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Hairy cell leukemia(HCL)

MedGen UID:
9727
Concept ID:
C0023443
Neoplastic Process
Synonyms: HCL; Leukemic reticuloendotheliosis
SNOMED CT: LRE - Leukemic reticuloendotheliosis (118613001); HCL - Hairy cell leukemia (118613001); Leukemic reticuloendotheliosis (54087003); Hairy cell leukemia (clinical) (118613001); Hairy cell leukemia (118613001); Leukemic reticuloendotheliosis (118613001); Hairy cell leukemia (54087003)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
Concept ID:
CN307042
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
 
Monarch Initiative: MONDO:0018935
Orphanet: ORPHA58017

Definition

A rare, slowly progressive, chronic leukemia characterized by presence of abnormal B-lymphocytes (medium sized with abundant irregular pale cytoplasm, hair-like cytoplasmic projections/ruffled cytoplasmic border, a round or bean-shaped nucleus and absent nucleoli) in the blood or bone marrow, spleen and peripheral blood pancytopenia, notable monocytopenia, and marked susceptibility to infection. The characteristic immunophenotype is CD11c+, CD25+, CD103+ and CD123+ with a BRAF mutation in most cases. [from ORDO]

Term Hierarchy

Professional guidelines

PubMed

Troussard X, Maître E, Cornet E
Am J Hematol 2022 Feb 1;97(2):226-236. Epub 2021 Nov 8 doi: 10.1002/ajh.26390. PMID: 34710243
Chihara D, Kreitman RJ
Expert Rev Hematol 2020 Oct;13(10):1107-1117. Epub 2020 Sep 24 doi: 10.1080/17474086.2020.1819231. PMID: 32893700
Maitre E, Cornet E, Troussard X
Am J Hematol 2019 Dec;94(12):1413-1422. Epub 2019 Oct 31 doi: 10.1002/ajh.25653. PMID: 31591741

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Hairy Cell Leukemia, 2022

Recent clinical studies

Etiology

Troussard X, Maître E, Cornet E
Am J Hematol 2022 Feb 1;97(2):226-236. Epub 2021 Nov 8 doi: 10.1002/ajh.26390. PMID: 34710243
Kantarjian HM, Jain N, Garcia-Manero G, Welch MA, Ravandi F, Wierda WG, Jabbour EJ
Cancer 2022 Jan 15;128(2):240-259. Epub 2021 Oct 6 doi: 10.1002/cncr.33933. PMID: 34614211Free PMC Article
Chihara D, Kreitman RJ
Expert Rev Hematol 2020 Oct;13(10):1107-1117. Epub 2020 Sep 24 doi: 10.1080/17474086.2020.1819231. PMID: 32893700
Maitre E, Cornet E, Troussard X
Am J Hematol 2019 Dec;94(12):1413-1422. Epub 2019 Oct 31 doi: 10.1002/ajh.25653. PMID: 31591741
Grever MR, Abdel-Wahab O, Andritsos LA, Banerji V, Barrientos J, Blachly JS, Call TG, Catovsky D, Dearden C, Demeter J, Else M, Forconi F, Gozzetti A, Ho AD, Johnston JB, Jones J, Juliusson G, Kraut E, Kreitman RJ, Larratt L, Lauria F, Lozanski G, Montserrat E, Parikh SA, Park JH, Polliack A, Quest GR, Rai KR, Ravandi F, Robak T, Saven A, Seymour JF, Tadmor T, Tallman MS, Tam C, Tiacci E, Troussard X, Zent CS, Zenz T, Zinzani PL, Falini B
Blood 2017 Feb 2;129(5):553-560. Epub 2016 Nov 30 doi: 10.1182/blood-2016-01-689422. PMID: 27903528Free PMC Article

Diagnosis

Troussard X, Maître E, Cornet E
Am J Hematol 2022 Feb 1;97(2):226-236. Epub 2021 Nov 8 doi: 10.1002/ajh.26390. PMID: 34710243
Chihara D, Kreitman RJ
Expert Rev Hematol 2020 Oct;13(10):1107-1117. Epub 2020 Sep 24 doi: 10.1080/17474086.2020.1819231. PMID: 32893700
Kreitman RJ
Leuk Lymphoma 2019 Dec;60(12):2869-2879. Epub 2019 May 9 doi: 10.1080/10428194.2019.1608536. PMID: 31068044Free PMC Article
Fanta PT, Saven A
Cancer Treat Res 2008;142:193-209. doi: 10.1007/978-0-387-73744-7_8. PMID: 18283787
Swords R, Giles F
Med Oncol 2007;24(1):7-15. doi: 10.1007/BF02685898. PMID: 17673807

Therapy

Tiacci E, De Carolis L, Simonetti E, Capponi M, Ambrosetti A, Lucia E, Antolino A, Pulsoni A, Ferrari S, Zinzani PL, Ascani S, Perriello VM, Rigacci L, Gaidano G, Della Seta R, Frattarelli N, Falcucci P, Foà R, Visani G, Zaja F, Falini B
N Engl J Med 2021 May 13;384(19):1810-1823. doi: 10.1056/NEJMoa2031298. PMID: 33979489
Chihara D, Kreitman RJ
Expert Rev Hematol 2020 Oct;13(10):1107-1117. Epub 2020 Sep 24 doi: 10.1080/17474086.2020.1819231. PMID: 32893700
Chihara D, Arons E, Stetler-Stevenson M, Yuan CM, Wang HW, Zhou H, Raffeld M, Xi L, Steinberg SM, Feurtado J, James L, Wilson W, Braylan RC, Calvo KR, Maric I, Dulau-Florea A, Kreitman RJ
J Clin Oncol 2020 May 10;38(14):1527-1538. Epub 2020 Feb 28 doi: 10.1200/JCO.19.02250. PMID: 32109194Free PMC Article
Kreitman RJ
Leuk Lymphoma 2019 Dec;60(12):2869-2879. Epub 2019 May 9 doi: 10.1080/10428194.2019.1608536. PMID: 31068044Free PMC Article
Ravandi F
Clin Lymphoma Myeloma 2009;9 Suppl 3:S254-9. doi: 10.3816/CLM.2009.s.020. PMID: 19778849

Prognosis

Troussard X, Maître E, Cornet E
Am J Hematol 2022 Feb 1;97(2):226-236. Epub 2021 Nov 8 doi: 10.1002/ajh.26390. PMID: 34710243
Chihara D, Kreitman RJ
Expert Rev Hematol 2020 Oct;13(10):1107-1117. Epub 2020 Sep 24 doi: 10.1080/17474086.2020.1819231. PMID: 32893700
Cross M, Dearden C
Curr Oncol Rep 2020 Apr 16;22(5):42. doi: 10.1007/s11912-020-00911-0. PMID: 32297104
Maitre E, Cornet E, Troussard X
Am J Hematol 2019 Dec;94(12):1413-1422. Epub 2019 Oct 31 doi: 10.1002/ajh.25653. PMID: 31591741
Kreitman RJ
Leuk Lymphoma 2019 Dec;60(12):2869-2879. Epub 2019 May 9 doi: 10.1080/10428194.2019.1608536. PMID: 31068044Free PMC Article

Clinical prediction guides

Troussard X, Maître E, Cornet E
Am J Hematol 2022 Feb 1;97(2):226-236. Epub 2021 Nov 8 doi: 10.1002/ajh.26390. PMID: 34710243
Tiacci E, De Carolis L, Simonetti E, Capponi M, Ambrosetti A, Lucia E, Antolino A, Pulsoni A, Ferrari S, Zinzani PL, Ascani S, Perriello VM, Rigacci L, Gaidano G, Della Seta R, Frattarelli N, Falcucci P, Foà R, Visani G, Zaja F, Falini B
N Engl J Med 2021 May 13;384(19):1810-1823. doi: 10.1056/NEJMoa2031298. PMID: 33979489
Maitre E, Cornet E, Troussard X
Am J Hematol 2019 Dec;94(12):1413-1422. Epub 2019 Oct 31 doi: 10.1002/ajh.25653. PMID: 31591741
Ravandi F
Clin Lymphoma Myeloma 2009;9 Suppl 3:S254-9. doi: 10.3816/CLM.2009.s.020. PMID: 19778849
Lill MC, Golde DW
Blood Rev 1990 Dec;4(4):238-44. doi: 10.1016/0268-960x(90)90003-b. PMID: 1706207

Recent systematic reviews

Siddiqui R, Sardar M, Shahzad M, Jose J, Selene I, Shah Z, Qureshi A, Shafqat M, Kashif R, Ahmad M, Mejia-Garcia A, Anwer F
Clin Lymphoma Myeloma Leuk 2021 Oct;21(10):659-666. Epub 2021 Jun 24 doi: 10.1016/j.clml.2021.06.007. PMID: 34275772
Parry-Jones N, Joshi A, Forconi F, Dearden C; BSH guidelines committee
Br J Haematol 2020 Dec;191(5):730-737. Epub 2020 Oct 14 doi: 10.1111/bjh.17055. PMID: 33053222
Andrasiak I, Rybka J, Wrobel T
Clin Lymphoma Myeloma Leuk 2018 Jun;18(6):392-399.e3. Epub 2018 Mar 31 doi: 10.1016/j.clml.2018.03.011. PMID: 29685423
Filippi AR, Franco P, Marinone C, Tarella C, Ricardi U
Am J Hematol 2007 Nov;82(11):1017-21. doi: 10.1002/ajh.20785. PMID: 17654508

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2022
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Hairy Cell Leukemia, 2022

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