HCL
MCID: HRY003
MIFTS: 55

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 74 52 36 29 15 17 71
Hairy Cell Leukemia Variant 12 58 71
Hcl-V 12 58 15
Prolymphocytic Variant of Hairy Cell Leukemia 58
Leukemic Reticuloendotheliosis Variant 58
Leukemic Reticuloendotheliosis 52
Prolymphocytic Variant of Hcl 58
Hairy Cell Leukaemia Variant 12
Leukemia, Hairy Cell 43
Leukemia Hairy Cell 54
Hcl 52

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 43 D007943
NCIt 49 C7401 C7402
SNOMED-CT 67 277568007 54087003
ICD10 32 C91.4
ICD10 via Orphanet 33 C91.4
UMLS via Orphanet 72 C0349633
Orphanet 58 ORPHA300878
UMLS 71 C0023443 C0349633

Summaries for Hairy Cell Leukemia

NIH Rare Diseases : 52 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 Cefepime and Levofloxacin have been mentioned in the context of this disorder. Affiliated tissues include b cells, bone and bone marrow, 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 : 74 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 679)
# Related Disease Score Top Affiliating Genes
1 refractory hairy cell leukemia 34.6 IL2RA CD22 BRAF
2 neutropenia 31.7 IL3 IL2 IFNA2 IFNA1
3 herpes zoster 31.0 IL2 IFNA2 IFNA1
4 hemolytic anemia 31.0 IL3 IL2 IFNA2 ADA
5 capillary leak syndrome 30.9 IL3RA IL2 CD22
6 t-cell lymphoblastic leukemia/lymphoma 30.9 IL3 IL2RA IL2 CCND1 ADA
7 adult t-cell leukemia 30.8 IL2RA IL2 IFNA1
8 mastocytosis 30.8 IL3 IL2RA IFNA2 IFNA1
9 thrombocytopenia 30.7 U2AF1 IL3 IL2 IFNA2 IFNA1
10 pancytopenia 30.6 U2AF1 IL3 IL2RA CD5
11 prolymphocytic leukemia 30.6 IL2RA CD5 CCND1 ADA
12 red cell aplasia 30.6 IL2RA IL2 IFNA1
13 pure red-cell aplasia 30.6 IL2RA IL2 IFNA1
14 mycosis fungoides 30.6 ITGAE IL2RA IL2 IFNA2 IFNA1 ADA
15 polycythemia vera 30.5 U2AF1 IL3 IFNA2 IFNA1
16 acute leukemia 30.5 IL3RA IL3 IL2 CD22
17 diarrhea 30.5 IL3 IL2 IFNA2 IFNA1
18 stomatitis 30.5 IL2 IFNA2 IFNA1
19 mucositis 30.5 IL3 IL2 IFNA1
20 myelofibrosis 30.5 U2AF1 IL3 IL2RA IFNA2 IFNA1 ACP5
21 acquired immunodeficiency syndrome 30.4 IL2RA IL2 IFNA2 IFNA1 CCND1 ADA
22 histiocytosis 30.4 U2AF1 ITGAX BRAF
23 pulmonary tuberculosis 30.4 IL2RA IL2 ADA
24 cutaneous t cell lymphoma 30.3 ITGAE IL2RA IL2 IFNA2 IFNA1 ADA
25 lymphoma 30.3 IL2RA IL2 FCRL5 CD5 CCND1 BRAF
26 viral infectious disease 30.3 IL2RA IL2 IFNA2 IFNA1
27 aplastic anemia 30.3 U2AF1 IL3 IL2RA IL2 IFNA1
28 mantle cell lymphoma 30.3 ITGAX CD5 CD200 CCND1
29 b cell prolymphocytic leukemia 30.3 IL2RA CD5
30 langerhans cell histiocytosis 30.3 U2AF1 ITGAX BRAF
31 cryoglobulinemia 30.2 IFNA2 IFNA1 CD5
32 thrombocytosis 30.2 U2AF1 IL3 IFNA1
33 hypereosinophilic syndrome 30.1 IL3 IL2RA IL2 IFNA2 IFNA1
34 renal cell carcinoma, nonpapillary 30.1 IL2RA IL2 IFNA2 IFNA1 CCND1 BRAF
35 cll/sll 30.1 CD5 CD22 CCND1
36 marginal zone b-cell lymphoma 30.0 U2AF1 CD5 CD22 CCND1
37 essential thrombocythemia 29.9 U2AF1 IL3 IFNA2 IFNA1 CCND1
38 diphtheria 29.9 ITGAX IL3RA IL3 IL2RA IL2 IFNA1
39 leukemia, chronic myeloid 29.9 U2AF1 IL3 IFNA2 IFNA1 CCND1 ADA
40 blood platelet disease 29.9 U2AF1 IL3 IL2 IFNA1
41 cryoglobulinemia, familial mixed 29.9 IFNA2 IFNA1 CD5
42 plasmacytoma 29.8 IL2 CD5 CCND1
43 myeloproliferative neoplasm 29.8 U2AF1 IL3 IFNA2 IFNA1
44 sezary's disease 29.7 IL2RA IL2 IFNA2 IFNA1 CD5
45 immune deficiency disease 29.7 IL2RA IL2 IFNA1 CD5 ADA
46 systemic mastocytosis 29.6 U2AF1 IL3RA IL2RA IFNA2 IFNA1
47 thymoma 29.5 IL2 IFNA1 CD5 CCND1
48 acute promyelocytic leukemia 29.4 U2AF1 ITGAX IL3 IFNA1 CCND1
49 lymphoma, mucosa-associated lymphoid type 29.3 CD5 CD22 CCND1
50 mature b-cell neoplasm 29.2 U2AF1 IL2 CD5 CD22 CCND1

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:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.73 ADA ANXA1 BRAF CCND1 CD200 CD22
2 immune system MP:0005387 9.4 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 278)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cefepime Approved, Investigational Phase 3 88040-23-7 5479537
2
Levofloxacin Approved, Investigational Phase 3 100986-85-4 149096
3
Ofloxacin Approved Phase 3 82419-36-1 4583
4
Ketamine Approved, Vet_approved Phase 3 6740-88-1 3821
5
Ondansetron Approved Phase 3 99614-02-5 4595
6
Iron Approved, Experimental Phase 3 15438-31-0, 7439-89-6 27284 23925
7
Dalteparin Approved Phase 3 9005-49-6
8
Tinzaparin Approved Phase 3 9041-08-1, 9005-49-6 25244225
9
Dextromethorphan Approved Phase 3 125-71-3 5360696 5362449
10
Darbepoetin alfa Approved, Investigational Phase 3 11096-26-7, 209810-58-2
11
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
12
Caspofungin Approved Phase 3 162808-62-0, 179463-17-3 2826718 468682
13
Tazobactam Approved Phase 3 89786-04-9 123630
14
Vancomycin Approved Phase 3 1404-90-6 14969 441141
15
Piperacillin Approved Phase 3 66258-76-2 43672
16
Amphotericin B Approved, Investigational Phase 3 1397-89-3 5280965 14956
17
Itraconazole Approved, Investigational Phase 3 84625-61-6 55283
18
Captopril Approved Phase 3 62571-86-2 44093
19
Fluconazole Approved, Investigational Phase 3 86386-73-4 3365
20
Ribavirin Approved Phase 3 36791-04-5 37542
21
Palivizumab Approved, Investigational Phase 3 188039-54-5
22
Acyclovir Approved Phase 3 59277-89-3 2022
23
Cladribine Approved, Investigational Phase 2, Phase 3 4291-63-8 20279
24
Estradiol Approved, Investigational, Vet_approved Phase 3 50-28-2 5757
25
Norgestimate Approved, Investigational Phase 3 35189-28-7 6540478
26
Moxifloxacin Approved, Investigational Phase 3 354812-41-2, 151096-09-2 152946
27
Ethinyl Estradiol Approved Phase 3 57-63-6 5991
28
Polyestradiol phosphate Approved Phase 3 28014-46-2
29
St. John's Wort Approved, Investigational, Nutraceutical Phase 3 84082-80-4
30
Ginseng Approved, Investigational, Nutraceutical Phase 3 50647-08-0
31 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
32 Anesthetics, Dissociative Phase 3
33 Immunoglobulins Phase 3
34 Antibodies Phase 3
35 Antibodies, Monoclonal Phase 3
36 Cola Phase 3
37 Neurotransmitter Agents Phase 3
38 Analgesics, Opioid Phase 3
39 Psychotropic Drugs Phase 3
40 Anti-Anxiety Agents Phase 3
41 Antipsychotic Agents Phase 3
42 Liver Extracts Phase 3
43 Heparin, Low-Molecular-Weight Phase 3
44 Excitatory Amino Acid Antagonists Phase 3
45 Narcotics Phase 3
46 Analgesics Phase 3
47 Hematinics Phase 3
48 Cytochrome P-450 Enzyme Inhibitors Phase 3
49 Antiparasitic Agents Phase 3
50 Antiprotozoal Agents Phase 3

Interventional clinical trials:

(show top 50) (show all 231)
# Name Status NCT ID Phase Drugs
1 Multicenter, Double-Blind, Randomized Study to Compare the Safety and Efficacy of Levofloxacin With That of Cefepime in the Treatment of Fever and Neutropenia - Phase IIIB Unknown status NCT00020865 Phase 3 cefepime hydrochloride;levofloxacin
2 A Randomized Double-Blind Controlled Trial of Ketamine Versus Placebo in Conjunction With Best Pain Management in Neuropathic Pain in Cancer Patients Unknown status NCT01316744 Phase 3 ketamine hydrochloride
3 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
4 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
5 INSPIRE: An Internet-based RCT for Long-term Survivors of Hematopoietic Stem Cell Transplantation Completed NCT00799461 Phase 3
6 Phase III, Randomized, Double-Blind, Placebo-Controlled Crossover Trial of Ondansetron in the Control of Chronic Nausea and Vomiting Not Due to Antineoplastic Therapy in Patients With Advanced Cancer Completed NCT00006348 Phase 3 ondansetron
7 A Phase III Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Effects of Fragmin (5,000 IU Subcutaneously) in Preventing Catheter-Related Complications When Given Daily to Cancer Patients With Central Venous Catheters Completed NCT00006083 Phase 3 Fragmin
8 A Phase III Double-Blind Equivalence Study of Two Different Formulations of Slow-Release Morphine Followed by a Randomization Between Dextromethorphan or Placebo Plus Statex SR for Chronic Cancer Pain Relief in Terminally Ill Patients Completed NCT00003687 Phase 3 dextromethorphan hydrobromide;morphine sulfate
9 Does Hypericum Reduce Fatigue in Cancer Patients on Chemotherapy? A Randomized, Double-Blind, Placebo-Controlled Clinical Trial Completed NCT00005805 Phase 3
10 A Phase III, Randomized Study of the Effects of Parenteral Iron, Oral Iron, or No Iron Supplementation on the Erythropoietic Response to Darbepoetin Alfa for Cancer Patients With Chemotherapy-Associated Anemia Completed NCT00661999 Phase 3 sodium ferric gluconate complex in sucrose
11 Preparatory Aid to Improve Decision Making About Cancer Clinical Trials (PRE-ACT) Completed NCT00750009 Phase 3
12 Vancomycin Versus Placebo in Persistently Febrile Granulocytopenic Patients Given Piperacillin/Tazobactam Completed NCT00003805 Phase 3 piperacillin sodium;piperacillin-tazobactam;tazobactam sodium;vancomycin
13 A Strategic Study to Determine the Optimal Moment to Initiate Systemic Antifungal Therapy With Ambisome in Granulocytopenic Cancer Patients With Unexplained Fever Refractory to Empirical Antibacterials Completed NCT00003938 Phase 3 liposomal amphotericin B
14 A Multicenter, Double-Blind, Randomized, Comparative Study To Evaluate The Safety, Tolerability, And Efficacy Of MK-0991 Versus (Amphotericin B) Liposome For Injection As Empirical Therapy In Patients With Persistent Fever And Neutropenia Completed NCT00008359 Phase 3 caspofungin acetate;liposomal amphotericin B
15 The Use of American Ginseng (Panax Quinquefolius) to Improve Cancer-Related Fatigue: A Randomized, Double-Blind, Placebo-Controlled Phase III Study Completed NCT00719563 Phase 3 American ginseng
16 Phase III Randomized Trial of an Opioid Titration Order Sheet Compared to Standard of Care in Patients With Cancer Related Pain. Completed NCT00666211 Phase 3
17 Phase III Study of Captopril in Patients Undergoing Autologous Bone Marrow/Stem Cell Transplantation Completed NCT00004230 Phase 3 captopril;cyclophosphamide
18 A Randomized, Comparative Study of Itraconazole Versus Fluconazole for Prevention of Aspergillus Infections in Peripheral Blood Stem Cell and Marrow Transplant Recipients Completed NCT00003883 Phase 3 fluconazole;itraconazole
19 A Randomized Double-Blind Placebo-Controlled Phase III Study To Evaluate The Safety And Efficacy Of Palivizumab Combined With Aerosolized Ribavirin Compared To Ribavirin Alone To Treat RSV Pneumonia In Patients With Bone Marrow Transplants (BMT) Completed NCT00014391 Phase 3 ribavirin
20 A Phase III Multicenter Study of Cytomegalovirus Prophylaxis With Valacyclovir for the Prevention of Serious Fungal and Bacterial Infections Among Cytomegalovirus Seronegative Recipients of Cytomegalovirus Seropositive Sx Stem Cell Transplants Completed NCT00045292 Phase 3 acyclovir;acyclovir sodium;valacyclovir
21 Music Video and Adolescent/Young Adult Resilience During Transplant Completed NCT00305851 Phase 3
22 A Randomized, Double-blind, Placebo-controlled Multi-center Study to Evaluate the Safety and Efficacy of Fentanyl Sublingual Spray (Fentanyl SL Spray) for the Treatment of Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
23 Therapy Optimisation for the Treatment of Hairy Cell Leukemia Recruiting NCT02131753 Phase 2, Phase 3 Cladribine s.c. injection, HCL treatment
24 Randomized, Double Blinded, Placebo-Controlled Trial of Antibacterial Prophylaxis for the Prevention of Bacterial Infections in the Post-Engraftment Phase After Allogeneic Hematopoeitic Stem Cell Transplantation Terminated NCT00324324 Phase 3 moxifloxacin hydrochloride;Placebo
25 A Multiinstitutional Trial To Evaluate The Prophylactic Use Of NASA-Developed Light Emitting Diodes For The Prevention Of Oral Mucositis In Bone Marrow Transplant Patients Unknown status NCT00036712 Phase 2
26 Study on the Treatment of Hairy Cell Leukaemia Variant and Relapsing Hairy Cell Leukaemia Completed NCT02157181 Phase 2 2CdA +/- Rituximab
27 Rituximab (IDEC-C2B8, Mabthera) in Patients With Hairy Cell Leukemia Relapsing After Treatment With 2-Chlorodeoxyadenosine (CDA) Completed NCT00003757 Phase 2
28 Phase II Trial Of BL22 Immunotoxin In Hairy Cell Leukemia Completed NCT00074048 Phase 2 BL22
29 A Phase II Efficacy Study of Roferon-A in Hairy Cell Leukemia Completed NCT00001567 Phase 2 Roferon-A
30 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
31 Phase II Study of MK-2206 in Patients With Relapsed Lymphoma Completed NCT01258998 Phase 2 Akt inhibitor MK2206
32 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
33 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
34 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
35 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
36 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
37 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
38 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
39 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
40 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
41 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
42 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
43 Adoptive Immunotherapy of Epstein Barr Virus Induced Lymhoproliferative Disease. A Comparison of Allogeneic and Autologous Lymphocyte Responses ex Vivo and Use of Highly Selected Reactive Cells as an Alternative to Chemotherapy in Vivo. Completed NCT00005606 Phase 2
44 OUTPATIENT SUBCUTANEOUS IL-2 AND ALPHA INTERFERON IN THE MANAGEMENT OF METASTATIC CANCER Completed NCT00002504 Phase 2
45 The Effect of Cyproheptadine Hydrochloride (Periactin) and Megestrol Acetate (Megace) on Weight in Children With Cancer/Treatment Related Cachexia Completed NCT00066248 Phase 2 cyproheptadine hydrochloride;megestrol acetate
46 RC05CB A Pilot, Randomized Comparison of Standard Weekly Epoetin Alfa to Every-3-Week-Epoetin Alfa and Every 3-Week Darbepoetin Alfa Completed NCT00416624 Phase 2 darbepoetin alfa;epoetin alfa
47 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
48 Donor NK Cell Infusion for Progression/Recurrence of Underlying Malignant Disorders After HLA-haploidentical HCT - a Phase 1-2 Study Completed NCT00823524 Phase 1, Phase 2
49 Treatment of Patients With Hematological Malignancies Using Marrow Transplantation From Unrelated Donors Incompatible for One HLA Locus Antigen Completed NCT00005804 Phase 2 cyclophosphamide
50 A Phase I-II Intensive-Dose Ifosfamide, Carboplatin and Taxotere (IC-T) Combination Chemotherapy Followed by Autologous Stem Cell Rescue for Patients With Refractory Malignancies Completed NCT00003406 Phase 1, Phase 2 carboplatin;docetaxel;ifosfamide

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 29

Anatomical Context for Hairy Cell Leukemia

MalaCards organs/tissues related to Hairy Cell Leukemia:

40
B Cells, Bone, Bone Marrow, T Cells, Spleen, Myeloid, Lymph Node

Publications for Hairy Cell Leukemia

Articles related to Hairy Cell Leukemia:

(show top 50) (show all 3211)
# 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. 54 61
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. 54 61
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. 61 54
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. 61 54
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. 61 54
15621813 2005
12
Immunoglobulin superfamily receptor translocation associated 2 protein on lymphoma cell lines and hairy cell leukemia cells detected by novel monoclonal antibodies. 54 61
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. 54 61
11755467 2002
19
Quantification of the leukocyte common antigen (CD45) in mature B-cell malignancies. 61 54
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. 54 61
11161227 2001
24
Immunohistochemical detection of cyclin D1 using optimized conditions is highly specific for mantle cell lymphoma and hairy cell leukemia. 54 61
11144927 2000
25
Biology of tartrate-resistant acid phosphatase. 54 61
11342331 2000
26
Reconstitution of endogenous interferon a by recombinant interferon in hairy cell leukemia. 61 54
11034083 2000
27
Induction of apoptosis in vivo and in vitro in hairy cell leukemia treated by deoxycoformycin. 54 61
11128872 2000
28
B7 costimulatory molecules from malignant cells in patients with b-cell chronic lymphoproliferative disorders trigger t-cell proliferation. 61 54
11002221 2000
29
Prolonged interferon-alpha-2b treatment of hairy cell leukemia patients. 54 61
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. 61 54
10456674 1999
33
The chemokine receptor CXCR3 is expressed on malignant B cells and mediates chemotaxis. 54 61
10393705 1999
34
Long term outcome of patients with hairy cell leukemia treated with pentostatin. 54 61
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. 61 54
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. 54 61
9291460 1997
41
Retrospective analysis of 34 cases of hairy cell leukemia treated with interferon-alpha and/or 2-chlorodeoxyadenosine. 61 54
9299866 1997
42
Blastic transformation of hairy cell leukemia. 54 61
9240906 1997
43
Hypertriglyceridemia during long-term interferon-alpha therapy in a series of hematologic patients. 54 61
9181461 1997
44
Characterization of monoclonal antibodies specific to human tartrate-resistant acid phosphatase. 54 61
9145320 1997
45
CD30 ligand is frequently expressed in human hematopoietic malignancies of myeloid and lymphoid origin. 61 54
9058727 1997
46
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
47
Response duration and recovery of CD4+ lymphocytes following deoxycoformycin in interferon-alpha-resistant hairy cell leukemia: 7-year follow-up. 61 54
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. 54 61
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. 61 54
8840984 1996

Variations for Hairy Cell Leukemia

Cosmic variations for Hairy Cell Leukemia:

9
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM87898723 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,mantle cell lymphoma c.817C>T p.R273C 17:7673803-7673803 4

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
12.72 IL2RA IL2 IFNA2 IFNA1 CCND1
9
Show member pathways
12.68 IL3 IL2RA IL2 IFNA2 IFNA1 CCND1
10 12.48 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
Show member pathways
12.2 IL3RA IL3 IL2RA IL2 IFNA2 IFNA1
14 12.14 ITGAX ITGAE IL3RA IL2RA
15
Show member pathways
12.14 IL3 IL2RA IL2 IFNA2 IFNA1
16
Show member pathways
12.07 ITGAX ITGAE IL3RA IL2RA CCND1 BRAF
17
Show member pathways
12.03 IL3RA IL3 IL2RA IL2
18
Show member pathways
12 IL2RA IL2 BRAF
19 11.76 IL3RA IL3 IL2RA CD5 CD22
20 11.69 ITGAE IL2RA IL2
21
Show member pathways
11.66 IL3 IL2RA IL2
22 11.6 ITGAX ITGAE IL3 IL2 BRAF
23
Show member pathways
11.53 IL2RA IL2 BRAF
24 11.29 ITGAX IL3RA IL3 IL2RA IL2 IFNA2
25 11.22 ITGAX ITGAE IL3RA 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 14)
# Name GO ID Score Top Affiliating Genes
1 adaptive immune response GO:0002250 9.85 IL2 IGHV4-34 IFNA2 IFNA1 ANXA1
2 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.67 IL3 IL2 IFNA2
3 negative regulation of inflammatory response GO:0050728 9.62 IL2RA IL2 ADA ACP5
4 positive regulation of T cell proliferation GO:0042102 9.61 IL2RA IL2 ANXA1
5 MAPK cascade GO:0000165 9.55 IL3RA IL3 IL2RA IL2 BRAF
6 regulation of regulatory T cell differentiation GO:0045589 9.54 IL2RA IL2
7 regulation of T cell differentiation GO:0045580 9.52 BRAF ADA
8 interleukin-2-mediated signaling pathway GO:0038110 9.51 IL2RA IL2
9 response to vitamin E GO:0033197 9.48 CCND1 ADA
10 alpha-beta T cell differentiation GO:0046632 9.4 BRAF ANXA1
11 negative regulation of lymphocyte proliferation GO:0050672 9.37 IL2RA IL2
12 cytokine-mediated signaling pathway GO:0019221 9.28 ITGAX IL3RA IL3 IL2RA IL2 IFNA2
13 regulation of T cell homeostatic proliferation GO:0046013 9.26 IL2RA IL2
14 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 type I interferon receptor binding GO:0005132 8.96 IFNA2 IFNA1
2 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
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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