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B-cell prolymphocytic leukemia

MedGen UID:
105396
Concept ID:
C0475801
Neoplastic Process
Synonyms: B Cell Prolymphocytic Leukemia; B-Cell Prolymphocytic Leukemia; B-Cell Prolymphocytic Leukemias; Leukemia, B-Cell Prolymphocytic; Leukemia, Prolymphocytic, B-Cell; Leukemias, B-Cell Prolymphocytic; Prolymphocytic Leukemia, B-Cell; Prolymphocytic Leukemias, B-Cell
SNOMED CT: Prolymphocytic leukemia, B-cell type (128820007); Prolymphocytic leukemia of B-cell type (277619001); B-cell prolymphocytic leukemia (277619001)
 
Monarch Initiative: MONDO:0019461
Orphanet: ORPHA86852

Definition

A rare mature B-cell neoplasm characterized by clonal proliferation of B-cell prolymphocytes, with prolymphocytes constituting more than 55% of lymphoid cells in peripheral blood. IG genes are clonally rearranged. Neoplastic cells are present in the bone marrow, peripheral blood, and spleen. Patients usually present with B symptoms, massive splenomegaly but absent or minimal lymphadenopathy, rapidly increasing lymphocyte count, anemia, and thrombocytopenia. Therapy response is poor. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVB-cell prolymphocytic leukemia
Follow this link to review classifications for B-cell prolymphocytic leukemia in Orphanet.

Professional guidelines

PubMed

Moore J, Baran AM, Meacham PJ, Evans AG, Barr PM, Zent CS
Am J Hematol 2020 May;95(5):E108-E110. Epub 2020 Jan 31 doi: 10.1002/ajh.25733. PMID: 31951033
Collignon A, Wanquet A, Maitre E, Cornet E, Troussard X, Aurran-Schleinitz T
Curr Oncol Rep 2017 Apr;19(4):29. doi: 10.1007/s11912-017-0581-x. PMID: 28324286
Robak T
Cancer Treat Rev 2011 Feb;37(1):3-10. Epub 2010 Jun 16 doi: 10.1016/j.ctrv.2010.05.003. PMID: 20558005

Recent clinical studies

Etiology

Chen LY, Eyre TA
Eur J Haematol 2022 Nov;109(5):590-592. Epub 2022 Aug 5 doi: 10.1111/ejh.13837. PMID: 35871485
Chapiro E, Pramil E, Diop M, Roos-Weil D, Dillard C, Gabillaud C, Maloum K, Settegrana C, Baseggio L, Lesesve JF, Yon M, Jondreville L, Lesty C, Davi F, Le Garff-Tavernier M, Droin N, Dessen P, Algrin C, Leblond V, Gabarre J, Bouzy S, Eclache V, Gaillard B, Callet-Bauchu E, Muller M, Lefebvre C, Nadal N, Ittel A, Struski S, Collonge-Rame MA, Quilichini B, Fert-Ferrer S, Auger N, Radford-Weiss I, Wagner L, Scheinost S, Zenz T, Susin SA, Bernard OA, Nguyen-Khac F; the Groupe Francophone de Cytogénétique Hématologique (GFCH); the French Innovative Leukemia Organization (FILO)
Blood 2019 Nov 21;134(21):1821-1831. doi: 10.1182/blood.2019001187. PMID: 31527074
Flatley E, Chen AI, Zhao X, Jaffe ES, Dunlap JB, Pittaluga S, Abdullah S, Olson SB, Spurgeon SE, Fan G
Am J Clin Pathol 2014 Sep;142(3):347-54. doi: 10.1309/AJCPUBHM8U7ZFLOB. PMID: 25125625
Isobe Y, Tomomatsu J, Tsukune Y, Tsukada N, Sasaki M, Sugimoto K, Komatsu N
Intern Med 2012;51(15):1977-81. Epub 2012 Aug 1 doi: 10.2169/internalmedicine.51.7579. PMID: 22864121
Homminga I, Zwaan CM, Manz CY, Parker C, Bantia S, Smits WK, Higginbotham F, Pieters R, Meijerink JP
Blood 2011 Aug 25;118(8):2184-90. Epub 2011 Jul 5 doi: 10.1182/blood-2011-02-337840. PMID: 21730354

Diagnosis

Gonzalez-Mancera MS, Lopategui J, Hoffman D, Kitahara S, Alkan S
Int J Hematol 2024 Aug;120(2):252-255. Epub 2024 May 26 doi: 10.1007/s12185-024-03774-4. PMID: 38796826
El Hussein S, Khoury JD, Medeiros LJ
Ann Diagn Pathol 2021 Oct;54:151790. Epub 2021 Jul 16 doi: 10.1016/j.anndiagpath.2021.151790. PMID: 34293709
Chapiro E, Pramil E, Diop M, Roos-Weil D, Dillard C, Gabillaud C, Maloum K, Settegrana C, Baseggio L, Lesesve JF, Yon M, Jondreville L, Lesty C, Davi F, Le Garff-Tavernier M, Droin N, Dessen P, Algrin C, Leblond V, Gabarre J, Bouzy S, Eclache V, Gaillard B, Callet-Bauchu E, Muller M, Lefebvre C, Nadal N, Ittel A, Struski S, Collonge-Rame MA, Quilichini B, Fert-Ferrer S, Auger N, Radford-Weiss I, Wagner L, Scheinost S, Zenz T, Susin SA, Bernard OA, Nguyen-Khac F; the Groupe Francophone de Cytogénétique Hématologique (GFCH); the French Innovative Leukemia Organization (FILO)
Blood 2019 Nov 21;134(21):1821-1831. doi: 10.1182/blood.2019001187. PMID: 31527074
Collignon A, Wanquet A, Maitre E, Cornet E, Troussard X, Aurran-Schleinitz T
Curr Oncol Rep 2017 Apr;19(4):29. doi: 10.1007/s11912-017-0581-x. PMID: 28324286
Quest GR, Johnston JB
Best Pract Res Clin Haematol 2015 Dec;28(4):180-92. Epub 2015 Oct 26 doi: 10.1016/j.beha.2015.10.017. PMID: 26614896

Therapy

Cross M, Dearden C
Best Pract Res Clin Haematol 2019 Sep;32(3):217-228. Epub 2019 Jun 6 doi: 10.1016/j.beha.2019.06.001. PMID: 31585622
Collignon A, Wanquet A, Maitre E, Cornet E, Troussard X, Aurran-Schleinitz T
Curr Oncol Rep 2017 Apr;19(4):29. doi: 10.1007/s11912-017-0581-x. PMID: 28324286
Nachat A, Turoff-Ortmeyer S, Liu C, Mcculloch M
Perm J 2016 Spring;20(2):74-80. doi: 10.7812/TPP/15-153. PMID: 27168399Free PMC Article
Quest GR, Johnston JB
Best Pract Res Clin Haematol 2015 Dec;28(4):180-92. Epub 2015 Oct 26 doi: 10.1016/j.beha.2015.10.017. PMID: 26614896
Dearden C
Hematology Am Soc Hematol Educ Program 2012;2012:645-51. doi: 10.1182/asheducation-2012.1.645. PMID: 23233647

Prognosis

Czader M, Amador C, Cook JR, Thakkar D, Parker C, Dave SS, Dogan A, Duffield AS, Nejati R, Ott G, Xiao W, Wasik M, Goodlad JR
Am J Clin Pathol 2023 Jun 1;159(6):554-571. doi: 10.1093/ajcp/aqad027. PMID: 37052539Free PMC Article
Cross M, Dearden C
Best Pract Res Clin Haematol 2019 Sep;32(3):217-228. Epub 2019 Jun 6 doi: 10.1016/j.beha.2019.06.001. PMID: 31585622
Chapiro E, Pramil E, Diop M, Roos-Weil D, Dillard C, Gabillaud C, Maloum K, Settegrana C, Baseggio L, Lesesve JF, Yon M, Jondreville L, Lesty C, Davi F, Le Garff-Tavernier M, Droin N, Dessen P, Algrin C, Leblond V, Gabarre J, Bouzy S, Eclache V, Gaillard B, Callet-Bauchu E, Muller M, Lefebvre C, Nadal N, Ittel A, Struski S, Collonge-Rame MA, Quilichini B, Fert-Ferrer S, Auger N, Radford-Weiss I, Wagner L, Scheinost S, Zenz T, Susin SA, Bernard OA, Nguyen-Khac F; the Groupe Francophone de Cytogénétique Hématologique (GFCH); the French Innovative Leukemia Organization (FILO)
Blood 2019 Nov 21;134(21):1821-1831. doi: 10.1182/blood.2019001187. PMID: 31527074
Collignon A, Wanquet A, Maitre E, Cornet E, Troussard X, Aurran-Schleinitz T
Curr Oncol Rep 2017 Apr;19(4):29. doi: 10.1007/s11912-017-0581-x. PMID: 28324286
Quest GR, Johnston JB
Best Pract Res Clin Haematol 2015 Dec;28(4):180-92. Epub 2015 Oct 26 doi: 10.1016/j.beha.2015.10.017. PMID: 26614896

Clinical prediction guides

Czader M, Amador C, Cook JR, Thakkar D, Parker C, Dave SS, Dogan A, Duffield AS, Nejati R, Ott G, Xiao W, Wasik M, Goodlad JR
Am J Clin Pathol 2023 Jun 1;159(6):554-571. doi: 10.1093/ajcp/aqad027. PMID: 37052539Free PMC Article
Bhoi S, Ljungström V, Baliakas P, Mattsson M, Smedby KE, Juliusson G, Rosenquist R, Mansouri L
Epigenetics 2016 Jun 2;11(6):449-55. Epub 2016 Apr 29 doi: 10.1080/15592294.2016.1178432. PMID: 27128508Free PMC Article
Qiufan Z, Shumei X, Xifeng D, Shuwen D, Huaquan W, Zonghong S
Clin Lab 2015;61(7):831-4. doi: 10.7754/clin.lab.2014.141140. PMID: 26299084
Fiegl M, Stauder R, Steurer M, Mian M, Hopfinger G, Brychtova Y, Skrabs C, Zabernigg A, Schmid F, Haslbaur F, Winder G, Walder A, Lang A, Voskova D, Greil R, Mayer J, Gastl G; Austrian Collaborative Study Group on Alemtuzumab in Chronic Lymphocytic Leukemia, in cooperation with The Czech Leukemia Study Group for Life, CELL
Ann Hematol 2014 Feb;93(2):267-77. Epub 2013 Nov 30 doi: 10.1007/s00277-013-1966-z. PMID: 24292560
Isobe Y, Tomomatsu J, Tsukune Y, Tsukada N, Sasaki M, Sugimoto K, Komatsu N
Intern Med 2012;51(15):1977-81. Epub 2012 Aug 1 doi: 10.2169/internalmedicine.51.7579. PMID: 22864121

Recent systematic reviews

Nachat A, Turoff-Ortmeyer S, Liu C, Mcculloch M
Perm J 2016 Spring;20(2):74-80. doi: 10.7812/TPP/15-153. PMID: 27168399Free PMC Article

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