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Hairy cell leukemia variant

MedGen UID:
91160
•Concept ID:
C0349633
•
Neoplastic Process
Synonyms: Hairy Cell Leukemia Variant; hairy cell leukemia variant; Hairy Cell Leukemia-Variant; HCL-v; HCL-V; leukemic reticuloendotheliosis variant; Leukemic reticuloendotheliosis variant; prolymphocytic variant of hairy cell leukaemia; Prolymphocytic Variant of Hairy Cell Leukemia; Prolymphocytic variant of hairy cell leukemia; prolymphocytic variant of hairy cell leukemia; Prolymphocytic variant of HCL; prolymphocytic variant of HCL; Splenic B-Cell Lymphoma/Leukemia with Prominent Nucleoli
SNOMED CT: Hairy cell leukemia variant (277568007); Hairy cell leukemia variant (1187193000)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
•Concept ID:
CN307042
•
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
See: This record
Unknown inheritance (Orphanet)
 
Monarch Initiative: MONDO:0017600
Orphanet: ORPHA300878

Definition

A rare, malignant splenic B-cell lymphoma/leukemia characterized by circulating abnormal lymphocytes with intermediate morphology between prolymphocytes and hairy cells with positive expression of CD11c and negative expression of CD25, CD123 and the BRAFV600E mutation. Manifestations include splenomegaly, elevated white blood cell (WBC) count, hyper-cellular bone marrow and anemia/thrombocytopenia, but no monocytopenia. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHairy cell leukemia variant
Follow this link to review classifications for Hairy cell leukemia variant in Orphanet.

Professional guidelines

PubMed

Advances in the Treatment of Hairy Cell Leukemia Variant.
Tran J, Gaulin C, Tallman MS
Curr Treat Options Oncol 2022 Jan;23(1):99-116. Epub 2022 Feb 18 doi: 10.1007/s11864-021-00927-z. PMID: 35178674
Management of hairy cell leukemia variant.
Robak T
Leuk Lymphoma 2011 Jun;52 Suppl 2:53-6. doi: 10.3109/10428194.2011.566392. PMID: 21599606
Hairy cell leukemia diagnostic criteria and differential diagnosis.
Summers TA, Jaffe ES
Leuk Lymphoma 2011 Jun;52 Suppl 2(Suppl 2):6-10. Epub 2011 Mar 21 doi: 10.3109/10428194.2011.565435. PMID: 21417827Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Hairy Cell Leukemia, 2024

Recent clinical studies

Etiology

Purine nucleoside analogs plus rituximab are an effective treatment choice for hairy cell leukemia-variant.
Wang Y, Wang T, Yu Y, Wang Q, Yan Y, Li R, Sun Q, Xiong W, Lyu R, Yu Z, Liu W, Sui W, Huang W, Wang H, Li C, Wang J, Zou D, An G, Wang J, Qiu L, Yi S
Ann Hematol 2022 Jun;101(6):1201-1210. Epub 2022 Apr 18 doi: 10.1007/s00277-022-04795-x. PMID: 35437610
CD5-Negative, CD10-Negative Low-Grade B-Cell Lymphoproliferative Disorders of the Spleen.
Schmieg JJ, Muir JM, Aguilera NS, Auerbach A
Curr Oncol 2021 Dec 4;28(6):5124-5147. doi: 10.3390/curroncol28060430. PMID: 34940069Free PMC Article
Diagnostic and therapeutic challenges in hairy cell leukemia-variant: where are we in 2021?
Matutes E
Expert Rev Hematol 2021 Apr;14(4):355-363. Epub 2021 Apr 5 doi: 10.1080/17474086.2021.1908121. PMID: 33759673
Clinicopathologic and molecular features in hairy cell leukemia-variant: single institutional experience.
Angelova EA, Medeiros LJ, Wang W, Muzzafar T, Lu X, Khoury JD, Ravandi F, Patel KP, Hu Z, Kanagal-Shamanna R
Mod Pathol 2018 Nov;31(11):1717-1732. Epub 2018 Jun 28 doi: 10.1038/s41379-018-0093-8. PMID: 29955146
Hairy cell leukemia: Update on molecular profiling and therapeutic advances.
Grever MR, Blachly JS, Andritsos LA
Blood Rev 2014 Sep;28(5):197-203. Epub 2014 Jul 11 doi: 10.1016/j.blre.2014.06.003. PMID: 25110197

Diagnosis

The many faces of nodal and splenic marginal zone lymphomas. A report of the 2022 EA4HP/SH lymphoma workshop.
Zamò A, van den Brand M, Climent F, de Leval L, Dirnhofer S, Leoncini L, Ng SB, Ondrejka SL, Quintanilla-Martinez L, Soma L, Wotherspoon A
Virchows Arch 2023 Sep;483(3):317-331. Epub 2023 Sep 1 doi: 10.1007/s00428-023-03633-3. PMID: 37656249Free PMC Article
Diagnostic and therapeutic challenges in hairy cell leukemia-variant: where are we in 2021?
Matutes E
Expert Rev Hematol 2021 Apr;14(4):355-363. Epub 2021 Apr 5 doi: 10.1080/17474086.2021.1908121. PMID: 33759673
Refractory hairy cell leukemia-variant.
Hu Z, Sun Y, Wang W, Medeiros LJ, Kanagal-Shamanna R
Am J Hematol 2017 Dec;92(12):1398-1399. Epub 2016 Nov 12 doi: 10.1002/ajh.24580. PMID: 27727469
Diagnosis of hairy cell leukemia by flow cytometry.
Stetler-Stevenson M, Tembhare PR
Leuk Lymphoma 2011 Jun;52 Suppl 2:11-3. Epub 2011 Apr 19 doi: 10.3109/10428194.2011.570820. PMID: 21504292
Hairy cell leukemia diagnostic criteria and differential diagnosis.
Summers TA, Jaffe ES
Leuk Lymphoma 2011 Jun;52 Suppl 2(Suppl 2):6-10. Epub 2011 Mar 21 doi: 10.3109/10428194.2011.565435. PMID: 21417827Free PMC Article

Therapy

Hairy Cell Leukemia (HCL) and HCL Variant: Updates and Spotlights on Therapeutic Advances.
Paillassa J, Maitre E, Troussard X
Curr Oncol Rep 2022 Sep;24(9):1133-1143. Epub 2022 Apr 11 doi: 10.1007/s11912-022-01285-1. PMID: 35403971
Refractory hairy cell leukemia-variant.
Hu Z, Sun Y, Wang W, Medeiros LJ, Kanagal-Shamanna R
Am J Hematol 2017 Dec;92(12):1398-1399. Epub 2016 Nov 12 doi: 10.1002/ajh.24580. PMID: 27727469
Potential breakthroughs with investigational drugs for hairy cell leukemia.
Robak T, Wolska A, Robak P
Expert Opin Investig Drugs 2015;24(11):1419-31. Epub 2015 Sep 2 doi: 10.1517/13543784.2015.1081895. PMID: 26329588
Management of hairy cell leukemia variant.
Robak T
Leuk Lymphoma 2011 Jun;52 Suppl 2:53-6. doi: 10.3109/10428194.2011.566392. PMID: 21599606
Hairy-cell leukemia variant: recent view on diagnosis, biology and treatment.
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

Prognosis

Advances in the Treatment of Hairy Cell Leukemia Variant.
Tran J, Gaulin C, Tallman MS
Curr Treat Options Oncol 2022 Jan;23(1):99-116. Epub 2022 Feb 18 doi: 10.1007/s11864-021-00927-z. PMID: 35178674
Ibrutinib in relapsed hairy cell leukemia variant: A case report and review of the literature.
Visentin A, Imbergamo S, Trimarco V, Pravato S, Romano Gargarella L, Frezzato F, Scapinello G, Bertorelle R, Piva E, Facco M, Semenzato G, Piazza F, Trentin L
Hematol Oncol 2020 Dec;38(5):823-826. Epub 2020 Oct 11 doi: 10.1002/hon.2810. PMID: 32979282
Clinicopathologic and molecular features in hairy cell leukemia-variant: single institutional experience.
Angelova EA, Medeiros LJ, Wang W, Muzzafar T, Lu X, Khoury JD, Ravandi F, Patel KP, Hu Z, Kanagal-Shamanna R
Mod Pathol 2018 Nov;31(11):1717-1732. Epub 2018 Jun 28 doi: 10.1038/s41379-018-0093-8. PMID: 29955146
Hairy cell leukemia: Update on molecular profiling and therapeutic advances.
Grever MR, Blachly JS, Andritsos LA
Blood Rev 2014 Sep;28(5):197-203. Epub 2014 Jul 11 doi: 10.1016/j.blre.2014.06.003. PMID: 25110197
Management of hairy cell leukemia variant.
Robak T
Leuk Lymphoma 2011 Jun;52 Suppl 2:53-6. doi: 10.3109/10428194.2011.566392. PMID: 21599606

Clinical prediction guides

Cell therapy for a rare disease- hairy cell leukemia variant.
Fritz C, Wong DP, Rock P, Burack R, Radhakrishnan A, Parameswaran R
Oncoimmunology 2024 Dec 31;13(1):2432062. Epub 2024 Nov 27 doi: 10.1080/2162402X.2024.2432062. PMID: 39604816
Distinguishing hairy cell leukemia variant from hairy cell leukemia: development and validation of diagnostic criteria.
Shao H, Calvo KR, Grönborg M, Tembhare PR, Kreitman RJ, Stetler-Stevenson M, Yuan CM
Leuk Res 2013 Apr;37(4):401-409. Epub 2013 Jan 22 doi: 10.1016/j.leukres.2012.11.021. PMID: 23347903Free PMC Article
Hairy-cell leukemia variant: recent view on diagnosis, biology and treatment.
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
VH4-34+ hairy cell leukemia, a new variant with poor prognosis despite standard therapy.
Arons E, Suntum T, Stetler-Stevenson M, Kreitman RJ
Blood 2009 Nov 19;114(21):4687-95. Epub 2009 Sep 10 doi: 10.1182/blood-2009-01-201731. PMID: 19745070Free PMC Article
Current treatment options in hairy cell leukemia and hairy cell leukemia variant.
Robak T
Cancer Treat Rev 2006 Aug;32(5):365-76. Epub 2006 Jun 15 doi: 10.1016/j.ctrv.2006.04.010. PMID: 16781083

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, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Hairy Cell Leukemia, 2024

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

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