chronic Lymphatic
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WHO-ICD-10 version:2010

Neoplasms

Malignant neoplasms

OMIM Number

151400

Mode of Inheritance

Autosomal dominant; Somatic mutation

Gene Map Locus

6p25.3,9q34,11p11, 11q24.1,13q14,17p13

Description

Chronic lymphocytic leukemia (CLL) is a malignant disorder affecting the lymphocytes. The cells affected are usually the clonal B lymphocytes, which are arrested in an intermediate stage between pre-B cells and mature B-cells. The use of the term 'chronic' in the name refers to the typical slow progression of the cancer. Unlike the acute form of lymphocytic leukemia, CLL usually affects older adults. Most affected individuals do not show any symptoms in the early stages of the disease. In many cases, the leukemia is diagnosed incidentally. Among those who do develop some symptoms of the condition, the most common presenting sign is painless enlargement of the lymph nodes. Other symptoms of the condition include fatigue, fever, upper abdominal pain resulting from splenomegaly, night sweats, weight loss, and recurrent infections. Certain risk factors may predispose an individual to developing CLL. These include having an age over 60 years, being a white male, having a family history of the condition, and exposure to certain carcinogens.

Since CLL is symptom-free for much of its progression, early diagnosis is often missed. In any case, early-stage CLL, even if diagnosed, is usually not subjected to any form of treatment. Doctors prefer to adopt a 'wait-and-watch' policy to monitor the patient's condition and start treatment if the leukemia begins to progress. As in other forms of leukemia, treatment strategies consist of chemotherapy, targeted drug therapy, and bone marrow transplant. Prognosis depends on the stage of the disease at diagnosis, as well as the presence or absence of high-risk markers. In most patients, the leukemia follows an initial phase of up to a decade of a benign course, followed by a progressive and terminal phase of 1-2 years. The latter phase is associated with high morbidity and drug resistance.

CLL, more often than not, is an acquired disorder. Familial cases of CLL are extremely rare. However, most affected patients show an abnormal karyotype upon examination. Abnormalities in or deletion of chromosome 13q is one of the most common karyotypic defects associated with CLL. Fortunately, this is associated with a stable or slowly progressive form. On the other hand, trisomy 13 and 17p13 deletions are both associated with progressive disease and decreased survival. 11q22-q23 deletions are seen in cases with extensive lymph node involvement and an aggressive disease. Other studies have noted that B-cell CLL is associated with over-expression of the proto-oncogene Bcl-2.

Epidemiology in the Arab World

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Other Reports

Kuwait

Al-Bahar et al. (1994) studied the occurrence of leukemia subtypes in Kuwait between 1979 and 1989. Of the total 723 cases, CLL was found in 8.6%. This rate is lesser than that reported in Western countries, and similar to other Oriental populations.

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