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)'.)
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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