HCL
MCID: HRY003
MIFTS: 55

Hairy Cell Leukemia (HCL)

Categories: Blood diseases, Bone diseases, Cancer diseases, Immune diseases, Rare diseases
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Aliases & Classifications for Hairy Cell Leukemia

MalaCards integrated aliases for Hairy Cell Leukemia:

Name: Hairy Cell Leukemia 11 19 75 28 14 16 71
Hairy Cell Leukemia Variant 11 58 71
Hcl-V 11 58 14
Hairy-Cell Leukaemia Without Mention of Remission 33
Prolymphocytic Variant of Hairy Cell Leukemia 58
Leukemic Reticuloendotheliosis Variant 58
Lre - [leukemic Reticuloendotheliosis] 33
Leukaemic Reticuloendotheliosis 33
Leukemic Reticuloendotheliosis 19
Prolymphocytic Variant of Hcl 58
Hairy Cell Leukaemia Variant 11
Hcl - [hairy Cell Leukaemia] 33
Hairy-Cell Leukaemia, Nos 33
Leukemia, Hairy Cell 43
Leukemia Hairy Cell 53
Hcl 19

Characteristics:


Prevelance:

Hairy Cell Leukemia Variant: 1-9/1000000 (United States) 58

Age Of Onset:

Hairy Cell Leukemia Variant: Adult,Elderly 58

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

Disease Ontology 11 DOID:285 DOID:713
ICD9CM 34 202.4
MeSH 43 D007943
NCIt 49 C7401 C7402
SNOMED-CT 68 118613001 54087003
ICD10 31 C91.4
ICD10 via Orphanet 32 C91.4
UMLS via Orphanet 72 C0349633
Orphanet 58 ORPHA300878
ICD11 33 82152208
UMLS 71 C0023443 C0349633

Summaries for Hairy Cell Leukemia

GARD: 19 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) in a gene involved in cell growth, the BRAF gene, that increases the growth and spread of cancer cells. The cause is still unknown.

MalaCards based summary: Hairy Cell Leukemia, also known as hairy cell leukemia variant, is related to refractory hairy cell leukemia and prolymphocytic leukemia. An important gene associated with Hairy Cell Leukemia is IGHV4-34 (Immunoglobulin Heavy Variable 4-34), and among its related pathways/superpathways are Innate Immune System and MIF Mediated Glucocorticoid Regulation. 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 Increased shRNA abundance (Z-score > 2) and immune system

Orphanet: 58 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.

Disease Ontology: 11 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.

Wikipedia: 75 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 869)
# Related Disease Score Top Affiliating Genes
1 refractory hairy cell leukemia 32.7 IL3RA CD22
2 prolymphocytic leukemia 31.4 CD5 CCND1 ADA
3 neutropenia 31.3 IL3 IL2 IFNA2 IFNA1 CCND1
4 pancytopenia 31.3 U2AF1 IL3RA IL3 IL2RA CD5 CD22
5 lymphoma, hodgkin, classic 31.3 IL3RA IL3 IL2RB IL2RA IL2
6 lymphoma 31.2 IL2RA IL2 FCRL5 CD5 CCND1 BRAF
7 lymphopenia 31.1 IL2RA IL2 ADA
8 myelofibrosis 31.1 U2AF1 IL3 IL2RA IFNA2 IFNA1 ACP5
9 marginal zone b-cell lymphoma 31.0 U2AF1 CD5 CD22 CCND1
10 hematologic cancer 30.9 U2AF1 IL3RA IL3 IL2RA IL2 DCK
11 polycythemia vera 30.9 U2AF1 IL3 IFNA2 IFNA1
12 herpes zoster 30.8 IL2 IFNA2 IFNA1
13 thrombocytopenia 30.8 U2AF1 IL3 IL2 IFNB1 IFNA2 IFNA1
14 myeloproliferative neoplasm 30.7 U2AF1 IL3 IFNA2 IFNA1
15 capillary leak syndrome 30.7 IL3RA IL2 CD22
16 splenic marginal zone lymphoma 30.7 U2AF1 ITGAX IL3RA CD5 CD22 CCND1
17 cryoglobulinemia 30.7 IFNA2 IFNA1 CD5
18 kaposi sarcoma 30.7 IFNB1 IFNA2 IFNA1
19 viral infectious disease 30.7 IL2RA IL2 IFNB1 IFNA2 IFNA1
20 mantle cell lymphoma 30.7 U2AF1 IL2 CD5 CD22 CCND1
21 listeriosis 30.7 ITGAX IL2 IFNB1
22 histiocytosis 30.7 U2AF1 ITGAX BRAF
23 mastocytosis 30.7 IL3RA IL3 IL2RA IFNA2
24 chronic lymphocytic leukemia/small lymphocytic lymphoma 30.6 CD5 CD22 CCND1
25 mycosis fungoides 30.6 IL2RB IL2RA IL2 IFNA2 IFNA1 ADA
26 lymphoplasmacytic lymphoma 30.6 CD5 CD22 CCND1
27 sarcoidosis 1 30.6 IL2RB IL2RA IL2 ADA
28 b-cell prolymphocytic leukemia 30.6 ITGAX CD5 CD22 CCND1
29 deficiency anemia 30.6 U2AF1 IL3 IL2 IFNA2 IFNA1 CD5
30 miliary tuberculosis 30.6 IL2 ADA
31 essential thrombocythemia 30.5 U2AF1 IL3 IFNA2 IFNA1
32 leukemia, chronic myeloid 30.5 U2AF1 IL3 IL2 IFNA2 IFNA1 CCND1
33 lymphoma, mucosa-associated lymphoid type 30.5 CD5 CD22 CCND1
34 adult t-cell leukemia/lymphoma 30.5 IL2RA IL2 CD5
35 t-cell acute lymphoblastic leukemia 30.5 U2AF1 IL3 IL2RB IL2RA IL2 CD5
36 melanoma 30.4 IL2RB IL2 IFNB1 IFNA2 IFNA1 CCND1
37 myeloma, multiple 30.4 U2AF1 IL3 IL2 IFNA2 IFNA1 CCND1
38 common cold 30.4 IL2 IFNB1 IFNA2 IFNA1
39 acute graft versus host disease 30.4 IL2RA IL2
40 pleural tuberculosis 30.4 IL2RA IL2 ADA
41 composite lymphoma 30.4 CD5 CD22
42 diarrhea 30.4 IL3 IL2 IFNB1 IFNA2 IFNA1
43 peripheral t-cell lymphoma 30.4 IL2RB IL2RA CD5
44 mature b-cell neoplasm 30.3 U2AF1 CD5 CD22 CCND1
45 blood platelet disease 30.3 U2AF1 IL3 IL2 IFNA1 CD5
46 cryoglobulinemia, familial mixed 30.3 IFNA2 IFNA1 CD5
47 plasmacytoma 30.3 IL2 CD5 CCND1
48 acquired immunodeficiency syndrome 30.3 IL2RB IL2RA IL2 IFNA2 IFNA1 CCND1
49 t-cell large granular lymphocyte leukemia 30.3 U2AF1 IL2RB IL2 CD5
50 sezary's disease 30.3 IL2RB IL2RA IL2 IFNA2 IFNA1 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

GenomeRNAi Phenotypes related to Hairy Cell Leukemia according to GeneCards Suite gene sharing:

25 (show all 30)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-104 9.83 IL2RA
2 Increased shRNA abundance (Z-score > 2) GR00366-A-114 9.83 IL2RB
3 Increased shRNA abundance (Z-score > 2) GR00366-A-115 9.83 IL2RA
4 Increased shRNA abundance (Z-score > 2) GR00366-A-121 9.83 FCRL5 IL2RA
5 Increased shRNA abundance (Z-score > 2) GR00366-A-122 9.83 FCRL5
6 Increased shRNA abundance (Z-score > 2) GR00366-A-130 9.83 IL2RA
7 Increased shRNA abundance (Z-score > 2) GR00366-A-132 9.83 IL3RA
8 Increased shRNA abundance (Z-score > 2) GR00366-A-138 9.83 IL2RA
9 Increased shRNA abundance (Z-score > 2) GR00366-A-15 9.83 IL2RB
10 Increased shRNA abundance (Z-score > 2) GR00366-A-174 9.83 IL2RA
11 Increased shRNA abundance (Z-score > 2) GR00366-A-18 9.83 IL3RA
12 Increased shRNA abundance (Z-score > 2) GR00366-A-181 9.83 IL3RA
13 Increased shRNA abundance (Z-score > 2) GR00366-A-183 9.83 IL3RA
14 Increased shRNA abundance (Z-score > 2) GR00366-A-202 9.83 IL2RA
15 Increased shRNA abundance (Z-score > 2) GR00366-A-214 9.83 IL2RB
16 Increased shRNA abundance (Z-score > 2) GR00366-A-25 9.83 IL2RA
17 Increased shRNA abundance (Z-score > 2) GR00366-A-26 9.83 FCRL5
18 Increased shRNA abundance (Z-score > 2) GR00366-A-34 9.83 IL2RA
19 Increased shRNA abundance (Z-score > 2) GR00366-A-35 9.83 FCRL5
20 Increased shRNA abundance (Z-score > 2) GR00366-A-36 9.83 FCRL5
21 Increased shRNA abundance (Z-score > 2) GR00366-A-39 9.83 FCRL5
22 Increased shRNA abundance (Z-score > 2) GR00366-A-46 9.83 IL2RA IL3RA
23 Increased shRNA abundance (Z-score > 2) GR00366-A-5 9.83 IL2RB
24 Increased shRNA abundance (Z-score > 2) GR00366-A-52 9.83 IL2RA
25 Increased shRNA abundance (Z-score > 2) GR00366-A-67 9.83 IL2RA
26 Increased shRNA abundance (Z-score > 2) GR00366-A-79 9.83 IL3RA
27 Increased shRNA abundance (Z-score > 2) GR00366-A-81 9.83 FCRL5
28 Increased shRNA abundance (Z-score > 2) GR00366-A-88 9.83 FCRL5
29 Increased shRNA abundance (Z-score > 2) GR00366-A-89 9.83 IL3RA
30 Increased shRNA abundance (Z-score > 2) GR00366-A-91 9.83 FCRL5

MGI Mouse Phenotypes related to Hairy Cell Leukemia:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 10.11 ACP5 ADA BRAF CCND1 CD22 CD5
2 normal MP:0002873 9.91 ADA BRAF CCND1 CD5 DCK IFNA1
3 hematopoietic system MP:0005397 9.58 ACP5 ADA BRAF CCND1 CD22 CD5
4 respiratory system MP:0005388 9.56 ADA BRAF CCND1 IFNA1 IFNA2 IL2

Drugs & Therapeutics for Hairy Cell Leukemia

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

(show top 50) (show all 149)
# 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 14485373
3
Alemtuzumab Approved, Investigational Phase 2 216503-57-0
4
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
5
Clotrimazole Approved, Vet_approved Phase 2 23593-75-1 2812
6
Ondansetron Approved, Withdrawn Phase 2 99614-02-5 4595
7
Levoleucovorin Approved, Experimental, Investigational Phase 2 68538-85-2, 58-05-9, 73951-54-9 149436 6006
8
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 4112 126941
9
Busulfan Approved, Investigational Phase 2 55-98-1 2478
10
Melphalan Approved Phase 2 148-82-3 4053 460612
11
Mechlorethamine Approved, Investigational Phase 2 51-75-2 4033
12
Vorinostat Approved, Investigational Phase 2 149647-78-9 5311
13
Adenosine Approved, Investigational Phase 2 58-61-7 60961
14
Pentostatin Approved, Investigational Phase 2 53910-25-1 439693
15
Thiotepa Approved, Investigational Phase 2 52-24-4 5453
16
Obinutuzumab Approved, Investigational Phase 2 949142-50-1
17
Prednisone Approved, Vet_approved Phase 1, Phase 2 53-03-2 5865
18
Vemurafenib Approved Phase 2 918504-65-1 42611257
19
Triamcinolone Approved, Vet_approved Phase 2 124-94-7 31307
20
Acalabrutinib Approved, Investigational Phase 1, Phase 2 1420477-60-6 71226663 71226662
21
Pembrolizumab Approved Phase 1, Phase 2 1374853-91-4 254741536
22
Sunitinib Approved, Investigational Phase 2 557795-19-4 5329102
23
Brentuximab vedotin Approved, Investigational Phase 1, Phase 2 914088-09-8
24
Ipilimumab Approved Phase 1, Phase 2 477202-00-9
25
Iron Approved Phase 2 7439-89-6 29936
26
Deferasirox Approved, Investigational Phase 2 201530-41-8 214348 5493381
27
Folic acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
28
D-Phenylalanine Approved, Experimental, Investigational, Nutraceutical Phase 2 63-91-2, 673-06-3 6140 71567
29
D-Tyrosine Approved, Experimental, Investigational, Nutraceutical Phase 2 133585-56-5, 60-18-4, 556-02-5 1153 6057
30
Veliparib Investigational Phase 1, Phase 2 912444-00-9 11960529
31
Cordycepin Investigational Phase 2 73-03-0 248010
32
Daclizumab Investigational, Withdrawn Phase 2 152923-56-3
33 Neurotransmitter Agents Phase 2
34 Antiemetics Phase 2
35 Gastrointestinal Agents Phase 2
36 Dermatologic Agents Phase 2
37 Cyclosporins Phase 2
38 Antifungal Agents Phase 2
39 Interferon alpha-2 Phase 2
40 interferons Phase 2
41 Interferon-alpha Phase 2
42 Bendamustine Hydrochloride Phase 1, Phase 2
43 Antipsychotic Agents Phase 2
44 Anti-Anxiety Agents Phase 2
45 Psychotropic Drugs Phase 2
46 Folic Acid Antagonists Phase 2
47 Folate Phase 2
48 Vitamin B9 Phase 2
49 Anti-Asthmatic Agents Phase 2
50 Vitamin B Complex Phase 2

Interventional clinical trials:

(show top 50) (show all 97)
# 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 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 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
6 A Phase II Efficacy Study of Roferon-A in Hairy Cell Leukemia Completed NCT00001567 Phase 2 Roferon-A
7 Rituximab (IDEC-C2B8, Mabthera) in Patients With Hairy Cell Leukemia Relapsing After Treatment With 2-Chlorodeoxyadenosine (CDA) Completed NCT00003757 Phase 2
8 Phase II Trial Of BL22 Immunotoxin In Hairy Cell Leukemia Completed NCT00074048 Phase 2 BL22
9 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
10 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 Completed NCT03010358 Phase 1, Phase 2 Entospletinib
11 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
12 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
13 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
14 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
15 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
16 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
17 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
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 Two Step Approach to Reduced Intensity Allogeneic Hematopoietic Stem Cell Transplantation for Patients With Hematologic Malignancies Completed NCT01384513 Phase 2 Fludarabine;Busulfan;Cyclophosphamide (CY);Tacrolimus;Mycophenolate mofetil
21 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
22 Study on the Treatment of Hairy Cell Leukaemia Variant and Relapsing Hairy Cell Leukaemia Completed NCT02157181 Phase 2 2CdA +/- Rituximab
23 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
24 Phase II Study of 2-Chlorodeoxyadenosine (2CDA) Followed by Rituximab in Hairy Cell Leukemia Recruiting NCT00412594 Phase 2 Cladribine
25 Phase 2 Trial for Binimetinib for Patients With Relapsed/Refractory BRAF Wild Type Hairy Cell Leukemia and Variant Recruiting NCT04322383 Phase 2 binimetinib
26 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
27 Randomized Trial of Cladribine (CdA) With Simultaneous or Delayed Rituximab to Eliminate Hairy Cell Leukemia Minimal Residual Disease Recruiting NCT00923013 Phase 2 Cladribine;Rituximab
28 A Single Arm Phase II Pilot Study of Low Dose Vemurafenib Plus Rituximab in the Front-line and Relapsed/Refractory Treatment of Hairy Cell Leukemia Recruiting NCT05388123 Phase 2 Low dose vemurafenib plus rituximab
29 A Phase 2, Open-Label, Multicenter, Basket Study Evaluating the Safety and Efficacy of Brexucabtagene Autoleucel in Adults With Rare B-cell Malignancies (ZUMA-25) Recruiting NCT05537766 Phase 2 Cyclophosphamide;Fludarabine
30 Phase 2 Trial of Encorafenib Plus Binimetinib for Patients With BRAF V600 Mutated Relapsed/Refractory HCL Recruiting NCT04324112 Phase 2 binimetinib;Encorafenib
31 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
32 A Multicenter Phase 2 Study of the Bruton's Tyrosine Kinase Inhibitor PCI-32765 (Ibrutinib) for Treatment of Relapsed Hairy Cell Leukemia Active, not recruiting NCT01841723 Phase 2 Ibrutinib
33 Prospective, Multi-center Phase I/II Trial of Lenalidomide and Dose-Adjusted EPOCH-R in MYC-Associated B-Cell Lymphomas Active, not recruiting NCT02213913 Phase 1, Phase 2 lenalidomide;etoposide;prednisone;vincristine sulfate;doxorubicin hydrochloride;cyclophosphamide
34 A Phase II Study of the BRAF Inhibitor, Vemurafenib, Plus Obinutuzumab in Patients With Previously Untreated Classical Hairy Cell Leukemia Active, not recruiting NCT03410875 Phase 2 Vemurafenib;Obinutuzumab
35 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
36 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
37 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
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 Retreatment Protocol for BL22 Immunotherapy in Relapsed or Refractory Hairy Cell Leukemia Terminated NCT00924040 Phase 2 BL22 (CAT-3888)
41 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
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 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
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 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
47 Hematopoietic Stem Cell Transplantation Using Alternate Donor Umbilical Cord Blood Options Withdrawn NCT01652014 Phase 2 cyclophosphamide;fludarabine phosphate;mycophenolate mofetil;tacrolimus
48 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
49 A Phase I Study of Flavopiridol, Fludarabine and Rituximab in Indolent B-cell Lymphoproliferative Disorders and Mantle Cell Lymphoma Completed NCT00058227 Phase 1 alvocidib;fludarabine phosphate
50 A Phase I Pharmacokinetic Study of STI571 in Patients With Advanced Malignancies and Varying Levels of Liver Dysfunction Completed NCT00025415 Phase 1 imatinib mesylate

Search NIH Clinical Center for Hairy Cell Leukemia

Inferred drug relations via UMLS 71 / NDF-RT 50 :


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 28

Anatomical Context for Hairy Cell Leukemia

Organs/tissues related to Hairy Cell Leukemia:

MalaCards : Bone Marrow, B Cells, Bone, Spleen, T Cells, Liver, Myeloid

Publications for Hairy Cell Leukemia

Articles related to Hairy Cell Leukemia:

(show top 50) (show all 3482)
# 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. 53 62
19801969 2010
2
Long-term follow-up of interferon-alpha induction and low-dose maintenance therapy in hairy cell leukemia. 53 62
19077050 2009
3
The purification and characterization of alpha interferons and related cytokine receptors--a personal account. 53 62
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. 53 62
17255340 2007
5
Possible pentostatin-induced symptomatic hyponatremia. 53 62
17192171 2007
6
Role of interferon-alpha administration after 2-deoxycoformycin in the treatment of hairy cell leukemia patients. 53 62
16856905 2006
7
Pentostatin and purine analogs for indolent lymphoid malignancies. 53 62
16563086 2006
8
RhoGTPases and p53 are involved in the morphological appearance and interferon-alpha response of hairy cells. 53 62
16436670 2006
9
High specificity of combined TRAP and DBA.44 expression for hairy cell leukemia. 53 62
15767800 2005
10
The value of tartrate resistant acid phosphatase (TRAP) immunoreactivity in diagnosis of hairy cell leukemia. 53 62
15799758 2005
11
Multifocal leukoencephalopathy and polyneuropathy after 18 years on interferon alpha. 53 62
15621813 2005
12
Immunoglobulin superfamily receptor translocation associated 2 protein on lymphoma cell lines and hairy cell leukemia cells detected by novel monoclonal antibodies. 53 62
15671532 2005
13
[Successful induction and complete improvement of myelofibrosis and erythema nosodum with cladribine in a case of hairy cell leukemia]. 53 62
15222122 2004
14
The diagnostic value of CD123 in B-cell disorders with hairy or villous lymphocytes. 53 62
15020268 2004
15
Phase II study of cladribine (2-chlorodeoxyadenosine) in relapsed or refractory adult T-cell leukemia-lymphoma. 53 62
12841391 2003
16
CD11c gene expression in hairy cell leukemia is dependent upon activation of the proto-oncogenes ras and junD. 53 62
12576324 2003
17
Long-term outcome with pentostatin treatment in hairy cell leukemia patients. A French retrospective study of 238 patients. 53 62
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. 53 62
11755467 2002
19
Interleukin-3 receptor alpha chain (CD123) is widely expressed in hematologic malignancies. 53 62
11726317 2001
20
Quantification of the leukocyte common antigen (CD45) in mature B-cell malignancies. 53 62
11754202 2001
21
Characterizations of recombinant human tartrate-resistant acid phosphatase from osteosarcoma: comparison study between recombinant and placental proteins. 53 62
11838317 2001
22
Interferon in oncological practice: review of interferon biology, clinical applications, and toxicities. 53 62
11161227 2001
23
2-chlorodeoxyadenosine (cladribine) in the treatment of hairy cell leukemia. 53 62
11310372 2001
24
Immunohistochemical detection of cyclin D1 using optimized conditions is highly specific for mantle cell lymphoma and hairy cell leukemia. 53 62
11144927 2000
25
Biology of tartrate-resistant acid phosphatase. 53 62
11342331 2000
26
Reconstitution of endogenous interferon a by recombinant interferon in hairy cell leukemia. 53 62
11034083 2000
27
Induction of apoptosis in vivo and in vitro in hairy cell leukemia treated by deoxycoformycin. 53 62
11128872 2000
28
B7 costimulatory molecules from malignant cells in patients with b-cell chronic lymphoproliferative disorders trigger t-cell proliferation. 53 62
11002221 2000
29
Prolonged interferon-alpha-2b treatment of hairy cell leukemia patients. 53 62
10863983 2000
30
The human tartrate-resistant acid phosphatase (TRAP): involvement of the hemin responsive elements (HRE) in transcriptional regulation. 53 62
10784406 2000
31
Cyclin D1 expression in patients with multiple myeloma. 53 62
11920187 2000
32
Hairy cell leukemia with translocation (11;20)(q13;q11) and overexpression of cyclin D1. 53 62
10456674 1999
33
The chemokine receptor CXCR3 is expressed on malignant B cells and mediates chemotaxis. 53 62
10393705 1999
34
Long term outcome of patients with hairy cell leukemia treated with pentostatin. 53 62
9921975 1999
35
Species specificity of monoclonal antibodies to human tartrate-resistant acid phosphatase. 53 62
9888357 1998
36
[Intermittent interferon alfa in the treatment of hairy cell leukemia]. 53 62
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. 53 62
9619876 1998
38
Red cell distribution width (RDW) as a marker of disease activity in patients with hairy cell leukemia. 53 62
9588069 1998
39
B lymphocytes from patients with chronic lymphoproliferative disorders are equipped with different costimulatory molecules. 53 62
9354461 1997
40
Immunohistochemical demonstration of acid phosphatase isoenzyme 5 (tartrate-resistant) in paraffin sections of hairy cell leukemia and other hematologic disorders. 53 62
9291460 1997
41
Retrospective analysis of 34 cases of hairy cell leukemia treated with interferon-alpha and/or 2-chlorodeoxyadenosine. 53 62
9299866 1997
42
Blastic transformation of hairy cell leukemia. 53 62
9240906 1997
43
Hypertriglyceridemia during long-term interferon-alpha therapy in a series of hematologic patients. 53 62
9181461 1997
44
Characterization of monoclonal antibodies specific to human tartrate-resistant acid phosphatase. 53 62
9145320 1997
45
Adhesion receptors on peripheral blood leukemic B cells. A comparative study on B cell chronic lymphocytic leukemia and related lymphoma/leukemias. 53 62
9067581 1997
46
CD30 ligand is frequently expressed in human hematopoietic malignancies of myeloid and lymphoid origin. 53 62
9058727 1997
47
Behçet's disease in patients with chronic myelogenous leukemia: possible role of interferon-alpha treatment in the occurrence of Behçet's symptoms. 53 62
9031616 1997
48
Response duration and recovery of CD4+ lymphocytes following deoxycoformycin in interferon-alpha-resistant hairy cell leukemia: 7-year follow-up. 53 62
9001417 1997
49
Cytokine therapy for hematological malignancies. 53 62
8990622 1996
50
Inadequate production of hematopoietic growth factors in hairy cell leukemia: up-regulation of interleukin 6 by recombinant IFN-alpha in vitro. 53 62
8840984 1996

Variations for Hairy Cell Leukemia

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 GeneCards Suite gene sharing:

(show all 32)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.88 ITGAX IL3RA IL3 IL2RB IL2RA IL2
2
Show member pathways
13.51 IL3RA IL3 IL2RB IL2RA IL2 IFNB1
3
Show member pathways
13.39 ITGAX IL3RA IL3 IL2RB IL2RA IL2
4
Show member pathways
13.31 ITGAX IL3RA IL3 IL2RB IL2RA IL2
5
Show member pathways
13.15 CCND1 IFNA1 IFNA2 IFNB1 IL2 IL2RA
6
Show member pathways
12.77 IL2RB IL2RA IL2 CCND1 BRAF
7
Show member pathways
12.59 IL2RB IL2RA IL2 IFNB1 IFNA2 IFNA1
8
Show member pathways
12.51 IL3RA IL3 IL2RB IL2RA IL2 IFNB1
9
Show member pathways
12.46 ITGAX IL3RA IL3 IL2RB IL2RA IL2
10 12.45 IL3 IL2RB IL2RA IL2 IFNA1
11
Show member pathways
12.35 ITGAX IL3RA IL2RB IL2RA
12
Show member pathways
12.17 IL3RA IL3 IL2RB IL2RA IL2
13
Show member pathways
12.09 ITGAX IL3RA IL2RB IL2RA CCND1 BRAF
14
Show member pathways
12.07 IL2RB IL2RA IL2
15
Show member pathways
11.87 IL2RB IL2RA IL2
16 11.84 IL3 IFNB1 BRAF
17 11.77 IFNB1 IFNA2 IFNA1
18
Show member pathways
11.77 IL2RA IL3 IL3RA
19 11.7 IL2RB IL2RA IL2
20 11.64 ITGAX IL3 IL2 BRAF
21
Show member pathways
11.6 IL3 IL2RA IL2
22 11.59 ITGAX IL3RA IL2 IFNB1 IFNA2
23 11.5 IL2 IL2RA IL2RB
24 11.41 IL2RB IL2RA IL2
25 11.4 CD22 FCRL5 IGHV4-34 IL2 ITGAX
26 11.34 ITGAX IL3RA IL3 IL2RB IL2RA IL2
27
Show member pathways
11.26 IL2RB IL2RA IL2
28 11.2 IL2 IL2RA IL2RB
29
Show member pathways
11.13 IL2 IL2RA IL2RB
30 11.13 IL3 IL2 IFNB1
31 10.61 IL2RA IL2
32 10.61 IL2RA IL2

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 external side of plasma membrane GO:0009897 9.53 ITGAX IL3RA IL2RB IL2RA IGHV4-34 CD5
2 interleukin-2 receptor complex GO:0005893 9.26 IL2RB IL2RA

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

(show all 20)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of inflammatory response GO:0050728 10.1 IL2RA IL2 ADA ACP5
2 humoral immune response GO:0006959 10.07 IFNB1 IFNA2 IFNA1
3 type I interferon-mediated signaling pathway GO:0060337 10.01 IFNA1 IFNA2 IFNB1
4 response to exogenous dsRNA GO:0043330 9.95 IFNB1 IFNA2 IFNA1
5 activated T cell proliferation GO:0050798 9.88 IL2RA IL2
6 natural killer cell activation GO:0030101 9.88 IL2RB IL2 IFNB1
7 negative regulation of T cell differentiation GO:0045581 9.86 IFNB1 IFNA2
8 T cell activation involved in immune response GO:0002286 9.85 IFNB1 IFNA2 IFNA1
9 interleukin-2-mediated signaling pathway GO:0038110 9.83 IL2RB IL2RA
10 positive regulation of T cell differentiation GO:0045582 9.81 IL2RA IL2 ADA
11 negative regulation of T-helper 2 cell cytokine production GO:2000552 9.76 IFNA2 IFNB1
12 alpha-beta T cell differentiation GO:0046632 9.75 BRAF ADA
13 regulation of T cell homeostatic proliferation GO:0046013 9.73 IL2RA IL2
14 positive regulation of peptidyl-serine phosphorylation of STAT protein GO:0033141 9.73 IFNA1 IFNA2 IFNB1
15 negative regulation of lymphocyte proliferation GO:0050672 9.72 IL2RA IL2
16 cytokine-mediated signaling pathway GO:0019221 9.65 IL3RA IL2RB IFNB1 IFNA2 IFNA1
17 natural killer cell activation involved in immune response GO:0002323 9.63 IFNB1 IFNA2 IFNA1
18 regulation of CD4-positive, alpha-beta T cell proliferation GO:2000561 9.62 IL2RA IL2
19 lymphocyte proliferation GO:0046651 9.57 IL2RA IL2
20 B cell proliferation GO:0042100 9.23 IFNB1 IFNA2 IFNA1 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 10.02 IL3 IL2 IFNB1 IFNA2 IFNA1
2 interleukin-2 binding GO:0019976 9.56 IL2RB IL2RA
3 interleukin-2 receptor activity GO:0004911 9.46 IL2RB IL2RA
4 cytokine receptor binding GO:0005126 9.26 IFNB1 IFNA2 IFNA1
5 type I interferon receptor binding GO:0005132 9.1 IFNB1 IFNA2 IFNA1

Sources for Hairy Cell Leukemia

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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