HCL
MCID: HRY003
MIFTS: 61

Hairy Cell Leukemia (HCL)

Categories: Blood diseases, Bone diseases, Cancer diseases, Immune diseases, Rare diseases

Aliases & Classifications for Hairy Cell Leukemia

MalaCards integrated aliases for Hairy Cell Leukemia:

Name: Hairy Cell Leukemia 12 73 20 36 29 15 17 70
Hairy Cell Leukemia Variant 12 58 70
Hcl-V 12 58 15
Prolymphocytic Variant of Hairy Cell Leukemia 58
Leukemic Reticuloendotheliosis Variant 58
Leukemic Reticuloendotheliosis 20
Prolymphocytic Variant of Hcl 58
Hairy Cell Leukaemia Variant 12
Leukemia, Hairy Cell 44
Leukemia Hairy Cell 54
Hcl 20

Characteristics:

Orphanet epidemiological data:

58
hairy cell leukemia variant
Inheritance: Multigenic/multifactorial; Prevalence: 1-9/1000000 (United States); Age of onset: Adult; Age of death: adult;

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

Disease Ontology 12 DOID:285 DOID:713
KEGG 36 H00006
ICD9CM 34 202.4
MeSH 44 D007943
NCIt 50 C7401 C7402
SNOMED-CT 67 118613001 54087003
ICD10 32 C91.4
ICD10 via Orphanet 33 C91.4
UMLS via Orphanet 71 C0349633
Orphanet 58 ORPHA300878
UMLS 70 C0023443 C0349633

Summaries for Hairy Cell Leukemia

GARD : 20 Hairy cell leukemia is a rare, slow-growing cancer of the blood in which the bone marrow makes too many B cells ( lymphocytes ), a type of white blood cell that fights infection. The condition is named after these excess B cells which look 'hairy' under a microscope. As the number of leukemia cells increases, fewer healthy white blood cells, red blood cells and platelets are produced. The symptoms include a large spleen (splenomegalia) but without an increase of lymph nodes, and general symptoms such as fever, night sweats, fatigue, weight loss. Blood exams show the decreased number of blood cells and platelets. The diagnosis can be made with the biopsy of the bone marrow, blood exams showing antigens that are released by the B-cells. The leukemia cells show an specific change (variation or mutation ) in a gene involved in cell growth, the BRAF gene, that increases the growth and spread of cancer cells. The decision to treat depends in whether or not symptoms, splenomegaly, or other complications are present. About 10% of all patients will never require treatment. The cause is still unknown. While there is no cure, treatment can lead to remission which can last for years.

MalaCards based summary : Hairy Cell Leukemia, also known as hairy cell leukemia variant, is related to refractory hairy cell leukemia and neutropenia. An important gene associated with Hairy Cell Leukemia is ACP5 (Acid Phosphatase 5, Tartrate Resistant), and among its related pathways/superpathways are Transcriptional misregulation in cancer and Innate Immune System. The drugs Cladribine and 2-chloro-3'-deoxyadenosine have been mentioned in the context of this disorder. Affiliated tissues include bone marrow, b cells and bone, and related phenotypes are hematopoietic system and immune system

Disease Ontology : 12 A chronic lymphocytic leukemia that is characterized by over production of B cells (lymphocytes) by the bone marrow where the B cells appear hairy under a microscope.

KEGG : 36 Hairy cell Leukemia (HCL) is a chronic lymphoproliferative disorder that is defined, according to the WHO classification, as a mature (peripheral) B-cell neoplasm. HCL accounts for between 2-3% of all leukemia cases, with about 600 new cases diagnosed in the U.S. each year. With regard to genetic abnormalities of potential pathogenetic importance in HCL, only a few have been described. Mutations of p53 and Bcl-6 have been reported in about one-third of cases, but their functional significance is unclear. Over-expression of cyclin D1, an important cell cycle regulator, has been reported in cases of HCL as well as mantle cell lymphoma (MCL) and in a few cases of chronic lymphocytic leukemia among lymphoid malignancies.

Wikipedia : 73 Hairy cell leukemia is an uncommon hematological malignancy characterized by an accumulation of abnormal... more...

Related Diseases for Hairy Cell Leukemia

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

(show top 50) (show all 680)
# Related Disease Score Top Affiliating Genes
1 refractory hairy cell leukemia 32.8 IL2RA CD22 BRAF
2 neutropenia 31.5 IL3 IL2 IFNA2 IFNA1
3 lymphopenia 31.1 IL2RA IL2 ADA
4 pancytopenia 30.9 U2AF1 IL3 IL2RA CD5
5 herpes zoster 30.9 IL2 IFNA2 IFNA1
6 t-cell lymphoblastic leukemia/lymphoma 30.8 IL3 IL2RA IL2 CCND1 ADA
7 capillary leak syndrome 30.8 IL3RA IL2 CD22
8 prolymphocytic leukemia 30.8 IL2RA CD5 CCND1 ADA
9 mastocytosis 30.6 IL3 IL2RA IFNA2 IFNA1
10 acquired immunodeficiency syndrome 30.6 IL2RA IL2 IFNA2 IFNA1 ADA
11 myelofibrosis 30.6 U2AF1 IL3 IL2RA IFNA2 IFNA1 ACP5
12 polycythemia vera 30.6 U2AF1 IL3 IFNA2 IFNA1
13 lymphoma 30.6 IL2RA IL2 FCRL5 CD5 CCND1 BRAF
14 adult t-cell leukemia 30.6 IL2RA IL2 IFNA1
15 mycosis fungoides 30.6 ITGAE IL2RA IL2 IFNA2 IFNA1 ADA
16 severe combined immunodeficiency 30.5 IL3 IL2RA IL2 ADA
17 acute leukemia 30.5 IL3RA IL3 IL2 CD22
18 mantle cell lymphoma 30.4 ITGAX CD5 CD200 CCND1
19 pure red-cell aplasia 30.4 IL2RA IL2 IFNA1
20 aplastic anemia 30.4 U2AF1 IL3 IL2RA IL2 IFNA1
21 pleural tuberculosis 30.4 IL2RA IL2 ADA
22 histiocytosis 30.4 U2AF1 ITGAX BRAF
23 mucositis 30.4 IL3 IL2 IFNA1
24 marginal zone b-cell lymphoma 30.3 U2AF1 CD5 CD22 CCND1
25 cutaneous t cell lymphoma 30.3 ITGAE IL2RA IL2 IFNA2 IFNA1 ADA
26 b cell prolymphocytic leukemia 30.3 IL2RA CD5
27 cryoglobulinemia 30.3 IFNA2 IFNA1 CD5
28 langerhans cell histiocytosis 30.3 U2AF1 ITGAX BRAF
29 stomatitis 30.3 IL2 IFNA2 IFNA1
30 cll/sll 30.2 CD5 CD22 CCND1
31 viral infectious disease 30.2 IL2RA IL2 IFNA2 IFNA1
32 systemic mastocytosis 30.2 IL3RA IL2RA IFNA2 IFNA1
33 leukemia, chronic myeloid 30.2 U2AF1 IL3 IFNA2 IFNA1 BRAF ADA
34 renal cell carcinoma, nonpapillary 30.1 IL2RA IL2 IFNA2 IFNA1 CCND1 BRAF
35 skin carcinoma 30.1 IL2 IFNA2 IFNA1 CCND1 BRAF
36 hemolytic anemia 30.1 IL3 IL2RA IL2 IFNA2 IFNA1 CD5
37 essential thrombocythemia 30.1 U2AF1 IL3 IFNA2 IFNA1 CCND1
38 diphtheria 30.1 ITGAX IL3RA IL3 IL2RA IL2
39 hypereosinophilic syndrome 30.0 IL3 IL2RA IL2 IFNA2 IFNA1
40 cryoglobulinemia, familial mixed 29.9 IFNA2 IFNA1 CD5
41 plasmacytoma 29.9 IL2 CD5 CCND1
42 myeloproliferative neoplasm 29.9 U2AF1 IL3 IFNA2 IFNA1
43 sezary's disease 29.9 IL2RA IL2 IFNA2 IFNA1 CD5
44 peripheral t-cell lymphoma 29.7 IL2 CD5 CCND1
45 hematologic cancer 29.6 U2AF1 IL3RA IL3 IL2RA IL2
46 myeloma, multiple 29.6 U2AF1 IL3 IL2 CD5 CCND1 BRAF
47 mature b-cell neoplasm 29.5 U2AF1 IL2 CD5 CD22 CCND1
48 lymphoma, mucosa-associated lymphoid type 29.5 CD5 CD22 CCND1
49 viral hepatitis 29.4 IL2 IFNA2 IFNA1
50 lymphoma, non-hodgkin, familial 29.3 U2AF1 ITGAX IL3 IL2RA IL2 CD5

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



Diseases related to Hairy Cell Leukemia

Symptoms & Phenotypes for Hairy Cell Leukemia

MGI Mouse Phenotypes related to Hairy Cell Leukemia:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.77 ADA ANXA1 BRAF CCND1 CD200 CD22
2 immune system MP:0005387 9.44 ADA ANXA1 BRAF CCND1 CD200 CD22

Drugs & Therapeutics for Hairy Cell Leukemia

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

(show top 50) (show all 148)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cladribine Approved, Investigational Phase 2, Phase 3 4291-63-8 20279
2 2-chloro-3'-deoxyadenosine Phase 2, Phase 3
3
Ondansetron Approved Phase 2 99614-02-5 4595
4
alemtuzumab Approved, Investigational Phase 2 216503-57-0
5
Clotrimazole Approved, Vet_approved Phase 2 23593-75-1 2812
6
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
7
Melphalan Approved Phase 2 148-82-3 4053 460612
8
Mechlorethamine Approved, Investigational Phase 2 51-75-2 4033
9
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
10
Levoleucovorin Approved, Investigational Phase 2 68538-85-2 149436
11
Vorinostat Approved, Investigational Phase 2 149647-78-9 5311
12
Adenosine Approved, Investigational Phase 2 58-61-7 60961
13
Pentostatin Approved, Investigational Phase 2 53910-25-1 439693 40926
14
Thiotepa Approved, Investigational Phase 2 52-24-4 5453
15
Prednisone Approved, Vet_approved Phase 1, Phase 2 53-03-2 5865
16
Pembrolizumab Approved Phase 1, Phase 2 1374853-91-4
17
Triamcinolone Approved, Vet_approved Phase 2 124-94-7 31307
18
Vemurafenib Approved Phase 2 918504-65-1 42611257 23252090
19
Obinutuzumab Approved, Investigational Phase 1, Phase 2 949142-50-1
20
Vidarabine Approved, Investigational Phase 2 24356-66-9 21704 32326
21
Busulfan Approved, Investigational Phase 2 55-98-1 2478
22
Sunitinib Approved, Investigational Phase 2 557795-19-4, 341031-54-7 5329102
23
Ipilimumab Approved Phase 1, Phase 2 477202-00-9
24
Iron Approved Phase 2 7439-89-6 23925 29936
25
Deferasirox Approved, Investigational Phase 2 201530-41-8 5493381 214348
26
Phenylalanine Approved, Investigational, Nutraceutical Phase 2 63-91-2 6140
27
Folic acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
28
Tyrosine Approved, Investigational, Nutraceutical Phase 1, Phase 2 60-18-4 6057
29
Veliparib Investigational Phase 1, Phase 2 912444-00-9 11960529
30 Cordycepin Investigational Phase 2 73-03-0
31
Daclizumab Investigational, Withdrawn Phase 2 152923-56-3
32 Neurotransmitter Agents Phase 2
33 Gastrointestinal Agents Phase 2
34 Psychotropic Drugs Phase 2
35 Antiemetics Phase 2
36 Antipsychotic Agents Phase 2
37 Anti-Anxiety Agents Phase 2
38 interferons Phase 2
39 Interferon-alpha Phase 2
40 Interferon alpha-2 Phase 2
41 Cyclosporins Phase 2
42 Antifungal Agents Phase 2
43 Vitamin B9 Phase 2
44 Folic Acid Antagonists Phase 2
45 Vitamin B Complex Phase 2
46 Histone Deacetylase Inhibitors Phase 2
47 Folate Phase 2
48 Respiratory System Agents Phase 2
49 Anti-Asthmatic Agents Phase 2
50
Beclomethasone Phase 2 4419-39-0 20469

Interventional clinical trials:

(show top 50) (show all 97)
# Name Status NCT ID Phase Drugs
1 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
2 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
3 INSPIRE: An Internet-based RCT for Long-term Survivors of Hematopoietic Stem Cell Transplantation Completed NCT00799461 Phase 3
4 Therapy Optimisation for the Treatment of Hairy Cell Leukemia Recruiting NCT02131753 Phase 2, Phase 3 Cladribine s.c. injection, HCL treatment
5 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
6 Study on the Treatment of Hairy Cell Leukaemia Variant and Relapsing Hairy Cell Leukaemia Completed NCT02157181 Phase 2 2CdA +/- Rituximab
7 A Phase II Efficacy Study of Roferon-A in Hairy Cell Leukemia Completed NCT00001567 Phase 2 Roferon-A
8 Phase II Trial Of BL22 Immunotoxin In Hairy Cell Leukemia Completed NCT00074048 Phase 2 BL22
9 Rituximab (IDEC-C2B8, Mabthera) in Patients With Hairy Cell Leukemia Relapsing After Treatment With 2-Chlorodeoxyadenosine (CDA) Completed NCT00003757 Phase 2
10 Campath (Alemtuzumab) Dose Escalation, Low-Dose TBI and Fludarabine Followed by HLA Class II Mismatched Donor Stem Cell Transplantation for Patients With Hematologic Malignancies: A Multicenter Trial Completed NCT00118352 Phase 2 fludarabine phosphate;cyclosporine;mycophenolate mofetil
11 Prolonged Mycophenolate Mofetil and Truncated Cyclosporine Postgrafting Immunosuppression to Reduce Life-Threatening GVHD After Unrelated Donor Peripheral Blood Cell Transplantation Using Nonmyeloablative Conditioning for Patients With Hematologic Malignancies and Renal Cell Carcinoma - A Multi-Center Trial Completed NCT00078858 Phase 1, Phase 2 fludarabine phosphate;cyclosporine;mycophenolate mofetil
12 Campath® [Alemtuzumab] Dose Escalation, Low-Dose TBI and Fludarabine Followed by HLA Class I Mismatched Donor Stem Cell Transplantation for Patients With Hematologic Malignancies - A Multi-Center Trial Completed NCT00040846 Phase 2 fludarabine phosphate;mycophenolate mofetil;cyclosporine
13 A Multi-Center Study of Nonmyeloablative Conditioning With TBI or Fludarabine/TBI for HLA-matched Related Hematopoietic Cell Transplantation for Treatment of Hematologic Malignancies With Post Grafting Immunosuppression With Tacrolimus and Mycophenolate Mofetil Completed NCT00089011 Phase 2 fludarabine phosphate;mycophenolate mofetil;tacrolimus
14 Induction of Mixed Hematopoietic Chimerism in Patients Using Fludarabine, Low Dose TBI, PBSC Infusion and Post-Transplant Immunosuppression With Cyclosporine and Mycophenolate Mofetil Completed NCT00006251 Phase 1, Phase 2 fludarabine phosphate;cyclosporine;mycophenolate mofetil
15 Pilot Study of Prophylactic Dose-Escalation Donor Lymphocyte Infusion After T Cell Depleted Allogeneic Stem Cell Transplant in High Risk Patients With Hematologic Malignancies Completed NCT01839916 Phase 2
16 A Pilot Trial of Vorinostat Plus Tacrolimus and Methotrexate to Prevent Graft Versus Host Disease Following Unrelated Donor Hematopoietic Stem Cell Transplantation Completed NCT01789255 Phase 2 vorinostat;tacrolimus;cyclosporine;methotrexate
17 A Phase II Study to Evaluate the Efficacy of Oral Beclomethasone Dipropionate for Prevention of Acute GVHD After Hematopoietic Cell Transplantation With Myeloablative Conditioning Regimens Completed NCT00489203 Phase 2 beclomethasone dipropionate;placebo;tacrolimus;methotrexate
18 Phase II Study of MK-2206 in Patients With Relapsed Lymphoma Completed NCT01258998 Phase 2 Akt inhibitor MK2206
19 A Phase II Trial of Reduced Intensity Allogeneic Stem Cell Transplantation With Fludarabine, Melphalan and Low Dose Total Body Irradiation Completed NCT01529827 Phase 2 fludarabine phosphate;melphalan;tacrolimus;mycophenolate mofetil;methotrexate
20 A Phase 1b/2a Study of ABT-888 in Combination With Bendamustine +/- Rituximab in Lymphoma, Multiple Myeloma and Solid Tumors Completed NCT01326702 Phase 1, Phase 2 Bendamustine Hydrochloride;Veliparib
21 Addition of Pre- and Post-Transplant Rituximab for Patients Undergoing Non-myeloablative Allogeneic Hematopoietic Cell Transplantation With Relapsed or Refractory CD20+ B-Cell Malignancies Completed NCT00867529 Phase 2
22 Prevention of DMSO-Related Nausea and Vomiting by Prophylactic Administration of Ondansetron for Patients Receiving Autologous Cryopreserved Peripheral Blood Stem Cells Completed NCT00795769 Phase 2 ondansetron
23 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
24 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
25 Phase II Study of 2-Chlorodeoxyadenosine (2CDA) Followed by Rituximab in Hairy Cell Leukemia Recruiting NCT00412594 Phase 2 Cladribine
26 Randomized Trial of Cladribine (CdA) With Simultaneous or Delayed Rituximab to Eliminate Hairy Cell Leukemia Minimal Residual Disease Recruiting NCT00923013 Phase 2 Cladribine;Rituximab
27 Phase 2 Trial of Encorafenib Plus Binimetinib for Patients With BRAF V600E Mutated Relapsed/Refractory HCL Recruiting NCT04324112 Phase 2 binimetinib;Encorafenib
28 Phase 2 Trial for Binimetinib for Patients With Relapsed/Refractory BRAF Wild Type Hairy Cell Leukemia and Variant Recruiting NCT04322383 Phase 2 binimetinib
29 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
30 A Two Step Approach to Reduced Intensity Allogeneic Hematopoietic Stem Cell Transplantation for High Risk Hematologic Malignancies Recruiting NCT01760655 Phase 2 Fludarabine phosphate;Thiotepa;Cyclophosphamide;Tacrolimus;Mycophenolate mofetil
31 Prospective, Multi-center Phase I/II Trial of Lenalidomide and Dose-Adjusted EPOCH-R in MYC-Associated B-Cell Lymphomas Recruiting NCT02213913 Phase 1, Phase 2 lenalidomide;etoposide;prednisone;vincristine sulfate;doxorubicin hydrochloride;cyclophosphamide
32 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 ACP-196;Pembrolizumab
33 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
34 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
35 A Phase I/II Study of Syk Inhibitor Entospletinib (GS-9973) in Combination With Obinutuzumab in Patients With Relapsed/Refractory Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL) and B-Cell Malignancies Active, not recruiting NCT03010358 Phase 1, Phase 2 Entospletinib
36 A Two Step Approach to Reduced Intensity Allogeneic Hematopoietic Stem Cell Transplantation for Patients With Hematologic Malignancies Active, not recruiting NCT01384513 Phase 2 Fludarabine;Busulfan;Cyclophosphamide (CY);Tacrolimus;Mycophenolate mofetil
37 Retreatment Protocol for BL22 Immunotherapy in Relapsed or Refractory Hairy Cell Leukemia Terminated NCT00924040 Phase 2 BL22 (CAT-3888)
38 A Phase II Study of Sunitinib Malate in Idiopathic Myelofibrosis Terminated NCT00387426 Phase 2 sunitinib malate
39 A Phase I-II Trial of Brentuximab Vedotin Plus Rituximab as Frontline Therapy for Patients With CD30+ and/or EBV+ Lymphomas Terminated NCT01805037 Phase 1, Phase 2 brentuximab vedotin
40 Phase I/II Study of Anti-CTLA-4 Monoclonal Antibody (MDX-010) in B-cell Non-Hodgkin's Lymphoma Terminated NCT00089076 Phase 1, Phase 2
41 A PHASE I/II STUDY OF INTRATUMORAL INJECTION OF IPILIMUMAB IN COMBINATION WITH LOCAL RADIATION IN MELANOMA, NON-HODGKIN LYMPHOMA AND COLORECTAL CARCINOMA Terminated NCT01769222 Phase 1, Phase 2
42 Deferasirox Treatment and Labile Plasma Iron in Iron Overloaded Patients Who Have Undergone Allogeneic Hematopoietic Stem Cell Transplantation Terminated NCT01159067 Phase 2 deferasirox
43 A Phase II Study Investigating Treatment of Post-Allogeneic Transplant Progression or Relapse of CLL/SLL/PLL or NHL With Lenalidomide Alone or With Rituximab Terminated NCT01419795 Phase 2 lenalidomide
44 A Phase I/II Trial of Romidepsin, Rituximab and Lenalidomide (R3) in Relapsed/Refractory B Cell Lymphomas Including Transformed Follicular Lymphoma Withdrawn NCT02281279 Phase 1, Phase 2 lenalidomide;romidepsin
45 A Phase 2 Study to Evaluate the Anti-Tumor Activity of Single Agent Flotetuzumab in Advanced CD123-Positive Hematological Malignancies Withdrawn NCT03739606 Phase 2
46 Hematopoietic Stem Cell Transplantation Using Alternate Donor Umbilical Cord Blood Options Withdrawn NCT01652014 Phase 2 cyclophosphamide;fludarabine phosphate;mycophenolate mofetil;tacrolimus
47 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
48 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)
49 A Phase 1 Study of Lenalidomide Maintenance Following Allogeneic Hematopoietic Cell Transplantation in Patients With Select High Risk Hematological Malignancies Completed NCT01254578 Phase 1 Lenalidomide
50 Phase I Clinical Trial Evaluating the Toxicity, Pharmacokinetics and Biological Effect of Intravenous Bevacizumab (Avastin TM) in Combination With Escalating Doses of Oral AZD2171 for Patients With Advanced Malignancies Completed NCT00458731 Phase 1 cediranib maleate

Search NIH Clinical Center for Hairy Cell Leukemia

Inferred drug relations via UMLS 70 / 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

Cochrane evidence based reviews: leukemia, hairy cell

Genetic Tests for Hairy Cell Leukemia

Genetic tests related to Hairy Cell Leukemia:

# Genetic test Affiliating Genes
1 Hairy Cell Leukemia 29

Anatomical Context for Hairy Cell Leukemia

MalaCards organs/tissues related to Hairy Cell Leukemia:

40
Bone Marrow, B Cells, Bone, Spleen, T Cells, Myeloid, Liver

Publications for Hairy Cell Leukemia

Articles related to Hairy Cell Leukemia:

(show top 50) (show all 3265)
# Title Authors PMID Year
1
Nuclear expression of sox11 is highly associated with mantle cell lymphoma but is independent of t(11;14)(q13;q32) in non-mantle cell B-cell neoplasms. 54 61
19801969 2010
2
Long-term follow-up of interferon-alpha induction and low-dose maintenance therapy in hairy cell leukemia. 61 54
19077050 2009
3
The purification and characterization of alpha interferons and related cytokine receptors--a personal account. 54 61
17683975 2007
4
Central role of protein kinase Cepsilon in constitutive activation of ERK1/2 and Rac1 in the malignant cells of hairy cell leukemia. 61 54
17255340 2007
5
Possible pentostatin-induced symptomatic hyponatremia. 61 54
17192171 2007
6
Role of interferon-alpha administration after 2-deoxycoformycin in the treatment of hairy cell leukemia patients. 54 61
16856905 2006
7
Pentostatin and purine analogs for indolent lymphoid malignancies. 54 61
16563086 2006
8
RhoGTPases and p53 are involved in the morphological appearance and interferon-alpha response of hairy cells. 61 54
16436670 2006
9
High specificity of combined TRAP and DBA.44 expression for hairy cell leukemia. 54 61
15767800 2005
10
The value of tartrate resistant acid phosphatase (TRAP) immunoreactivity in diagnosis of hairy cell leukemia. 54 61
15799758 2005
11
Multifocal leukoencephalopathy and polyneuropathy after 18 years on interferon alpha. 54 61
15621813 2005
12
Immunoglobulin superfamily receptor translocation associated 2 protein on lymphoma cell lines and hairy cell leukemia cells detected by novel monoclonal antibodies. 61 54
15671532 2005
13
[Successful induction and complete improvement of myelofibrosis and erythema nosodum with cladribine in a case of hairy cell leukemia]. 54 61
15222122 2004
14
The diagnostic value of CD123 in B-cell disorders with hairy or villous lymphocytes. 61 54
15020268 2004
15
Phase II study of cladribine (2-chlorodeoxyadenosine) in relapsed or refractory adult T-cell leukemia-lymphoma. 54 61
12841391 2003
16
CD11c gene expression in hairy cell leukemia is dependent upon activation of the proto-oncogenes ras and junD. 61 54
12576324 2003
17
Long-term outcome with pentostatin treatment in hairy cell leukemia patients. A French retrospective study of 238 patients. 61 54
12529659 2003
18
Interferon-alpha 2b and vesnarinone influence levels of tumor necrosis factor-alpha, apoptosis, or interleukin 6 in ESKOL, a hairy cell leukemic cell line. A potential cytokine and oncogene relationship regulating apoptosis is suggested. 61 54
11755467 2002
19
Quantification of the leukocyte common antigen (CD45) in mature B-cell malignancies. 54 61
11754202 2001
20
Interleukin-3 receptor alpha chain (CD123) is widely expressed in hematologic malignancies. 54 61
11726317 2001
21
Characterizations of recombinant human tartrate-resistant acid phosphatase from osteosarcoma: comparison study between recombinant and placental proteins. 61 54
11838317 2001
22
2-chlorodeoxyadenosine (cladribine) in the treatment of hairy cell leukemia. 61 54
11310372 2001
23
Interferon in oncological practice: review of interferon biology, clinical applications, and toxicities. 61 54
11161227 2001
24
Immunohistochemical detection of cyclin D1 using optimized conditions is highly specific for mantle cell lymphoma and hairy cell leukemia. 61 54
11144927 2000
25
Biology of tartrate-resistant acid phosphatase. 61 54
11342331 2000
26
Reconstitution of endogenous interferon a by recombinant interferon in hairy cell leukemia. 61 54
11034083 2000
27
B7 costimulatory molecules from malignant cells in patients with b-cell chronic lymphoproliferative disorders trigger t-cell proliferation. 61 54
11002221 2000
28
Induction of apoptosis in vivo and in vitro in hairy cell leukemia treated by deoxycoformycin. 61 54
11128872 2000
29
Prolonged interferon-alpha-2b treatment of hairy cell leukemia patients. 61 54
10863983 2000
30
The human tartrate-resistant acid phosphatase (TRAP): involvement of the hemin responsive elements (HRE) in transcriptional regulation. 54 61
10784406 2000
31
Cyclin D1 expression in patients with multiple myeloma. 61 54
11920187 2000
32
Hairy cell leukemia with translocation (11;20)(q13;q11) and overexpression of cyclin D1. 54 61
10456674 1999
33
The chemokine receptor CXCR3 is expressed on malignant B cells and mediates chemotaxis. 61 54
10393705 1999
34
Long term outcome of patients with hairy cell leukemia treated with pentostatin. 61 54
9921975 1999
35
Species specificity of monoclonal antibodies to human tartrate-resistant acid phosphatase. 54 61
9888357 1998
36
[Intermittent interferon alfa in the treatment of hairy cell leukemia]. 61 54
9830322 1998
37
Similarities between the sensitivity to 2-chlorodeoxyadenosine of lymphocytes from CLL patients and bryostatin 1-treated WSU-CLL cells: an infrared spectroscopic study. 54 61
9619876 1998
38
Red cell distribution width (RDW) as a marker of disease activity in patients with hairy cell leukemia. 61 54
9588069 1998
39
B lymphocytes from patients with chronic lymphoproliferative disorders are equipped with different costimulatory molecules. 54 61
9354461 1997
40
Immunohistochemical demonstration of acid phosphatase isoenzyme 5 (tartrate-resistant) in paraffin sections of hairy cell leukemia and other hematologic disorders. 61 54
9291460 1997
41
Retrospective analysis of 34 cases of hairy cell leukemia treated with interferon-alpha and/or 2-chlorodeoxyadenosine. 54 61
9299866 1997
42
Blastic transformation of hairy cell leukemia. 61 54
9240906 1997
43
Hypertriglyceridemia during long-term interferon-alpha therapy in a series of hematologic patients. 61 54
9181461 1997
44
Characterization of monoclonal antibodies specific to human tartrate-resistant acid phosphatase. 54 61
9145320 1997
45
Adhesion receptors on peripheral blood leukemic B cells. A comparative study on B cell chronic lymphocytic leukemia and related lymphoma/leukemias. 54 61
9067581 1997
46
CD30 ligand is frequently expressed in human hematopoietic malignancies of myeloid and lymphoid origin. 61 54
9058727 1997
47
Response duration and recovery of CD4+ lymphocytes following deoxycoformycin in interferon-alpha-resistant hairy cell leukemia: 7-year follow-up. 54 61
9001417 1997
48
Behçet's disease in patients with chronic myelogenous leukemia: possible role of interferon-alpha treatment in the occurrence of Behçet's symptoms. 61 54
9031616 1997
49
Cytokine therapy for hematological malignancies. 54 61
8990622 1996
50
Inadequate production of hematopoietic growth factors in hairy cell leukemia: up-regulation of interleukin 6 by recombinant IFN-alpha in vitro. 54 61
8840984 1996

Variations for Hairy Cell Leukemia

Cosmic variations for Hairy Cell Leukemia:

9 (show top 50) (show all 1118)
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM144183945 ZNF296 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.416G>A p.G139D 19:45072541-45072541 0
2 COSM85770123 ZNF296 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.488G>A p.G163D 19:45072541-45072541 0
3 COSM149262901 ZFHX3 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.4136C>T p.T1379M 16:72798546-72798546 0
4 COSM102021008 ZFHX3 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.1394C>T p.T465M 16:72798546-72798546 0
5 COSM87272037 ZFHX3 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.4136C>T p.T1379M 16:72798546-72798546 0
6 COSM124861325 UBR5 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.1331C>G p.S444* 8:102257672-102257672 0
7 COSM127535674 UBR5 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.8126C>G p.S2709* 8:102257672-102257672 0
8 COSM87437165 UBR5 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.6190G>A p.E2064K 8:102278924-102278924 0
9 COSM127546467 UBR5 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.6172G>A p.E2058K 8:102278924-102278924 0
10 COSM127061134 UBR5 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.8147C>G p.S2716* 8:102257672-102257672 0
11 COSM87426596 UBR5 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.8144C>G p.S2715* 8:102257672-102257672 0
12 COSM127073052 UBR5 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.6190G>A p.E2064K 8:102278924-102278924 0
13 COSM87898723 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.817C>T p.R273C 17:7673803-7673803 0
14 COSM143379614 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.527G>T p.S176I 17:7674887-7674887 0
15 COSM144091747 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.354T>A p.C118* 17:7673789-7673789 0
16 COSM142560612 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.323T>G p.V108G 17:7675172-7675172 0
17 COSM144313631 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.442+1G>A p.? 17:7675052-7675052 0
18 COSM111767517 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.644G>T p.S215I 17:7674887-7674887 0
19 COSM144087273 TP53 haematopoietic and lymphoid tissue,spleen,lymphoid neoplasm,mantle cell lymphoma c.265C>T p.R89W 17:7674221-7674221 0
20 COSM105659304 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.528C>G p.C176W 17:7675084-7675084 0
21 COSM144013214 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.626A>G p.Y209C 17:7674872-7674872 0
22 COSM144316769 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.464T>G p.L155R 17:7674950-7674950 0
23 COSM144021629 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.611G>T p.S204I 17:7674887-7674887 0
24 COSM142585417 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.380C>G p.S127* 17:7675115-7675115 0
25 COSM143943565 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.47G>A p.R16H 17:7675088-7675088 0
26 COSM143944858 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.109C>T p.R37* 17:7674945-7674945 0
27 COSM143156843 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.266G>A p.R89Q 17:7674220-7674220 0
28 COSM87897883 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.659A>G p.Y220C 17:7674872-7674872 0
29 COSM87907551 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.581T>G p.L194R 17:7674950-7674950 0
30 COSM112261074 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.581T>G p.L194R 17:7674950-7674950 0
31 COSM122271832 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.421C>T p.R141C 17:7673803-7673803 0
32 COSM145017297 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.701G>A p.R234H 17:7673802-7673802 0
33 COSM112263900 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.949C>T p.Q317* 17:7673579-7673579 0
34 COSM142561467 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.467T>C p.I156T 17:7674947-7674947 0
35 COSM145020962 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.262T>C p.S88P 17:7675233-7675233 0
36 COSM121876485 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.421C>T p.R141C 17:7673803-7673803 0
37 COSM143371865 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.469C>T p.R157* 17:7674945-7674945 0
38 COSM143397150 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.380C>G p.S127* 17:7675115-7675115 0
39 COSM122734399 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.346C>T p.R116W 17:7674221-7674221 0
40 COSM145018791 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.469C>T p.R157* 17:7674945-7674945 0
41 COSM105620655 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.782+378C>T p.? 17:7673803-7673803 0
42 COSM144013168 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.454T>C p.Y152H 17:7675125-7675125 0
43 COSM111773950 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.830G>T p.C277F 17:7673790-7673790 0
44 COSM144017424 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.526+1G>A p.? 17:7675052-7675052 0
45 COSM111758914 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.440T>G p.V147G 17:7675172-7675172 0
46 COSM142567502 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.470G>C p.R157P 17:7674944-7674944 0
47 COSM145022367 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.529G>A p.V177M 17:7674885-7674885 0
48 COSM143370646 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.370T>C p.Y124H 17:7675125-7675125 0
49 COSM142837551 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.742C>T p.R248W 17:7674221-7674221 0
50 COSM143943797 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.266G>A p.R89Q 17:7674220-7674220 0

Expression for Hairy Cell Leukemia

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

Pathways for Hairy Cell Leukemia

Pathways related to Hairy Cell Leukemia according to KEGG:

36
# Name Kegg Source Accession
1 Transcriptional misregulation in cancer hsa05202

Pathways related to Hairy Cell Leukemia according to GeneCards Suite gene sharing:

(show all 25)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.9 ITGAX IL3RA IL3 IL2RA IL2 IFNA2
2
Show member pathways
13.39 ITGAX ITGAE IL3RA IL3 IL2RA IL2
3
Show member pathways
13.31 ITGAX ITGAE IL3RA IL3 IL2RA IL2
4
Show member pathways
13.2 ITGAX IL3RA IL3 IL2RA IL2 IFNA2
5
Show member pathways
13.13 ITGAX IL3RA IL3 IL2RA IL2 IFNA2
6
Show member pathways
12.96 ITGAX ITGAE IL3 IL2RA IL2 BRAF
7
Show member pathways
12.81 IL3RA IL3 IL2RA IL2 IFNA2 IFNA1
8
Show member pathways
12.73 IL2RA IL2 IFNA2 IFNA1 CCND1
9
Show member pathways
12.68 IL3 IL2RA IL2 IFNA2 IFNA1 CCND1
10 12.58 IL3RA IL3 IL2RA IL2 IFNA2 IFNA1
11
Show member pathways
12.35 IL3 IL2 IFNA2 IFNA1 BRAF
12
Show member pathways
12.29 ITGAX ITGAE IL3 IL2 BRAF
13 12.14 ITGAX ITGAE IL3RA IL2RA
14
Show member pathways
12.14 IL3 IL2RA IL2 IFNA2 IFNA1
15
Show member pathways
12.07 ITGAX ITGAE IL3RA IL2RA CCND1 BRAF
16
Show member pathways
12.03 IL3RA IL3 IL2RA IL2
17
Show member pathways
11.9 IL3RA IL3 IL2RA IL2 IFNA2 IFNA1
18 11.76 IL3RA IL3 IL2RA CD5 CD22
19 11.69 ITGAE IL2RA IL2
20
Show member pathways
11.66 IL3 IL2RA IL2
21 11.62 IL2RA IL2 BRAF
22 11.6 ITGAX ITGAE IL3 IL2 BRAF
23
Show member pathways
11.53 IL2RA IL2 BRAF
24 11.42 ITGAX ITGAE IL3RA IL2 IFNA2
25 11.29 ITGAX IL3RA IL3 IL2RA IL2 IFNA2

GO Terms for Hairy Cell Leukemia

Cellular components related to Hairy Cell Leukemia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell surface GO:0009986 9.5 ITGAX IL2RA FCRL5 CD22 CD200 ANXA1
2 external side of plasma membrane GO:0009897 9.23 ITGAX ITGAE IL3RA IL2RA IGHV4-34 CD5

Biological processes related to Hairy Cell Leukemia according to GeneCards Suite gene sharing:

(show all 13)
# Name GO ID Score Top Affiliating Genes
1 adaptive immune response GO:0002250 9.88 IL2 IGHV4-34 IFNA2 IFNA1 ANXA1
2 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.63 IL3 IL2 IFNA2
3 positive regulation of T cell proliferation GO:0042102 9.58 IL2RA IL2 ANXA1
4 MAPK cascade GO:0000165 9.55 IL3RA IL3 IL2RA IL2 BRAF
5 regulation of regulatory T cell differentiation GO:0045589 9.52 IL2RA IL2
6 regulation of T cell differentiation GO:0045580 9.51 BRAF ADA
7 interleukin-2-mediated signaling pathway GO:0038110 9.49 IL2RA IL2
8 response to vitamin E GO:0033197 9.43 CCND1 ADA
9 alpha-beta T cell differentiation GO:0046632 9.37 BRAF ANXA1
10 negative regulation of lymphocyte proliferation GO:0050672 9.32 IL2RA IL2
11 cytokine-mediated signaling pathway GO:0019221 9.28 ITGAX IL3RA IL3 IL2RA IL2 IFNA2
12 regulation of T cell homeostatic proliferation GO:0046013 9.26 IL2RA IL2
13 positive regulation of T cell differentiation GO:0045582 9.13 IL2RA IL2 ADA

Molecular functions related to Hairy Cell Leukemia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 8.92 IL3 IL2 IFNA2 IFNA1

Sources for 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-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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