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Clinical presentation, pathologic features, diagnosis, and differential diagnosis of chronic lymphocytic leukemia

Kanti R Rai, MD
Stephan Stilgenbauer, MD
Section Editor
Richard A Larson, MD
Deputy Editor
Rebecca F Connor, MD


Chronic lymphocytic leukemia (CLL) is one of the chronic lymphoproliferative disorders (lymphoid neoplasms). It is characterized by a progressive accumulation of functionally incompetent lymphocytes, which are usually monoclonal in origin.

CLL is considered to be identical (ie, one disease with different manifestations) to the mature (peripheral) B cell neoplasm small lymphocytic lymphoma (SLL), one of the indolent non-Hodgkin lymphomas. (See "Clinical manifestations, pathologic features, and diagnosis of small lymphocytic lymphoma".)

The epidemiology, clinical presentation, pathologic features, diagnosis, and differential diagnosis of CLL will be reviewed here. The pathophysiology, molecular biology, cytogenetic abnormalities, and treatment of CLL are discussed elsewhere. (See "Pathophysiology and genetic features of chronic lymphocytic leukemia" and "Overview of the treatment of chronic lymphocytic leukemia".)


CLL is the most common leukemia in adults in Western countries, accounting for approximately 25 to 30 percent of all leukemias in the United States [1]. The disorder is more common in men, with a male to female ratio of approximately 1.3:1 to 1.7:1 [1,2]. The incidence rates among men and women in the United States are approximately 6.75 and 3.65 cases per 100,000 population per year, respectively [3]. In Europe, these incidence rates are 5.87 and 4.01 cases per 100,000 population per year, respectively [4]. Worldwide, there are approximately 191,000 cases and 61,000 deaths per year attributed to CLL [5].

CLL is considered to be mainly a disease of older adults, with a median age at diagnosis of approximately 70 years [6]; however, it is not unusual to make this diagnosis in younger individuals from 30 to 39 years of age [2]. The incidence increases rapidly with increasing age. It is estimated that 20,110 new cases of CLL will be diagnosed in the United States in 2017: 12,310 in males and 7,800 in females [1].


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