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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.
 
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

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
Robak T
Leuk Lymphoma 2011 Jun;52 Suppl 2:53-6. doi: 10.3109/10428194.2011.566392. PMID: 21599606
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, 2022

Recent clinical studies

Etiology

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
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
Matutes E
Expert Rev Hematol 2021 Apr;14(4):355-363. Epub 2021 Apr 5 doi: 10.1080/17474086.2021.1908121. PMID: 33759673
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
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

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
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
Matutes E
Expert Rev Hematol 2021 Apr;14(4):355-363. Epub 2021 Apr 5 doi: 10.1080/17474086.2021.1908121. PMID: 33759673
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
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

Therapy

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
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
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
Robak T
Leuk Lymphoma 2011 Jun;52 Suppl 2:53-6. doi: 10.3109/10428194.2011.566392. PMID: 21599606
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

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
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
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
Robak T
Leuk Lymphoma 2011 Jun;52 Suppl 2:53-6. doi: 10.3109/10428194.2011.566392. PMID: 21599606
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

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
Archibald WJ, Baran AM, Williams AM, Salloum RM, Richard Burack W, Evans AG, Syposs CR, Zent CS
Leuk Res 2023 May;128:107053. Epub 2023 Mar 2 doi: 10.1016/j.leukres.2023.107053. PMID: 36906942
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
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
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, 2022
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Hairy Cell Leukemia, 2022

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