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B cell prolymphocytic leukemia

Arnold S Freedman, MD
Jon C Aster, MD
Claire Dearden, MD, BSc, FRCP, FRCPath
Section Editor
Andrew Lister, MD, FRCP, FRCPath, FRCR
Deputy Editor
Rebecca F Connor, MD


B cell prolymphocytic leukemia (B-PLL) is a very rare B cell neoplasm comprised of so-called prolymphocytes, typically with involvement of the peripheral blood, bone marrow, and spleen. The name "prolymphocyte" is actually a misnomer, as the tumor cells in this disease are mature activated B cells. By definition, these prolymphocytes comprise greater than 55 percent of the cells in the blood and bone marrow.

The epidemiology, clinical presentation, pathology, diagnosis, and treatment of B-PLL are discussed here.


B-PLL is an extremely rare disease, comprising far less than 1 percent of B cell leukemias [1]. Since the diagnosis was changed to exclude cases of mantle cell lymphoma, atypical chronic lymphocytic leukemia (CLL), and CLL/PLL (defined as between 15 and 55 percent prolymphocytes), B-PLL has become increasingly rare.

B-PLL mainly affects the elderly with a mean age at presentation of between 65 and 70 years [2]. Men and women appear to be equally affected [1]. The vast majority of patients are Caucasian.


Patients typically present with a rapidly rising white blood cell count >100,000/microL and massive splenomegaly; anemia and thrombocytopenia are present in approximately 65 and 35 percent, respectively [3,4]. Systemic B symptoms (ie, fevers, night sweats, weight loss) are common. If present, peripheral lymphadenopathy is not prominent. (See "Clinical presentation and diagnosis of non-Hodgkin lymphoma", section on 'Systemic complaints (B symptoms)'.)

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Literature review current through: Nov 2017. | This topic last updated: Oct 28, 2016.
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