B-cell prolymphocytic leukemia (B-PLL)

 B-cell prolymphocytic leukemia (B-PLL) is a rare lymphoproliferative disorder characterized by the out of control growth of B-cells (B-lymphocytes), primarily involving blood, bone marrow, and spleen. Prolymphocytes are medium-sized cells with variable amount of light basophilic cytoplasm, round, oval or indented nucleus, moderately condensed chromatin, and a prominent nucleolus. B-PLL usually affects older adults and it is slightly more common in men than women. B-PLL occurs as a transformation or evolution of a more slow-growing B-cell cancer, such as chronic lymphocytic leukemia. Rarely, this is a primary disorder.

The genetic features causing B-PLL are largely unknown. B-PLL can involve deletions from chromosome 11 and chromosome 13. Many cases have been reported with breakpoints involving 14q32, particularly with t(11;140(q13;q320, which is found in up to 20%v of cases of PLL. Mutations of p53 appear in the cells of 50% of PLL patients. The prognosis of B-PLL patients is quite poor

Immunophenotype:

Strongly express surface Immnoglobulins (kappa or lambda) and surface IgM as well as B cell markers CD19, CD20, CD22, CD79a, CD79b, FMC7. CD5 can be expressed and negative for CD23.

Reference:

  • Dearden C. How I treat prolymphocytic leukemia.Blood.2012;120(3):538-551.

  • Hercher C, Robain M, Davi F, et al; GroupeFranc ̧ais d’H ́ematologie Cellulaire. A multicentricstudy of 41 cases of B-prolymphocytic leukemia:two evolutive forms.Leuk Lymphoma.2001;42(5):981-987.

  • 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 JHaematol.2004;125(3):330-336.

  • Schlette E, Bueso-Ramos C, Giles F,Glassman A, Hayes K, Medeiros LJ. Mature B-cell leukemias with more than 55% prolymphocytes. A heterogeneous group that includes an unusual variant of mantle cell lymphoma. Am J Clin Pathol.2001;115(4):571-581.

  • Campo E, Catowsky D, Montserat E,Muller-Hermelink HK, Harris NL, Stein H. B-cellprolymphocytic leukeamia. In: Swerdlow SH,Campo E, Harris NL, et al, eds. WHOClassification of Tumours of Haematopietic andLymphoid Tissues,4th ed. Lyon: IARC Press;2008:183-184.

  • Vizcarra E, Martınez-Climent JA, Benet I, et al.Identification of two subgroups of mantle cell leukemia with distinct clinical and biologicalfeatures.Hematol J.2001;2(4):234-241.

  • Orchard J, Garand R, Davis Z, et al. A subsetof t(11;14) lymphoma with mantle cell featuresdisplays mutated IgVH genes and includespatients with good prognosis, nonnodal disease.Blood.2003;101(12):4975-4981.

  • Hoeller S, Zhou Y, Kanagal-Shamanna R, et al.Composite mantle cell lymphoma and chroniclymphocytic leukemia/small lymphocyticlymphoma: a clinicopathologic and molecularstudy.Hum Pathol.2013;44(1):110-121.

  • van Dongen JJ, Lhermitte L, B ̈ottcher S, et al;EuroFlow Consortium (EU-FP6, LSHB-CT-2006-018708). EuroFlow antibody panels for standardized n-dimensional flow cytometricimmunophenotyping of normal, reactive andmalignant leukocytes.Leukemia.2012;26(9):1908-1975.

  • Catowsky D, Montserat E, Muller-Hermelink HK,Harris NL. B-cell prolymphocytic leukeamia. In:Jaffe ES, Harris NL, Stein H, Vardiman JW, eds.World Health Organization Classification ofTumours Pathology and Genetics of Tumours ofHaematopoietic and Lymphoid Tissues.Lyon:IARC Press; 2001:131-132.

  • Berkowska MA, Driessen GJ, Bikos V, et al.Human memory B cells originate fromthree distinct germinal center-dependentand -independent maturation pathways.Blood.2011;118(8):2150-2158.

  • van Zelm MC, Szczepanski T, van der Burg M,van Dongen JJ. Replication history ofB lymphocytes reveals homeostatic proliferationand extensive antigen-induced B cell expansion.J Exp Med.2007;204(3):645-655.

Scholar Google Plagiarism Software

       Article invited for

          Vol 5,Issue 3,

                  2020

flowcytometry events

Copyright © 2015  www.flowcytometrynet.com. All right reserved
Follow Us
  • Facebook Basic Square
  • Google+ Basic Square