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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; 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
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

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
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
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
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
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
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
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
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
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

Clinical prediction guides

Differential Expression of CD43, CD81, and CD200 in Classic Versus Variant Hairy Cell Leukemia.
Salem DA, Scott D, McCoy CS, Liewehr DJ, Venzon DJ, Arons E, Kreitman RJ, Stetler-Stevenson M, Yuan CM
Cytometry B Clin Cytom 2019 Jul;96(4):275-282. Epub 2019 May 11 doi: 10.1002/cyto.b.21785. PMID: 31077558Free PMC Article
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
Molecular variant of hairy cell leukemia with poor prognosis.
Arons E, Kreitman RJ
Leuk Lymphoma 2011 Jun;52 Suppl 2(Suppl 2):99-102. doi: 10.3109/10428194.2011.565841. PMID: 21599610Free 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
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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