B cell prolymphocytic leukemia
- Arnold S Freedman, MD
Arnold S Freedman, MD
- Section Editor — Lymphoproliferative Disorders
- Professor of Medicine
- Harvard Medical School
- Jon C Aster, MD
Jon C Aster, MD
- Professor of Pathology
- Harvard Medical School
- Claire Dearden, MD, BSc, FRCP, FRCPath
Claire Dearden, MD, BSc, FRCP, FRCPath
- Consultant Haematologist
- The Royal Marsden Hospital and Institute of Cancer Research, London
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 . 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.
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)'.)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- CLINICAL FEATURES
- - Peripheral blood and bone marrow
- - Other tissues
- Genetic features
- DIFFERENTIAL DIAGNOSIS
- T cell prolymphocytic leukemia
- Chronic lymphocytic leukemia
- Mantle cell lymphoma
- Follicular lymphoma
- Lymphoplasmacytic lymphoma
- Hairy cell leukemia
- Splenic marginal zone lymphoma