U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

Hairy cell leukemia - etiopathogenesis, diagnosis and modern therapeutic approach.
Maćkowiak K, Jankowiak M, Szewczyk-Golec K, Hołyńska-Iwan I
Biochem Med (Zagreb) 2024 Jun 15;34(2):020502. doi: 10.11613/BM.2024.020502. PMID: 38882583Free PMC Article
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
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
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

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
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
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

Analysis of 3760 hematologic malignancies reveals rare transcriptomic aberrations of driver genes.
Cao X, Huber S, Ahari AJ, Traube FR, Seifert M, Oakes CC, Secheyko P, Vilov S, Scheller IF, Wagner N, Yépez VA, Blombery P, Haferlach T, Heinig M, Wachutka L, Hutter S, Gagneur J
Genome Med 2024 May 20;16(1):70. doi: 10.1186/s13073-024-01331-6. PMID: 38769532Free PMC Article
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

Clinical prediction guides

Analysis of 3760 hematologic malignancies reveals rare transcriptomic aberrations of driver genes.
Cao X, Huber S, Ahari AJ, Traube FR, Seifert M, Oakes CC, Secheyko P, Vilov S, Scheller IF, Wagner N, Yépez VA, Blombery P, Haferlach T, Heinig M, Wachutka L, Hutter S, Gagneur J
Genome Med 2024 May 20;16(1):70. doi: 10.1186/s13073-024-01331-6. PMID: 38769532Free PMC Article
Mutational Profile in 75 Patients With Anti-Myelin-Associated Glycoprotein Neuropathy: Clinical and Hematologic Therapy Response and Hints on New Therapeutic Targets.
Castellani F, Visentin A, Schirinzi E, Salvalaggio A, Cacciavillani M, Candiotto C, Baratè C, Cellini A, Bertorelle R, Siciliano G, Trentin L, Briani C
Neurol Neuroimmunol Neuroinflamm 2023 Jul;10(4) Epub 2023 May 3 doi: 10.1212/NXI.0000000000200122. PMID: 37137530Free 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

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...