Details of Disease

General Information of Disease (ID: DISTD2E5)

• Disease Name: Hairy cell leukaemia
• Synonyms: classic hairy cell leukemia; classic hairy cell leukaemia; HCL; hairy cell leukemia; HCL-C; leukemic reticuloendotheliosis
• Disease Class: 2A82: Mature B-cell leukaemia
• Definition: Hairy cell leukemia (HCL) is a rare type of leukemia in which abnormal B-lymphocytes are present in the bone marrow, spleen and peripheral blood. It is a slowly progressive chronic lymphocytic leukemia (CLL). The name comes from the abnormally shaped lymphocytes with hair-like projections.
• Disease Hierarchy:
  DIS30POX: Small lymphocytic lymphoma
  DISTD2E5: Hairy cell leukaemia
• ICD Code:
  ICD-11: ICD-11: 2A82.2
  ICD-10: ICD-10: C91.4
  ICD-9: ICD-9: 202.4
  Expand ICD-11: '2A82.2
  Expand ICD-10: 'C91.4
  Expand ICD-9: 202.4
• Disease Identifiers:
  MONDO ID: MONDO_0018935
  MESH ID: D007943
  UMLS CUI: C0023443
  MedGen ID: 9727
  Orphanet ID: 58017
  SNOMED CT ID: 118613001

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 6 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Alfaferone	DMCKPUR	Approved	NA	[1]
Cladribine	DM3JDRP	Approved	Small molecular drug	[2]
Interferon Alfa-2b, Recombinant	DM3TPY8	Approved	NA	[3]
Moxetumomab pasudotox	DMN63DZ	Approved	Monoclonal antibody	[4]
Peginterferon alfa-2a	DMJI3BA	Approved	NA	[3]
Pentostatin	DM0HXDS	Approved	Small molecular drug	[5]

This Disease is Treated as An Indication in 2 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
MK-3222	DMQBF7O	Phase 3	Antibody	[6]
Lumoxiti	DMQI970	Phase 1	Antibody	[8]

This Disease is Treated as An Indication in 1 Drugs in Phase 2 Trial

Drug Name	Drug ID	Highest Status	Drug Type	REF
Interferon-alpha 2b	DMQCVZ1	Phase 2/3 Trial	Protein/peptide drug	[7]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 12 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
HEXB	TTKIBKM	Limited	Altered Expression	[9]
ITGAE	TTH0Z37	Limited	Biomarker	[10]
TBX21	TTNF9PH	Limited	Biomarker	[11]
BTK	TTGM6VW	Strong	Biomarker	[12]
CD19	TTW640A	Strong	Biomarker	[13]
CD200	TT0BE68	Strong	Altered Expression	[14]
CD22	TTM6QSK	Strong	Biomarker	[15]
CR2	TT0HUN7	Strong	Biomarker	[16]
IL3RA	TTENHJ0	Strong	Altered Expression	[17]
MAP2K1	TTIDAPM	Strong	Genetic Variation	[18]
MAP2K7	TT6QY3J	Strong	Biomarker	[12]
MS4A1	TTUE541	Strong	Genetic Variation	[15]

This Disease Is Related to 16 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
ENC1	OTJEUB6U	Limited	Biomarker	[9]
FCRL5	OTA7VHNE	Limited	Biomarker	[19]
RHOH	OT1J9SEB	Limited	Altered Expression	[20]
ACSBG1	OTM040MW	moderate	Biomarker	[21]
RAPH1	OTMQXW7S	moderate	Biomarker	[21]
ALKBH3	OTS1CD9Z	Strong	Biomarker	[22]
DBR1	OTLTWLTE	Strong	Biomarker	[23]
GDF1	OTZ1VRBH	Strong	Biomarker	[24]
ITGAX	OTOGIMHE	Strong	Altered Expression	[25]
KLF2	OTIP1UFX	Strong	Genetic Variation	[26]
NDUFAB1	OTF906UR	Strong	Biomarker	[27]
NSA2	OTRU5QPO	Strong	Biomarker	[28]
RNH1	OT6EC79B	Strong	Biomarker	[24]
SHC3	OT305NPA	Strong	Biomarker	[24]
SYN2	OTK0DEGH	Strong	Biomarker	[29]
SYNJ2	OTLRHXP1	Strong	Genetic Variation	[29]

References

• [1] Drugs@FDA. U.S. Food and Drug Administration. U.S. Department of Health & Human Services. 2015
• [2] URL: http://www.guidetopharmacology.org Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Ligand id: 4799).
• [3] Natural products as sources of new drugs over the last 25 years. J Nat Prod. 2007 Mar;70(3):461-77.
• [4] FDA Approved Drug Products from FDA Official Website. 2018. Application Number: (ANDA) 761104.
• [5] URL: http://www.guidetopharmacology.org Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Ligand id: 4805).
• [6] URL: http://www.guidetopharmacology.org Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Ligand id: 8093).
• [7] FDA Approved Drug Products from FDA Official Website. 2019. Application Number: (BLA) 103132.
• [8] ClinicalTrials.gov (NCT03805932) Title: Moxetumomab Pasudotox-tdfk (Lumoxiti ) and Rituximab (Rituxan ) for Relapsed Hairy Cell Leukemia. U.S. National Institutes of Health.
• [9] Disruption of a novel ectodermal neural cortex 1 antisense gene, ENC-1AS and identification of ENC-1 overexpression in hairy cell leukemia.Hum Mol Genet. 2004 Dec 1;13(23):2925-36. doi: 10.1093/hmg/ddh315. Epub 2004 Sep 30.
• [10] Morphological and immunophenotypical features of hairy cell leukaemia involving lymph nodes and extranodal tissues.Histopathology. 2017 Jul;71(1):112-124. doi: 10.1111/his.13206. Epub 2017 Apr 26.
• [11] T-box-expressed-in-T-cells (T-bet) expression by the tumor cells of hairy-cell leukemia correlates with interferon-gamma production.Leuk Lymphoma. 2009 Oct;50(10):1687-92. doi: 10.1080/10428190903161042.
• [12] Contextualizing the Use of Moxetumomab Pasudotox in the Treatment of Relapsed or Refractory Hairy Cell Leukemia.Oncologist. 2020 Jan;25(1):e170-e177. doi: 10.1634/theoncologist.2019-0370. Epub 2019 Oct 18.
• [13] Morphologic Confounders and CD19 Negativity in a Case of Hairy Cell Leukemia.Mediterr J Hematol Infect Dis. 2017 May 1;9(1):e2017033. doi: 10.4084/MJHID.2017.033. eCollection 2017.
• [14] Differential Expression of CD43, CD81, and CD200 in Classic Versus Variant Hairy Cell Leukemia.Cytometry B Clin Cytom. 2019 Jul;96(4):275-282. doi: 10.1002/cyto.b.21785. Epub 2019 May 11.
• [15] New treatment options in hairy cell leukemia with focus on BRAF inhibitors.Hematol Oncol. 2019 Jun;37 Suppl 1:30-37. doi: 10.1002/hon.2594.
• [16] New cell line from hairy-cell leukaemia producing interleukin-6 after Epstein-Barr virus immortalization.Acta Haematol. 1994;92(1):8-13. doi: 10.1159/000204129.
• [17] CD123 as a Therapeutic Target in the Treatment of Hematological Malignancies.Cancers (Basel). 2019 Sep 12;11(9):1358. doi: 10.3390/cancers11091358.
• [18] High prevalence of MAP2K1 mutations in variant and IGHV4-34-expressing hairy-cell leukemias.Nat Genet. 2014 Jan;46(1):8-10. doi: 10.1038/ng.2828. Epub 2013 Nov 17.
• [19] Sandwich ELISAs for soluble immunoglobulin superfamily receptor translocation-associated 2 (IRTA2)/FcRH5 (CD307) proteins in human sera.Clin Chem Lab Med. 2006;44(5):594-602. doi: 10.1515/CCLM.2006.115.
• [20] Repression of the RHOH gene by JunD.Biochem J. 2011 Jul 1;437(1):75-88. doi: 10.1042/BJ20100829.
• [21] Differential and tumor-specific expression of CD160 in B-cell malignancies.Blood. 2011 Aug 25;118(8):2174-83. doi: 10.1182/blood-2011-02-334326. Epub 2011 Jun 28.
• [22] Hairy cell leukemia: a tumor of pre-plasma cells.Blood. 1985 Mar;65(3):620-9.
• [23] Class II human leucocyte antigen DRB1*11 in hairy cell leukaemia patients with and without haemolytic uraemic syndrome.Br J Haematol. 2014 Sep;166(5):729-38. doi: 10.1111/bjh.12956. Epub 2014 Jun 13.
• [24] Expression of CD45 isoforms in chronic B-cell leukaemias.Leuk Res. 1993 Mar;17(3):209-16. doi: 10.1016/0145-2126(93)90003-4.
• [25] CD11c expression in chronic lymphocytic leukemia revisited, related with complications and survival.Int J Lab Hematol. 2017 Oct;39(5):552-556. doi: 10.1111/ijlh.12695. Epub 2017 Jun 12.
• [26] Genomics of Hairy Cell Leukemia.J Clin Oncol. 2017 Mar 20;35(9):1002-1010. doi: 10.1200/JCO.2016.71.1556. Epub 2017 Feb 13.
• [27] Characterization and expression of tartrate-resistant acid phosphatase (TRAP) in hematopoietic cells.Leukemia. 1994 Mar;8(3):359-68.
• [28] A novel human TINP1 gene promotes cell proliferation through inhibition of p53 and p21 expression.Oncol Rep. 2013 Oct;30(4):1848-52. doi: 10.3892/or.2013.2647. Epub 2013 Aug 1.
• [29] Synaptojanin 2 is recognized by HLA class II-restricted hairy cell leukemia-specific T cells.Leukemia. 2003 Dec;17(12):2467-73. doi: 10.1038/sj.leu.2403174.