HCL-C
MCID: CLS052
MIFTS: 25

Classic Hairy Cell Leukemia (HCL-C)

Categories: Blood diseases, Cancer diseases, Rare diseases

Aliases & Classifications for Classic Hairy Cell Leukemia

MalaCards integrated aliases for Classic Hairy Cell Leukemia:

Name: Classic Hairy Cell Leukemia 58
Leukemic Reticuloendotheliosis 58
Hairy Cell Leukemia 71
Hcl-C 58

Characteristics:

Orphanet epidemiological data:

58
classic hairy cell leukemia
Prevalence: 1-9/1000000 (Europe),1-9/100000 (Europe),1-9/1000000 (United States); Age of onset: Adult;

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

MESH via Orphanet 45 D007943
ICD10 via Orphanet 33 C91.4
UMLS via Orphanet 72 C0023443
Orphanet 58 ORPHA58017
UMLS 71 C0023443

Summaries for Classic Hairy Cell Leukemia

MalaCards based summary : Classic Hairy Cell Leukemia, also known as leukemic reticuloendotheliosis, is related to intrapelvic lymph node leukemic reticuloendotheliosis and reticuloendotheliosis, x-linked. An important gene associated with Classic Hairy Cell Leukemia is BRAF (B-Raf Proto-Oncogene, Serine/Threonine Kinase). The drugs Cladribine and Immunosuppressive Agents have been mentioned in the context of this disorder. Affiliated tissues include spleen, bone marrow and b cells.

Related Diseases for Classic Hairy Cell Leukemia

Diseases related to Classic Hairy Cell Leukemia via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 23)
# Related Disease Score Top Affiliating Genes
1 intrapelvic lymph node leukemic reticuloendotheliosis 11.5
2 reticuloendotheliosis, x-linked 11.0
3 leukemia 10.5
4 splenomegaly 10.4
5 hairy cell leukemia 10.4
6 leukemia, chronic lymphocytic 10.2
7 thrombocytopenia 10.2
8 hypersplenism 10.2
9 monocytic leukemia 10.2
10 granulocytopenia 10.2
11 pancytopenia 10.2
12 neutropenia 10.1
13 vasculitis 10.1
14 mycosis fungoides 10.0
15 lymphoproliferative syndrome 10.0
16 human cytomegalovirus infection 10.0
17 acute leukemia 10.0
18 lymphadenitis 10.0
19 gaucher's disease 10.0
20 skin disease 10.0
21 toxoplasmosis 10.0
22 47,xyy 10.0
23 mycobacterium kansasii 10.0

Graphical network of the top 20 diseases related to Classic Hairy Cell Leukemia:



Diseases related to Classic Hairy Cell Leukemia

Symptoms & Phenotypes for Classic Hairy Cell Leukemia

Drugs & Therapeutics for Classic Hairy Cell Leukemia

Drugs for Classic Hairy Cell Leukemia (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 38)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cladribine Approved, Investigational Phase 2, Phase 3 4291-63-8 20279
2 Immunosuppressive Agents Phase 3
3 Immunologic Factors Phase 3
4 Immunoglobulins Phase 3
5 Immunotoxins Phase 3
6 Antibodies Phase 3
7 Immunotoxin HA22 Phase 3
8 Antibodies, Monoclonal Phase 3
9 Protective Agents Phase 3
10 2-chloro-3'-deoxyadenosine Phase 2, Phase 3
11
Obinutuzumab Approved, Investigational Phase 2 949142-50-1
12
rituximab Approved Phase 2 174722-31-7 10201696
13
Pentostatin Approved, Investigational Phase 2 53910-25-1 40926 439693
14
Adenosine Approved, Investigational Phase 2 58-61-7 60961
15
Pembrolizumab Approved Phase 1, Phase 2 1374853-91-4
16
Vemurafenib Approved Phase 2 918504-65-1 23252090 42611257
17
Triamcinolone Approved, Vet_approved Phase 2 124-94-7 31307
18
Tyrosine Approved, Investigational, Nutraceutical Phase 1, Phase 2 60-18-4 6057
19 Cordycepin Investigational Phase 2 73-03-0
20 interferons Phase 2
21 Anti-Infective Agents Phase 2
22 Interferon alpha-2 Phase 2
23 Antiviral Agents Phase 2
24 Interferon-alpha Phase 2
25 Antirheumatic Agents Phase 2
26 Antimetabolites Phase 2
27 Immunoglobulins, Intravenous Phase 2
28 Alkylating Agents Phase 2
29 Bendamustine Hydrochloride Phase 2
30 Antineoplastic Agents, Immunological Phase 1, Phase 2
31 Protein Kinase Inhibitors Phase 2
32 Triamcinolone hexacetonide Phase 2
33 triamcinolone acetonide Phase 2
34 Triamcinolone diacetate Phase 2
35
Everolimus Approved Phase 1 159351-69-6 6442177 70789204
36
Bortezomib Approved, Investigational Phase 1 179324-69-7 387447 93860
37 Cola
38 Antibodies, Blocking

Interventional clinical trials:

(show all 33)
# Name Status NCT ID Phase Drugs
1 Daily Versus Weekly Administration of 2-Chlorodeoxyadenosine (CDA) in Patients With Hairy Cell Leukemia Completed NCT00003746 Phase 3 2-chlorodeoxyadenosine (CDA) daily;2-chlorodeoxyadenosine weekly
2 A Pivotal Multicenter Trial of Moxetumomab Pasudotox in Relapsed/ Refractory Hairy Cell Leukemia Completed NCT01829711 Phase 3 Moxetumomab pasudotox;IV Bag Protectant for Moxetumomab pasudotox
3 Therapy Optimisation for the Treatment of Hairy Cell Leukemia Recruiting NCT02131753 Phase 2, Phase 3 Cladribine s.c. injection, HCL treatment
4 A Phase II Clinical Trial of 17-(Allylamino)-17- Demethoxygeldanamycin (17-AAG, NSC 330507 and EPL Diluent, NSC 704057) in Adults With Systemic Mastocytosis Completed NCT00132015 Phase 2 tanespimycin
5 Study on the Treatment of Hairy Cell Leukaemia Variant and Relapsing Hairy Cell Leukaemia Completed NCT02157181 Phase 2 2CdA +/- Rituximab
6 A Phase II Efficacy Study of Roferon-A in Hairy Cell Leukemia Completed NCT00001567 Phase 2 Roferon-A
7 Phase II Trial Of BL22 Immunotoxin In Hairy Cell Leukemia Completed NCT00074048 Phase 2 BL22
8 Rituximab (IDEC-C2B8, Mabthera) in Patients With Hairy Cell Leukemia Relapsing After Treatment With 2-Chlorodeoxyadenosine (CDA) Completed NCT00003757 Phase 2
9 A Phase II Study of the BRAF Inhibitor, Vemurafenib, Plus Obinutuzumab in Patients With Previously Untreated Classical Hairy Cell Leukemia Recruiting NCT03410875 Phase 2 Vemurafenib;Obinutuzumab
10 Phase 2 Trial for Binimetinib for Patients With Relapsed/Refractory BRAF Wild Type Hairy Cell Leukemia and Variant Recruiting NCT04322383 Phase 2 binimetinib
11 Phase II Study of 2-Chlorodeoxyadenosine (2CDA) Followed by Rituximab in Hairy Cell Leukemia Recruiting NCT00412594 Phase 2 Cladribine
12 Phase 2 Trial of Encorafenib Plus Binimetinib for Patients With BRAF V600E Mutated Relapsed/Refractory HCL Recruiting NCT04324112 Phase 2 binimetinib;Encorafenib
13 Randomized Phase II Trial of Rituximab With Either Pentostatin or Bendamustine for Multiply Relapsed or Refractory Hairy Cell Leukemia Recruiting NCT01059786 Phase 2 Pentostatin;Rituximab;Bendamustine
14 A Multicenter Phase 2 Study of the Bruton's Tyrosine Kinase Inhibitor PCI-32765 (Ibrutinib) for Treatment of Relapsed Hairy Cell Leukemia Recruiting NCT01841723 Phase 2 Ibrutinib
15 Randomized Trial of Cladribine (CdA) With Simultaneous or Delayed Rituximab to Eliminate Hairy Cell Leukemia Minimal Residual Disease Recruiting NCT00923013 Phase 2 Cladribine;Rituximab
16 A Phase 1b/2 Proof-of-Concept Study of the Combination of ACP-196 (Acalabrutinib) and Pembrolizumab in Subjects With Hematologic Malignancies Active, not recruiting NCT02362035 Phase 1, Phase 2 Acalabrutinib;Pembrolizumab
17 A Phase II Clinical Trial of Anti-Tac(Fv)-PE38 (LMB-2) Immunotoxin for CD25 Positive Hairy Cell Leukemia Active, not recruiting NCT00321555 Phase 2 Anti-Tac(Fv)-PE38 (LMB-2) Immunotoxin
18 A Phase II Study of the BRAF Inhibitor, Vemurafenib, in Patients With Relapsed or Refractory Hairy Cell Leukemia Active, not recruiting NCT01711632 Phase 2 Vemurafenib
19 Retreatment Protocol for BL22 Immunotherapy in Relapsed or Refractory Hairy Cell Leukemia Terminated NCT00924040 Phase 2 BL22 (CAT-3888)
20 A Phase 1, Multicenter, Dose Escalation Study of CAT-8015 in Patient With Relapsed or Refractory Hairy Cell Leukemia (HCL) Unknown status NCT00462189 Phase 1 Immunotoxin therapy;CAT-8015 Immunotoxin
21 A Phase I, Multicenter, Dose Escalation Study of CAT-8015 in Patients With Relapsed or Refractory Hairy Cell Leukemia (HCL) Completed NCT00586924 Phase 1 Moxetumomab Pasudotox (CAT 8015);Moxetumomab Pasudotox (CAT 8015);Moxetumomab Pasudotox (CAT 8015);Moxetumomab Pasudotox (CAT 8015);CAT 8015 (Moxetumomab Pasudotox);Moxetumomab Pasudotox (CAT 8015)
22 A Phase I Study of Moxetumomab Pasudotox-tdfk (Lumoxiti (TM)) and Rituximab (Rituxan (R)) for Relapsed Hairy Cell Leukemia Recruiting NCT03805932 Phase 1 Moxetumomab Pasudotox-tdfk
23 A Phase I/IIa Study to Assess the Safety, Pharmacokinetics, and Pharmacodynamics of PLX8394 in Patients With Advanced, Unresectable Solid Tumors Terminated NCT02012231 Phase 1 PLX8394
24 A Phase 1 Trial of the Combination of Everolimus (RAD001) and Bortezomib (VELCADE) for Relapsed or Refractory Lymphoma Terminated NCT00671112 Phase 1 Bortezomib;Everolimus
25 Descriptive Study to Evaluate the Information and Psychosocial Issues on the Risk on Infertility in Cancer Patients After Receiving Treatment in a Comprehensive Cancer Center Unknown status NCT01295463
26 An Early Access Programme for Moxetumomab Pasudotox in Relapsed/Refractory Hairy Cell Leukemia Approved for marketing NCT03501615 Moxetumomab Pasudotox
27 Rituximab in Hairy Cell Leukemia: a Multicenter Retrospective Study Completed NCT02883946
28 Assessment of the V600E Mutation in the B-RAF Gene in Chronic Lymphoproliferative Disease. Completed NCT01720758
29 Detection of Hairy Cell Leukemia Minimal Residual Disease: Detection by Flow Cytometry Completed NCT00005619
30 Collection of Human Samples to Study Hairy Cell and Other Leukemias and to Develop Recombinant Immunotoxins for Cancer Treatment Recruiting NCT01087333
31 US Post-Marketing Retrospective Observational Safety Study of Moxetumomab Pasudotox-tdfk (LUMOXITI)(TM) Recruiting NCT04125290
32 Clinical Research in Hairy Cell Leukemia: Surveillance and Documentation of Clinical Outcomes in a Rare Form of Adult Leukemia Recruiting NCT02560883
33 A Pilot Study of Aprepitant Versus Ondansetron for the Treatment of Opioid Induced Nausea and Vomiting Withdrawn NCT00499668 aprepitant;ondansetron hydrochloride

Search NIH Clinical Center for Classic Hairy Cell Leukemia

Inferred drug relations via UMLS 71 / NDF-RT 51 :


Cladribine
Interferon Alfa-2a
Interferon Alfa-2b
INTERFERON ALFA-3N,HUMAN LEUKOCYTE DERIVED
interferon alfacon-1
Interferon gamma-1b
Interferons
peginterferon alfa-2a
peginterferon alfa-2b
Recombinant interferon beta-1a
Recombinant interferon beta-1b

Genetic Tests for Classic Hairy Cell Leukemia

Anatomical Context for Classic Hairy Cell Leukemia

MalaCards organs/tissues related to Classic Hairy Cell Leukemia:

40
Spleen, Bone Marrow, B Cells

Publications for Classic Hairy Cell Leukemia

Articles related to Classic Hairy Cell Leukemia:

(show top 50) (show all 143)
# Title Authors PMID Year
1
A BRAF-Negative Classic Hairy Cell Leukemia Patient with Long-Lasting Complete Remission after Rituximab and Pentostatin 61
32539314 2020
2
BRAF inhibitor treatment in classic hairy cell leukemia: a long-term follow-up study of patients treated outside clinical trials. 61
31740808 2020
3
Genome-wide promoter methylation of hairy cell leukemia. 61
30723113 2019
4
Classic hairy cell leukemia complicated by pancytopenia and severe infection: a report of 3 cases treated with vemurafenib. 61
30630817 2019
5
[Hairy cell leukemia complicated by bone marrow necrosis following cladribine administration]. 61
31281144 2019
6
Clinicopathologic and molecular features in hairy cell leukemia-variant: single institutional experience. 61
29955146 2018
7
Consensus guidelines for the diagnosis and management of patients with classic hairy cell leukemia. 61
27903528 2017
8
Bouroncle BA, Wiseman BK, Doan CA. Leukemic reticuloendotheliosis. Blood. 1958;13(7):609-630. 61
27013208 2016
9
Long-term treatment of hairy cell leukemia with interferon-α: still a viable therapeutic option. 61
27429657 2016
10
BRAFV600E induces ABCB1/P-glycoprotein expression and drug resistance in B-cells via AP-1 activation. 61
26350141 2015
11
Current therapeutic options in hairy cell leukemia. 61
24971416 2014
12
How I treat hairy cell leukemia. 61
19843881 2010
13
Hairy cell leukemia variant developing in a background of polycythemia vera. 61
12683904 2003
14
Atypical hairy cell leukemia. 61
11079033 2000
15
Treatment of hairy cell leukemia-variant with cladribine. 61
10706459 1999
16
[A 65 year-old man with leukemic reticuloendotheliosis, fever of unknown origin and multiorgan failure]. 61
9774891 1998
17
Fine needle aspiration of the spleen in hairy cell leukemia. A case report. 61
7631555 1995
18
[Outline of proliferative disorders in the mononuclear phagocytic system--monocytic leukemia, myelomonocytic leukemia, leukemic reticuloendotheliosis]. 61
2733152 1989
19
Antibodies to porcine uteroferrin used in measurement of human tartrate-resistant acid phosphatase. 61
3117432 1987
20
[Hairy cell leukemia (leukemic reticuloendotheliosis). A diagnostic challenge for both the clinician and pathologist]. 61
3603497 1987
21
Hairy cell leukemia in pregnancy: a case report. 61
3799754 1987
22
Cutaneous leukocytoclastic vasculitis complicating hairy cell leukemia (leukemic reticuloendotheliosis). 61
3740885 1986
23
Transformed T lymphocytes infected by a novel isolate of human T cell leukemia virus type II. 61
2998509 1985
24
Prognostic factors in hairy cell leukemia (leukemic reticuloendotheliosis). 61
6692307 1984
25
[Monolayer culture of bone marrow and splenic fibroblasts in lymphoproliferative diseases]. 61
6548048 1984
26
Leukocytoclastic vasculitis in hairy cell leukemia (leukemic reticuloendotheliosis). 61
6651310 1983
27
The human T-cell leukemia/lymphoma virus associated with American adult T-cell leukemia/lymphoma. 61
6223675 1983
28
Phenotype of the hairy cells of leukemic reticuloendotheliosis defined by monoclonal antibodies. 61
6340460 1983
29
[A case of T-cell malignancy with clinical manifestations of chronic leukemic reticuloendotheliosis]. 61
6984088 1982
30
Second malignancies in hairy cell leukemia (leukemic reticuloendotheliosis). 61
6291381 1982
31
[Leukemic reticuloendotheliosis. Clinical entity and therapeutic problems]. 61
7146713 1982
32
A case of leukemic reticuloendotheliosis responding to oxymetholone. 61
7093884 1982
33
Bone involvement in hairy-cell leukemia. 61
7066868 1982
34
[Propedeutic and therapeutic considerations on leukemic reticuloendotheliosis. Report of a case]. 61
7345533 1981
35
Comparison of the tartrate-resistant acid phosphatase in Gaucher's disease and leukemic reticuloendotheliosis. 61
6794944 1981
36
Sideroblastic anemia associated wtih hairy cell leukemia. 61
7248902 1981
37
Beneficial effect of hepatitis in leukemic reticuloendotheliosis. 61
7247595 1981
38
Comparison of tartrate-resistant acid phosphatase in normal mouse tissues to that in the reticulum cells of leukemic reticuloendotheliosis. 61
7226020 1981
39
Infections in hairy cell leukemia (leukemic reticuloendotheliosis). 61
7226028 1981
40
[Hairy cell leukemia (leukemic reticuloendotheliosis) (author's transl)]. 61
7025110 1981
41
Radiation exposure as a possible etiologic factor in hairy cell leukemia (leukemic reticuloendotheliosis). 61
7417955 1980
42
A role for radiation in the treatment of hairy cell leukemia complicated by massive lymphadenopathy: a case report. 61
6154523 1980
43
Hairy cell leukemia with acquired dyserythropoiesis. 61
7362394 1980
44
Immunological and biochemical evidence for identity of tartrate-resistant isoenzymes of acid phosphatases from human serum and tissues. 61
6767568 1980
45
[Interaction of cells of hairy cell leukemia (leukemic reticuloendotheliosis) with colloidal iron]. 61
7376474 1980
46
Leukemic reticuloendotheliosis (hairy cell leukemia): a review of the evidence concerning the immunology and origin of the cell. 61
7015022 1980
47
Leukopheresis therapy of leukemic reticuloendotheliosis (hairy cell leukemia). 61
465739 1979
48
The effectiveness of rubidazone in hairy cell leukemia (leukemic reticuloendotheliosis). 61
454838 1979
49
Treatment of hairy cell leukemia (leukemic reticuloendotheliosis) II. Chlorambucil therapy in postsplenectomy patients with progressive disease. 61
454839 1979
50
Hairy cell leukemia (leukemic reticuloendotheliosis): chemotherapy for splenectomy failures. 61
454837 1979

Variations for Classic Hairy Cell Leukemia

Expression for Classic Hairy Cell Leukemia

Search GEO for disease gene expression data for Classic Hairy Cell Leukemia.

Pathways for Classic Hairy Cell Leukemia

GO Terms for Classic Hairy Cell Leukemia

Sources for Classic Hairy Cell Leukemia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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