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)'.)
- Yamamoto JF, Goodman MT. Patterns of leukemia incidence in the United States by subtype and demographic characteristics, 1997-2002. Cancer Causes Control 2008; 19:379.
- Melo JV, Catovsky D, Galton DA. The relationship between chronic lymphocytic leukaemia and prolymphocytic leukaemia. I. Clinical and laboratory features of 300 patients and characterization of an intermediate group. Br J Haematol 1986; 63:377.
- Ravandi F, O'Brien S. Chronic lymphoid leukemias other than chronic lymphocytic leukemia: diagnosis and treatment. Mayo Clin Proc 2005; 80:1660.
- Swerdlow SH, Campo E, Harris NL, et al. (Eds). World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, IARC Press, Lyon 2008.
- Catovsky D, Foa R. The Lymphoid Leukaemias, Butterworths, London 1990.
- Stone RM. Prolymphocytic leukemia. Hematol Oncol Clin North Am 1990; 4:457.
- Bearman RM, Pangalis GA, Rappaport H. Prolymphocytic leukemia: clinical, histopathological, and cytochemical observations. Cancer 1978; 42:2360.
- Galton DA, Goldman JM, Wiltshaw E, et al. Prolymphocytic leukaemia. Br J Haematol 1974; 27:7.
- Lampert I, Catovsky D, Marsh GW, et al. The histopathology of prolymphocytic leukaemia with particular reference to the spleen: a comparison with chronic lymphocytic leukaemia. Histopathology 1980; 4:3.
- Del Giudice I, Davis Z, Matutes E, et al. IgVH genes mutation and usage, ZAP-70 and CD38 expression provide new insights on B-cell prolymphocytic leukemia (B-PLL). Leukemia 2006; 20:1231.
- Flatley E, Chen AI, Zhao X, et al. Aberrations of MYC are a common event in B-cell prolymphocytic leukemia. Am J Clin Pathol 2014; 142:347.
- Lens D, De Schouwer PJ, Hamoudi RA, et al. p53 abnormalities in B-cell prolymphocytic leukemia. Blood 1997; 89:2015.
- Lens D, Coignet LJ, Brito-Babapulle V, et al. B cell prolymphocytic leukaemia (B-PLL) with complex karyotype and concurrent abnormalities of the p53 and c-MYC gene. Leukemia 1999; 13:873.
- Solé F, Woessner S, Espinet B, et al. Cytogenetic abnormalities in three patients with B-cell prolymphocytic leukemia. Cancer Genet Cytogenet 1998; 103:43.
- Ruchlemer R, Parry-Jones N, Brito-Babapulle V, et al. B-prolymphocytic leukaemia with t(11;14) revisited: a splenomegalic form of mantle cell lymphoma evolving with leukaemia. Br J Haematol 2004; 125:330.
- Del Giudice I, Osuji N, Dexter T, et al. B-cell prolymphocytic leukemia and chronic lymphocytic leukemia have distinctive gene expression signatures. Leukemia 2009; 23:2160.
- Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016; 127:2375.
- van der Velden VH, Hoogeveen PG, de Ridder D, et al. B-cell prolymphocytic leukemia: a specific subgroup of mantle cell lymphoma. Blood 2014; 124:412.
- Shvidel L, Shtalrid M, Bassous L, et al. B-cell prolymphocytic leukemia: a survey of 35 patients emphasizing heterogeneity, prognostic factors and evidence for a group with an indolent course. Leuk Lymphoma 1999; 33:169.
- Saven A, Lee T, Schlutz M, et al. Major activity of cladribine in patients with de novo B-cell prolymphocytic leukemia. J Clin Oncol 1997; 15:37.
- Doorduijn JK, Michiels JJ. Effectiveness of fludarabine in end-stage prolymphocytic leukemia. Leukemia 1994; 8:1439.
- Mourad YA, Taher A, Chehal A, Shamseddine A. Successful treatment of B-cell prolymphocytic leukemia with monoclonal anti-CD20 antibody. Ann Hematol 2004; 83:319.
- Tempescul A, Feuerbach J, Ianotto JC, et al. A combination therapy with fludarabine, mitoxantrone and rituximab induces complete immunophenotypical remission in B-cell prolymphocytic leukaemia. Ann Hematol 2009; 88:85.
- Chow KU, Kim SZ, von Neuhoff N, et al. Clinical efficacy of immunochemotherapy with fludarabine, epirubicin and rituximab in the treatment for chronic lymphocytic leukaemia and prolymphocytic leukaemia. Eur J Haematol 2011; 87:426.
- Weide R, Pandorf A, Heymanns J, Köppler H. Bendamustine/Mitoxantrone/Rituximab (BMR): a very effective, well tolerated outpatient chemoimmunotherapy for relapsed and refractory CD20-positive indolent malignancies. Final results of a pilot study. Leuk Lymphoma 2004; 45:2445.
- McCune SL, Gockerman JP, Moore JO, et al. Alemtuzumab in relapsed or refractory chronic lymphocytic leukemia and prolymphocytic leukemia. Leuk Lymphoma 2002; 43:1007.
- Chaar BT, Petruska PJ. Complete response to alemtuzumab in a patient with B prolymphocytic leukemia. Am J Hematol 2007; 82:417.
- Absi A, Hsi E, Kalaycio M. Prolymphocytic leukemia. Curr Treat Options Oncol 2005; 6:197.
- Oscier DG, Catovsky D, Errington RD, et al. Splenic irradiation in B-prolymphocytic leukaemia. Br J Haematol 1981; 48:577.
- Castagna L, Sarina B, Todisco E, et al. Allogeneic peripheral stem-cell transplantation with reduced-intensity conditioning regimen in refractory primary B-cell prolymphocytic leukemia: a long-term follow-up. Bone Marrow Transplant 2005; 35:1225.
- Hercher C, Robain M, Davi F, et al. A multicentric study of 41 cases of B-prolymphocytic leukemia: two evolutive forms. Leuk Lymphoma 2001; 42:981.
- 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