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MCID: LYM033
MIFTS: 58

Lymphoproliferative Syndrome

Categories: Blood diseases, Cancer diseases, Genetic diseases, Immune diseases, Infectious diseases, Nephrological diseases, Rare diseases
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Aliases & Classifications for Lymphoproliferative Syndrome

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MalaCards integrated aliases for Lymphoproliferative Syndrome:

Name: Lymphoproliferative Syndrome 11 58 53 14
Lymphoproliferative Disorders 53 71 75
Lymphoproliferative Disorder 28 5 14
Lymphoproliferative Disorders, Susceptibility to 28

Classifications:

MalaCards categories:
Global: Rare diseases Genetic diseases Cancer diseases Infectious diseases
Anatomical: Blood diseases Immune diseases Nephrological diseases
See all MalaCards categories (disease lists)
Orphanet: 58  
Rare immunological diseases


External Ids:

Disease Ontology 11 DOID:0060704
UMLS via Orphanet 72 C0024314
Orphanet 58 ORPHA238510
UMLS 71 C0024314
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Summaries for Lymphoproliferative Syndrome

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Disease Ontology: 11 A primary immunodeficiency disease characterized by immune dysregulation typically after viral infection, usually associated with Epstein-Barr viral infection.

MalaCards based summary: Lymphoproliferative Syndrome, also known as lymphoproliferative disorders, is related to lymphoproliferative syndrome 1 and autoimmune lymphoproliferative syndrome, type iii. An important gene associated with Lymphoproliferative Syndrome is SH2D1A (SH2 Domain Containing 1A), and among its related pathways/superpathways are Innate Immune System and ERK Signaling. The drugs Basiliximab and Epoetin Alfa have been mentioned in the context of this disorder. Affiliated tissues include bone marrow, t cells and lymph node, and related phenotypes are Reduced mammosphere formation and immune system

Wikipedia: 75 Lymphoproliferative disorders (LPDs) refer to a specific class of diagnoses, comprising a group of... more...
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Related Diseases for Lymphoproliferative Syndrome

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Diseases in the Lymphoproliferative Syndrome family:

Autoimmune Lymphoproliferative Syndrome Autoimmune Lymphoproliferative Syndrome, Type Iia
Lymphoproliferative Syndrome 1 Lymphoproliferative Syndrome 2
Autoimmune Lymphoproliferative Syndrome, Type Iii Lymphoproliferative Syndrome 3

Diseases related to Lymphoproliferative Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 916)
# Related Disease Score Top Affiliating Genes
1 lymphoproliferative syndrome 1 33.1 XIAP PRF1 ITK
2 autoimmune lymphoproliferative syndrome, type iii 33.1 PRKCD CASP10
3 caspase 8 deficiency 33.1 FADD CASP8 CASP10
4 lymphoproliferative syndrome, x-linked, 2 33.0 XIAP SLAMF1 SH2D1A ITK
5 lymphoproliferative syndrome 2 32.9 XIAP SLAMF1 SH2D1A ITK CD27
6 ras-associated autoimmune leukoproliferative disorder 32.9 SH2D1A PRKCD NRAS FASLG FAS CASP8
7 autoimmune lymphoproliferative syndrome 32.8 XIAP SH2D1A PRKCD PRF1 NRAS ITK
8 systemic epstein-barr virus positive t-cell lymphoma of childhood 32.3 TNFRSF8 SH2D1A
9 lymphoproliferative syndrome, x-linked, 1 32.2 XIAP TNFRSF8 SLAMF1 SH2D1A PTPN11 PRF1
10 chediak-higashi syndrome 32.0 SH2D1A PRF1 FASLG
11 pfeiffer syndrome 30.9 TNFRSF8 SLAMF1 SH2D1A PTPN11 PRF1 CD27
12 hemophagocytic lymphohistiocytosis 30.9 XIAP SLAMF1 SH2D1A PRF1 ITK CD27
13 leukemia, acute lymphoblastic 30.9 XIAP PTPN11 FADD CD5 CASP8
14 peripheral t-cell lymphoma 30.8 TNFRSF8 ITK CD5
15 aplastic anemia 30.8 SH2D1A PRF1 FASLG FAS
16 myeloma, multiple 30.8 XIAP TNFRSF8 PTPN11 NRAS FASLG FAS
17 primary cutaneous t-cell non-hodgkin lymphoma 30.8 TNFRSF8 FAS CD5
18 immune deficiency disease 30.7 TNFRSF8 SLAMF1 SH2D1A FASLG FAS CD5
19 common variable immunodeficiency 30.7 XIAP TNFRSF8 SH2D1A PRKCD FAS CD27
20 myelodysplastic syndrome 30.6 XIAP PTPN11 NRAS FASLG FAS
21 b-cell lymphoma 30.6 XIAP TNFRSF8 FAS CD5 CASP8
22 burkitt lymphoma 30.6 TNFRSF8 SH2D1A FAS CASP8
23 hepatosplenic t-cell lymphoma 30.5 TNFRSF8 CD5
24 aggressive nk-cell leukemia 30.5 TNFRSF8 PRF1 CD5
25 lymphopenia 30.5 ITK FASLG FAS
26 composite lymphoma 30.5 TNFRSF8 CD5
27 lymphoma 30.4 XIAP TNFRSF8 SH2D1A PTPN11 ITK FAS
28 high grade glioma 30.4 XIAP FASLG FAS CASP8
29 pancytopenia 30.4 XIAP SH2D1A PRF1 ITK CD5
30 neuroblastoma 30.3 XIAP PTPN11 NRAS FASLG FAS CASP8
31 dysgammaglobulinemia 30.3 XIAP SLAMF1 SH2D1A LY9 CD244
32 combined immunodeficiency 30.2 SH2D1A PRF1 PLAA ITK CD27
33 lung lymphoma 30.2 TNFRSF8 CD5
34 breast implant-associated anaplastic large cell lymphoma 30.2 TNFRSF8 CD5
35 b cell deficiency 30.2 SH2D1A PRKCD ITK CD27
36 colon adenocarcinoma 30.1 XIAP FASLG FAS CASP8
37 parapsoriasis 30.0 TNFRSF8 CD5
38 bone lymphoma 30.0 TNFRSF8 CD5
39 hashimoto thyroiditis 30.0 NRAS FASLG FAS
40 follicular mucinosis 30.0 TNFRSF8 CD5
41 lymphoma, non-hodgkin, familial 30.0 XIAP TNFRSF8 SH2D1A PRF1 NRAS FAS
42 primary cutaneous gamma-delta t-cell lymphoma 30.0 TNFRSF8 PRF1 CD5
43 lethal midline granuloma 30.0 TNFRSF8 PRF1 CD5
44 breast lymphoma 30.0 TNFRSF8 CD5
45 angioimmunoblastic t-cell lymphoma 29.9 TNFRSF8 ITK CD5
46 brain cancer 29.9 PTPN11 NRAS FASLG CASP8
47 childhood lymphoma 29.8 TNFRSF8 CD5
48 pleural empyema 29.8 TNFRSF8 CD5
49 richter's syndrome 29.8 TNFRSF8 CD5
50 mediastinal cancer 29.8 TNFRSF8 CD5

Graphical network of the top 20 diseases related to Lymphoproliferative Syndrome:



Diseases related to Lymphoproliferative Syndrome
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Symptoms & Phenotypes for Lymphoproliferative Syndrome

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GenomeRNAi Phenotypes related to Lymphoproliferative Syndrome according to GeneCards Suite gene sharing:

25
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 no effect GR00402-S-1 10.17 CASP10 CASP8 CD244 CD27 CD5 FADD
2 no effect GR00402-S-2 10.17 CASP10 CASP8 CD27 CD5 FADD FAS
3 Reduced mammosphere formation GR00396-S 9.56 CD27 CD5 FADD FAS FASLG ITK

MGI Mouse Phenotypes related to Lymphoproliferative Syndrome:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 10.11 CASP8 CD244 CD27 CD5 FADD FAS
2 endocrine/exocrine gland MP:0005379 10.1 CASP8 FADD FAS FASLG INPP5D ITK
3 cellular MP:0005384 10.1 CASP8 CD27 FADD FAS FASLG INPP5D
4 muscle MP:0005369 10.01 CASP8 FADD FAS INPP5D PLAA PRKCD
5 neoplasm MP:0002006 9.98 CASP8 FAS FASLG INPP5D NRAS PRF1
6 liver/biliary system MP:0005370 9.97 CASP8 FADD FAS FASLG NRAS PRF1
7 hematopoietic system MP:0005397 9.91 CASP8 CD244 CD27 CD5 FADD FAS
8 integument MP:0010771 9.32 CASP8 CD5 FADD FAS FASLG INPP5D
Sources

Drugs & Therapeutics for Lymphoproliferative Syndrome

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Drugs for Lymphoproliferative Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 160)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Basiliximab Approved, Investigational Phase 4 179045-86-4
2
Epoetin Alfa Phase 4
3 Hematinics Phase 4
4
Amitriptyline Approved Phase 3 50-48-6 2160
5
Baclofen Approved Phase 3 1134-47-0 2284
6
Ketamine Approved, Vet_approved Phase 3 6740-88-1, 1867-66-9 3821
7
Darbepoetin alfa Approved, Investigational Phase 3 209810-58-2
8
Pancrelipase Approved, Investigational Phase 3 53608-75-6 8519
9
Everolimus Approved Phase 3 159351-69-6 70789204 6442177
10
Busulfan Approved, Investigational Phase 2, Phase 3 55-98-1 2478
11
D-Tyrosine Approved, Experimental, Investigational, Nutraceutical Phase 3 133585-56-5, 60-18-4, 556-02-5 1153 6057
12 Ferric Oxide, Saccharated Phase 3
13 Adrenergic Agents Phase 3
14 Excitatory Amino Acid Antagonists Phase 3
15 Antidepressive Agents, Tricyclic Phase 3
16 Antidepressive Agents Phase 3
17 Anesthetics, Dissociative Phase 3
18 GABA Agonists Phase 3
19 Analgesics, Non-Narcotic Phase 3
20 Iron Supplement Phase 3
21 Ferric gluconate Phase 3
22 Adjuvants, Immunologic Phase 2, Phase 3
23 Liver Extracts Phase 3
24 Pancreatin Phase 3
25 polysaccharide-K Phase 3
26 Thymoglobulin Phase 2, Phase 3
27
Ginseng Investigational, Nutraceutical Phase 3 50647-08-0
28
Cisplatin Approved Phase 2 15663-27-1 2767 5702198 441203
29
Gemcitabine Approved Phase 2 95058-81-4, 122111-03-9 60750
30
Valproic acid Approved, Investigational Phase 1, Phase 2 99-66-1 3121
31
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
32
Prednisolone acetate Approved, Vet_approved Phase 2 52-21-1
33
Prednisolone Approved, Vet_approved Phase 2 50-24-8 4894 5755
34
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5 1875
35
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 4159 6741
36
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3 3680
37
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 3003 5743
38
Cytarabine Approved, Investigational Phase 2 147-94-4 6253
39
Oxaliplatin Approved, Investigational Phase 2 61825-94-3 43805 11947679 6857599
40
Vorinostat Approved, Investigational Phase 2 149647-78-9 5311
41
Lenalidomide Approved Phase 1, Phase 2 191732-72-6 216326
42
Panobinostat Approved, Investigational Phase 1, Phase 2 404950-80-7 6918837
43
Mechlorethamine Approved, Investigational Phase 2 51-75-2 4033
44
Melphalan Approved Phase 2 148-82-3 4053 460612
45
Nivolumab Approved Phase 2 946414-94-4
46
Coenzyme M Approved, Investigational Phase 2 3375-50-6 598 23662354
47
Acalabrutinib Approved, Investigational Phase 2 1420477-60-6 71226663 71226662
48
Pembrolizumab Approved Phase 2 1374853-91-4 254741536
49
Etoposide Approved Phase 2 33419-42-0 36462
50
Leflunomide Approved, Investigational Phase 2 75706-12-6 3899

Interventional clinical trials:

(show top 50) (show all 214)
# Name Status NCT ID Phase Drugs
1 Evaluation of the Immune Response After Vaccination Against Hepatitis B in Patients With Indolent Lymphoproliferative Disorders With no Treatment. Unknown status NCT01698866 Phase 4
2 Quality of Life Related Response to Treatment in Anemic Cancer Patients Receiving Recormon and Efficacy of the Drug Dosage 30,000 IU Once Weekly in Patients With Lymphoproliferative Disorders Completed NCT00776425 Phase 4 Epoetin beta
3 Fludarabine, Cyclophosphamide, Doxorubicin and Rituximab for the Treatment of Post-transplant Lymphoproliferative Disease (PTLD) Completed NCT01088724 Phase 4 fludarabine, cyclophosphamide, doxorubicin, rituximab
4 Multicenter Randomized Two Arms Study Evaluating the Efficacy of Prophylactic Rituximab in Adult EBV Negative Kidney Transplant Recipients on Incidence of EBV Primary Infection and Post-transplant Lymphoproliferative Disorders Recruiting NCT04989491 Phase 4 Rituximab
5 Cytotoxic T Cell Therapy for Post Transplant Lymphoproliferative Disease: Randomized Controlled Trial in Transplant Recipients Unknown status NCT00033475 Phase 3
6 Evaluation of Plasmatic Levels of Busulfan in Patients Undergoing Hematopoietic Stem Cell Transplantation Unknown status NCT01800643 Phase 2, Phase 3
7 Phase 3 Study of Recombinant Erythropoetin and Adjuvant I.V. Iron Therapy of Anemic Patients With Lymphoproliferative Disorders Completed NCT00145652 Phase 3 Neo-Recormon and Venofer
8 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
9 Preparatory Aid to Improve Decision Making About Cancer Clinical Trials (PRE-ACT) Completed NCT00750009 Phase 3
10 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
11 The Use of Topical Baclofen, Amitriptyline HCI, and Ketamine (BAK) in a PLO Gel vs. Placebo for the Treatment of Chemotherapy Induced Peripheral Neuropathy: A Phase III Randomized Double-Blind Placebo Controlled Study Completed NCT00516503 Phase 3 baclofen/amitriptyline/ketamine gel
12 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
13 Predictive Value of the "Cytocapacity Test" Following a Single-dose of rHu-G-CSF in Patients With Lymphoproliferative Diseases and High-dose Therapy Completed NCT01085058 Phase 2, Phase 3 lenograstim
14 A Phase III, Randomized Study Of Two Different Dosing Schedules Of Erythropoetin In Anemic Patients With Cancer Completed NCT00058331 Phase 3
15 A Double-Blind Study Of Nutritional Intervention For The Treatment Of Cancer Cachexia Using Juven® Nutritional Supplement Completed NCT00053053 Phase 3
16 Randomized Study for Comparison of Reduced Intensity Conditioning Protocols Containing Either Thymoglobuline or Alemtuzumab in Patients Undergoing Allogeneic Transplant From Voluntary Unrelated Donors Completed NCT00354120 Phase 2, Phase 3 Alentuzumab;Globulina antilinfocitaria
17 A Randomized, Open-Label, Multicenter Study Of Darbepoetin Alfa Administered Once Every Two Weeks (Q2W) Compared With rHuEPO Administered Once Every Week (QW) For The Treatment Of Anemia In Subjects With Non-Myeloid Malignancies Receiving Multiple Chemotherapy Completed NCT00070382 Phase 3 darbepoetin alfa;epoetin alfa
18 Multicenter, Open-Label, Phase 3 Study of Tabelecleucel for Solid Organ or Allogeneic Hematopoietic Cell Transplant Subjects With Epstein-Barr Virus-Associated Post-Transplant Lymphoproliferative Disease After Failure of Rituximab or Rituximab and Chemotherapy Recruiting NCT03394365 Phase 3
19 Multicenter, Double-blind, Placebo-controlled, Randomized Withdrawal Trial With Tadekinig Alfa (r-hIL-18BP) in Patients With IL-18 Driven Monogenic Autoinflammatory Conditions: NLRC4 Mutation and XIAP Deficiency Recruiting NCT03113760 Phase 3 Tadekinig alfa
20 Open-label Extension Study With Tadekinig Alfa (r-hIL-18BP) to Monitor Safety and Tolerability in Patients With IL-18 Driven Monogenic Autoinflammatory Conditions: NLRC4 Mutation and XIAP Deficiency Recruiting NCT03512314 Phase 3 Tadekinig alfa
21 Phase III Multicenter Open-label Randomized Clinical Trial Comparing Everolimus and Low Dose Tacrolimus to Tacrolimus and Mycophenolate Mofetil at 6 mo Post-Transplant to Prevent Long-term Complications After Pediatric Heart Transplantation Active, not recruiting NCT03386539 Phase 3 Everolimus;Tacrolimus;Mycophenolate Mofetil
22 A Phase 3, Randomized, Double-Blind, Placebo-Controlled, Multicenter Study of Zilovertamab (an ROR1 Antibody) Plus Ibrutinib Versus Ibrutinib Plus Placebo in Subjects With Relapsed or Refractory Mantle Cell Lymphoma Not yet recruiting NCT05431179 Phase 3 Zilovertamab;Ibrutinib;Placebo
23 In-vivo T-cell Depletion and Hematopoietic Stem Cell Transplantation for Life-Threatening Immune Deficiencies and Histiocytic Disorders Terminated NCT00176826 Phase 2, Phase 3 Myeloablative conditioning regimen
24 A Non-randomized, Open-label, Phase II Study of Chidamide Combined With R-GDP in Treating Patients With Relapsed or Refractory Diffuse Large B-cell Lymphoma (DLBCL) Not Suitable for Transplantation Unknown status NCT03373019 Phase 2 Chidamide combined with R-GDP
25 Pilot (Phase I-II) Study of Valproic Acid (Depakote) for the Treatment of the Autoimmune Lymphoproliferative Syndrome (ALPS) Completed NCT00605657 Phase 1, Phase 2 Valproic Acid
26 Sirolimus for Patients With Chronic and/or Refractory Autoimmune Cytopenias: A Pilot Series Completed NCT00392951 Phase 1, Phase 2 sirolimus
27 A Pilot Study of the Combination of Retinoic Acid and Interferon-Alpha2a for the Treatment of Lymphoproliferative Disorders in Children With Immunodeficiency Syndromes Completed NCT00001438 Phase 2 all-trans-retinoic acid with IFN-alpha2a
28 Treatment of T-Large Granular Lymphocyte (T-LGL) Lymphoproliferative Disorders With Alemtuzumab (Campath) Completed NCT00345345 Phase 2
29 A Phase II Study of the Novel Proteasome Inhibitor Bortezomib in Combination With Rituximab, Cyclophosphamide and Prednisone in Patients With Relapsed/Refractory Indolent B-Cell Lymphoproliferative Disorders and Mantle Cell Lymphoma (MCL) Completed NCT00295932 Phase 1, Phase 2 bortezomib;cyclophosphamide;prednisone
30 Phase II Trial of Rituximab in Patients With B-Cell Lymphoproliferative Disorders Associated With Pharmacologic Immunosuppression Completed NCT00003716 Phase 2
31 A Multicenter Phase II Study Of BMS 247550 (Epothilone B Analogue) In Indolent Lymphoproliferative Disorders Completed NCT00052572 Phase 2 ixabepilone
32 Phase II Study of PS-341 in Low Grade Lymphoproliferative Disorders Completed NCT00023764 Phase 2 bortezomib
33 Open Label Multicenter Study of Eltrombopag for the Treatment of Immune ThrombocytoPenia (ITP) Secondary to Chronic Lymphoproliferative Disorders (LPDs) Completed NCT01610180 Phase 2 Eltrombopag Olamine
34 A Phase II Study of the Therapeutic Effects Of Epstein-Barr Virus Immune T-Lymphocytes Derived From a Normal HLA-Compatible Or Partially-Matched Third-Party Donor in the Treatment of EBV Lymphoproliferative Disorders and EBV-Associated Malignancies Completed NCT01498484 Phase 2
35 Allogeneic Hematopoietic Stem Cell Transplant for Patients With Immunologic or Histiocytic Disorders Using a Non-Myeloablative Preparative Regimen to Achieve Stable Mixed Chimerism Completed NCT00176865 Phase 2 Fludarabine;Melphalan;Anti-thymocyte globulin (ATG);Campath 1H;Cyclosporin A;Mycophenolate mofetil;Intravenous immunoglobulin (IVIG)
36 Phase I/II Study of Allogeneic Stem Cell Transplantation for Patients With Hematologic Malignancy, Using MHC Identical or Near Identical Donors and Sub-Myeloablative Conditioning With CAMPATH 1H (DIMSUM) Completed NCT00048412 Phase 1, Phase 2 FLUDARABINE;CAMPATH 1H;FK50
37 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
38 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
39 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
40 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
41 Risk-stratified Sequential Treatment of Post-transplant Lymphoproliferative Disease (PTLD) With 4 Courses of Rituximab SC Followed by 4 Courses of Rituximab SC, 4 Courses of Rituximab SC Combined With CHOP-21 or 6 Courses of Rituximab SC Combined With Alternating CHOP-21 and DHAOx: The PTLD-2 Trial Completed NCT02042391 Phase 2 Rituximab sc;Rituximab sc consolidation;Rituximab sc combined with CHOP chemotherapy;Rituximab sc combined with alternating chemotherapy with CHOP and DHAOx
42 Phase II Study Evaluating Busulfan and Fludarabine as Preparative Therapy in Adults With Hematopoietic Disorders Undergoing Matched Unrelated Donor Stem Cell Transplantation Completed NCT00516152 Phase 2 Busulfan/Fludarabine phosphate/Tacrolimus/Methotrexate/G-CSF
43 Phase II Trial of Everolimus (RAD001) in Relapsed/Refractory Lymphoma Completed NCT00436618 Phase 2 Everolimus
44 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
45 Bortezomib Plus Rituximab for EBV + Post Transplant Lymphoproliferative Disease (PTLD) Completed NCT01058239 Phase 2 bortezomib;rituximab
46 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
47 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
48 Treatment of Patients With Posttransplant Lymphoproliferative Disorder (PTLD) With a Sequential Treatment Consisting of Anti-CD20 Antibody Rituximab and CHOP+GCSF Chemotherapy (PTLD-1/3) Completed NCT00590447 Phase 2 rituximab monotherapy;sequential R-CHOP
49 Nonmyeloablative Hematopoietic Stem Cell Transplantation for Patients With High-Risk Hematologic Malignancies Using Related, HLA-Haploidentical Donors: A Phase II Trial of Combined Immunosuppression Before and After Transplantation Completed NCT00049504 Phase 2 cyclophosphamide;fludarabine phosphate;tacrolimus;mycophenolate mofetil
50 A Phase I/II Study: Zevalin Radioimmunotherapy for Patients With Post Transplant Lymphoproliferative Disease Following Solid Organ Transplantation Completed NCT00064246 Phase 1, Phase 2

Search NIH Clinical Center for Lymphoproliferative Syndrome

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Genetic Tests for Lymphoproliferative Syndrome

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Genetic tests related to Lymphoproliferative Syndrome:

# Genetic test Affiliating Genes
1 Lymphoproliferative Disorder 28
2 Lymphoproliferative Disorders, Susceptibility to 28
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Anatomical Context for Lymphoproliferative Syndrome

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Organs/tissues related to Lymphoproliferative Syndrome:

MalaCards : Bone Marrow, T Cells, Lymph Node, B Cells, Liver, Bone, Kidney
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Publications for Lymphoproliferative Syndrome

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Articles related to Lymphoproliferative Syndrome:

(show top 50) (show all 12127)
# Title Authors PMID Year
1
AT7519, a cyclin-dependent kinase inhibitor, exerts its effects by transcriptional inhibition in leukemia cell lines and patient samples. 53 62
20354122 2010
2
Bortezomib as a treatment option in patients with Waldenström macroglobulinemia. 53 62
20371443 2010
3
Acquired factor VIII inhibitors: case reports of paclitaxel and penicillin-induced entities. 53 62
19696661 2009
4
Abnormal B-cell activation associated with TALL-1 over-expression and SOCS-1 suppression during chronic hepatitis C virus infection. 53 62
19740379 2009
5
Diagnostic and prognostic evaluation of phenotypic markers TRAF1, MUM1, BCL2 and CD15 in cutaneous CD30-positive lymphoproliferative disorders. 53 62
19416236 2009
6
Prognostic irrelevance of HLA-G in B-cell chronic lymphocytic leukemia. 53 62
18241213 2009
7
Clinicopathologic features of CDK6 translocation-associated B-cell lymphoproliferative disorders. 53 62
19145199 2009
8
Constitutive JAK3 activation induces lymphoproliferative syndromes in murine bone marrow transplantation models. 53 62
19139084 2009
9
Phenotypic differences between mice deficient in XIAP and SAP, two factors targeted in X-linked lymphoproliferative syndrome (XLP). 53 62
19595300 2009
10
SLAM receptors and SAP influence lymphocyte interactions, development and function. 53 62
19079134 2009
11
Causes, etiology and diagnosis of acquired von Willebrand disease: a prospective diagnostic workup to establish the most effective therapeutic strategies. 53 62
19506364 2009
12
Update on treatment recommendations from the Fourth International Workshop on Waldenstrom's Macroglobulinemia. 53 62
19047284 2009
13
Epigenetic biomarkers for human cancer: the time is now. 53 62
18430583 2008
14
Gamma c-signaling cytokines induce a regulatory T cell phenotype in malignant CD4+ T lymphocytes. 53 62
18684941 2008
15
The antileukemia activity of a human anti-CD40 antagonist antibody, HCD122, on human chronic lymphocytic leukemia cells. 53 62
18497318 2008
16
MUM1 expression in cutaneous CD30+ lymphoproliferative disorders: a valuable tool for the distinction between lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. 53 62
18410414 2008
17
The expression of MUM1 in cutaneous T-cell lymphoproliferative disorders. 53 62
18234282 2008
18
Quantitative measurement of cyclin D1 mRNA, a potent diagnostic tool to separate mantle cell lymphoma from other B-cell lymphoproliferative disorders. 53 62
18303407 2008
19
The latent membrane protein 1 (LMP1) encoded by Epstein-Barr virus induces expression of the putative oncogene Bcl-3 through activation of the nuclear factor-kappaB. 53 62
17963943 2008
20
The incidence and survival of Waldenström's Macroglobulinaemia in South East England. 53 62
17416416 2008
21
Detection of bcl-2 Translocation in Patients with Chronic Hepatitis C and its Possible Relation to Antiviral Therapy: Preliminary Study. 53 62
19190694 2007
22
Differences in survivin location and Bcl-2 expression in CD30+ lymphoproliferative disorders of the skin compared with systemic anaplastic large cell lymphomas: an immunohistochemical study. 53 62
17484779 2007
23
A new sensitive enzyme-linked immunosorbent assay (ELISA) for Alemtuzumab determination: development, validation and application. 53 62
17624249 2007
24
c-Jun expression and activation are restricted to CD30+ lymphoproliferative disorders. 53 62
17325487 2007
25
Associations among Epstein-Barr virus subtypes, human leukocyte antigen class I alleles, and the development of posttransplantation lymphoproliferative disorder in bone marrow transplant recipients. 53 62
17278061 2007
26
Altered expression of the tetraspanin CD81 on B and T lymphocytes during HIV-1 infection. 53 62
17177963 2007
27
CD5 does not regulate the signaling triggered through BCR in B cells from a subset of B-CLL patients. 53 62
17325858 2007
28
CD32B, the human inhibitory Fc-gamma receptor IIB, as a target for monoclonal antibody therapy of B-cell lymphoma. 53 62
16757681 2006
29
Alternative and classical NF-kappa B signaling retain autoreactive B cells in the splenic marginal zone and result in lupus-like disease. 53 62
16973390 2006
30
B cell receptors in TCL1 transgenic mice resemble those of aggressive, treatment-resistant human chronic lymphocytic leukemia. 53 62
16864779 2006
31
A homozygous Fas ligand gene mutation in a patient causes a new type of autoimmune lymphoproliferative syndrome. 53 62
16627752 2006
32
Eosinophilic ulcer of the oral mucosa: another histological simulator of CD30+ lymphoproliferative disorders. 53 62
16882190 2006
33
IgVH genes mutation and usage, ZAP-70 and CD38 expression provide new insights on B-cell prolymphocytic leukemia (B-PLL). 53 62
16642047 2006
34
JAK2 V617F is a rare finding in de novo acute myeloid leukemia, but STAT3 activation is common and remains unexplained. 53 62
16598306 2006
35
The broad spectrum of autoimmune lymphoproliferative disease: molecular bases, clinical features and long-term follow-up in 31 patients. 53 62
16537120 2006
36
D cyclins in CD5+ B-cell lymphoproliferative disorders: cyclin D1 and cyclin D2 identify diagnostic groups and cyclin D1 correlates with ZAP-70 expression in chronic lymphocytic leukemia. 53 62
16393687 2006
37
Effect of rapamycin on mouse chronic lymphocytic leukemia and the development of nonhematopoietic malignancies in Emu-TCL1 transgenic mice. 53 62
16424025 2006
38
Spectrum of infection, risk and recommendations for prophylaxis and screening among patients with lymphoproliferative disorders treated with alemtuzumab*. 53 62
16371014 2006
39
Prognostic significance of CD40 expression in malignant lymphoma developing in rheumatoid arthritis. 53 62
16177927 2005
40
X-linked lymphoproliferative disease in an adult. 53 62
16105760 2005
41
CCR10 is expressed in cutaneous T-cell lymphoma. 53 62
15700309 2005
42
Frequency of bcl-2/IgH translocation in patients with non-Hodgkin's lymphoma and chronic hepatitis C virus infection. 53 62
15990705 2005
43
Polymorphism of the CD30 promoter microsatellite repressive element is associated with development of primary cutaneous lymphoproliferative disorders. 53 62
15894695 2005
44
Death receptor apoptosis signaling mediated by FADD in CD30-positive lymphoproliferative disorders involving the skin. 53 62
15767797 2005
45
Regulation of CD23 expression by Notch2 in B-cell chronic lymphocytic leukemia. 53 62
15621797 2005
46
The CD45 isoform B220 identifies select subsets of human B cells and B-cell lymphoproliferative disorders. 53 62
15712182 2005
47
Polyclonal proliferation of plasma cells associated with marked hypergammaglobulinemia in an elderly patient. 53 62
15717691 2005
48
Treatment options in Waldenstrom's macroglobulinemia. 53 62
15636690 2004
49
Chemokine receptor expression in B-cell lymphoproliferative disorders. 53 62
15621766 2004
50
Enhanced tumorigenesis in HTLV-1 tax-transgenic mice deficient in interferon-gamma. 53 62
15292059 2004
Sources

Variations for Lymphoproliferative Syndrome

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ClinVar genetic disease variations for Lymphoproliferative Syndrome:

5 (show all 21)
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 PLAA NM_001031689.3(PLAA):c.1487-1G>A SNV Likely Pathogenic
 598964 rs1426488816 GRCh37: 9:26913946-26913946
GRCh38: 9:26913948-26913948
2 XIAP NM_001167.4(XIAP):c.*5180dup DUP Conflicting Interpretations Of Pathogenicity
 367846 rs60841987 GRCh37: X:123046198-123046199
GRCh38: X:123912348-123912349
3 SH2D1A NM_002351.5(SH2D1A):c.*51_*52del DEL Uncertain Significance
 367874 rs769919066 GRCh37: X:123505292-123505293
GRCh38: X:124371442-124371443
4 XIAP NM_001167.4(XIAP):c.*5070del DEL Uncertain Significance
 367843 rs751242607 GRCh37: X:123046090-123046090
GRCh38: X:123912240-123912240
5 XIAP NM_001167.4(XIAP):c.*5047dup DUP Uncertain Significance
 367841 rs368295939 GRCh37: X:123046066-123046067
GRCh38: X:123912216-123912217
6 XIAP NM_001167.4(XIAP):c.*4307_*4309dup DUP Uncertain Significance
 367835 rs397713705 GRCh37: X:123045327-123045328
GRCh38: X:123911477-123911478
7 ITK NM_005546.4(ITK):c.*1682G>T SNV Uncertain Significance
 352496 rs538864150 GRCh37: 5:156681370-156681370
GRCh38: 5:157254360-157254360
8 ITK NM_005546.4(ITK):c.622TGG[1] (p.Trp209del) MICROSAT Uncertain Significance
 352465 rs757871566 GRCh37: 5:156649999-156650001
GRCh38: 5:157222989-157222991
9 XIAP NM_001167.4(XIAP):c.*1175ATTA[1] MICROSAT Likely Benign
 367803 rs747235601 GRCh37: X:123042205-123042208
GRCh38: X:123908355-123908358
10 XIAP NM_001167.4(XIAP):c.*5034_*5035insAAA INSERT Likely Benign
 367840 rs770819952 GRCh37: X:123046064-123046065
GRCh38: X:123912214-123912215
11 XIAP NM_001167.4(XIAP):c.*6564CTGA[2] MICROSAT Likely Benign
 367865 rs28382756 GRCh37: X:123047595-123047598
GRCh38: X:123913745-123913748
12 SH2D1A NM_002351.4(SH2D1A):c.*1773G>A SNV Likely Benign
 369633 rs190741204 GRCh37: X:123507014-123507014
GRCh38: X:124373164-124373164
13 SH2D1A NM_002351.5(SH2D1A):c.*203del DEL Benign
 367875 rs67202578 GRCh37: X:123505440-123505440
GRCh38: X:124371590-124371590
14 XIAP NM_001167.4(XIAP):c.*5068_*5069insT INSERT Benign
 367844 rs58114044 GRCh37: X:123046099-123046100
GRCh38: X:123912249-123912250
15 XIAP NM_001167.4(XIAP):c.*5519del DEL Benign
 367852 rs374013599 GRCh37: X:123046537-123046537
GRCh38: X:123912687-123912687
16 XIAP NM_001167.4(XIAP):c.*5505del DEL Benign
 367851 rs111508332 GRCh37: X:123046536-123046536
GRCh38: X:123912686-123912686
17 XIAP NM_001167.4(XIAP):c.*5180del DEL Benign
 367847 rs60841987 GRCh37: X:123046199-123046199
GRCh38: X:123912349-123912349
18 XIAP NM_001167.4(XIAP):c.*5047del DEL Benign
 367842 rs368295939 GRCh37: X:123046067-123046067
GRCh38: X:123912217-123912217
19 XIAP NM_001167.4(XIAP):c.*4309dup DUP Benign
 367834 rs397713705 GRCh37: X:123045327-123045328
GRCh38: X:123911477-123911478
20 XIAP NM_001167.4(XIAP):c.*3702dup DUP Benign
 367826 rs762141177 GRCh37: X:123044717-123044718
GRCh38: X:123910867-123910868
21 XIAP NM_001167.4(XIAP):c.*2296_*2300del DEL Benign
 367809 rs79291374 GRCh37: X:123043325-123043329
GRCh38: X:123909475-123909479
Sources

Expression for Lymphoproliferative Syndrome

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Search GEO for disease gene expression data for Lymphoproliferative Syndrome.
Sources

Pathways for Lymphoproliferative Syndrome

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Pathways related to Lymphoproliferative Syndrome according to GeneCards Suite gene sharing:

(show all 45)
# Super pathways Score Top Affiliating Genes
1
Innate Immune System 66
Show member pathways
 13.94 TNFRSF8 SH2D1A PTPN11 PRKCD NRAS ITK
2
ERK Signaling 64
Show member pathways
 13.78 CASP10 CASP8 CD27 FADD FAS FASLG
3
Signal Transduction 66
13.73 XIAP PTPN11 PRKCD NRAS FASLG FAS
4
MIF Mediated Glucocorticoid Regulation 64
Show member pathways
 13.59 TNFRSF8 PRKCD NRAS FASLG FAS CD27
5
Akt Signaling 64
Show member pathways
 13.35 CD27 FADD FAS FASLG ITK NRAS
6
Cytokine Signaling in Immune system 66
Show member pathways
 13.29 TNFRSF8 PTPN11 PRKCD NRAS INPP5D FASLG
7
PAK Pathway 64
Show member pathways
 13.18 CASP10 CASP8 CD27 FAS FASLG NRAS
8
p75 NTR receptor-mediated signalling 66
Show member pathways
 12.86 CASP10 CASP8 FADD FAS FASLG XIAP
9
CCR5 Pathway in Macrophages 64
Show member pathways
 12.81 PRKCD NRAS ITK FASLG FAS
10
Toll-like receptor signaling pathway 74
Show member pathways
 12.77 SLAMF1 NRAS FASLG FAS FADD CASP8
11
TNFR1 Pathway 64
Show member pathways
 12.76 XIAP PRKCD PRF1 FASLG FAS FADD
12
Programmed Cell Death 66
Show member pathways
 12.64 XIAP PRKCD FASLG FAS FADD CASP8
13
4-1BB Pathway 64
Show member pathways
 12.63 PRKCD NRAS FASLG FAS CD27
14
Overview of interferons-mediated signaling pathway 74
Show member pathways
 12.59 PTPN11 PRKCD NRAS FADD CASP8
15
Complement cascade 66
Show member pathways
 12.58 XIAP FASLG FAS CASP8 CASP10
16
DREAM Repression and Dynorphin Expression 64
Show member pathways
 12.58 PRF1 NRAS FASLG FAS FADD CASP8
17
Chks in Checkpoint Regulation 64
Show member pathways
 12.57 XIAP FASLG FAS FADD CASP8 CASP10
18
NF-kappaB Signaling 3
12.52 TNFRSF8 SH2D1A ITK INPP5D FASLG FAS
19
Development EGFR signaling pathway 22
Show member pathways
 12.48 CASP10 CASP8 NRAS PRKCD XIAP
20
TNF Superfamily - Human Ligand-Receptor Interactions and their Associated Functions 65
Show member pathways
 12.47 CASP10 CASP8 CD27 FADD FAS FASLG
21
Hepatocyte growth factor receptor signaling 74
Show member pathways
 12.41 FAS PRKCD PTPN11 XIAP
22
NF-kappaB Pathway 70
Show member pathways
 12.38 PRKCD NRAS CASP8 CASP10
23
Development FGFR signaling pathway 22
Show member pathways
 12.36 PTPN11 PRKCD NRAS INPP5D
24
MAPK signaling pathway 74
12.35 PRKCD NRAS FASLG FAS
25
Cell surface interactions at the vascular wall 66
Show member pathways
 12.19 PTPN11 NRAS INPP5D CD244
26
p53 Signaling 64
Show member pathways
 12.17 FASLG FAS CASP8 CASP10
27
Death Receptor Signaling 67
Show member pathways
 12.16 XIAP FASLG FAS FADD CD27 CASP8
28
RIPK1-mediated regulated necrosis 66
Show member pathways
 12.15 XIAP FASLG FAS FADD CASP8
29
Regulation by c-FLIP 66
Show member pathways
 12.1 FASLG FAS FADD CASP8 CASP10
30
Apoptosis and Autophagy 3
Show member pathways
 12.05 XIAP PTPN11 PRF1 FASLG FAS FADD
31
Direct p53 effectors 61
11.98 SH2D1A FAS CASP10
32
Hematopoietic Stem Cells and Lineage-specific Markers 65
11.98 SLAMF1 LY9 CD5 CD244
33
IL2 signaling events mediated by STAT5 61
Show member pathways
 11.95 PTPN11 PRF1 FASLG
34
Ceramide Pathway 64
Show member pathways
 11.92 PRKCD NRAS FADD CASP8 CASP10
35
Allograft rejection 74
11.86 PRF1 FASLG FAS CASP8
36
CCL18 signaling pathway 74
11.84 PRKCD NRAS CASP8
37
VEGF Pathway (Tocris) 70
Show member pathways
 11.77 PRKCD NRAS CASP8 CASP10
38
Head and neck squamous cell carcinoma 74
11.7 NRAS FADD CASP8
39
Apoptosis and survival FAS signaling cascades 22
Show member pathways
 11.53 XIAP PRF1 FASLG FAS FADD CASP8
40
Nonalcoholic fatty liver disease 74
11.48 FASLG FAS CASP8
41
IL-18 signaling pathway 74
11.48 PRKCD FASLG FAS CASP8
42
Transcription_Transcription factor Tubby signaling pathways 22
11.28 PRKCD NRAS CASP8
43
Host-pathogen interaction of human coronaviruses - apoptosis 74
11.08 FASLG FADD CASP8
44
CTL Mediated Apoptosis 64
10.93 PRF1 FASLG FAS FADD CASP8
45
Nanoparticle triggered regulated necrosis 74
10.76 FADD CASP8
Sources

GO Terms for Lymphoproliferative Syndrome

About this section

Cellular components related to Lymphoproliferative Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 external side of plasma membrane GO:0009897 10.1 CD244 CD27 CD5 FAS FASLG LY9
2 membrane raft GO:0045121 9.97 INPP5D FASLG FAS FADD CASP8
3 death-inducing signaling complex GO:0031264 9.63 FAS FADD CASP8
4 ripoptosome GO:0097342 9.43 FADD CASP8 CASP10
5 CD95 death-inducing signaling complex GO:0031265 9.23 FAS FADD CASP8 CASP10

Biological processes related to Lymphoproliferative Syndrome according to GeneCards Suite gene sharing:

(show all 14)
# Name GO ID Score Top Affiliating Genes
1 signal transduction GO:0007165 10.41 TNFRSF8 PTPN11 PRKCD PLAA NRAS ITK
2 positive regulation of apoptotic process GO:0043065 10.15 CASP8 FADD FAS FASLG INPP5D TNFRSF8
3 regulation of apoptotic process GO:0042981 10.08 XIAP FAS FADD CASP8 CASP10
4 cellular response to mechanical stimulus GO:0071260 10.06 CASP8 FADD FAS TNFRSF8
5 T cell activation GO:0042110 10.01 LY9 ITK CASP8
6 cellular defense response GO:0006968 10 SH2D1A PRF1 ITK
7 extrinsic apoptotic signaling pathway via death domain receptors GO:0008625 9.97 FASLG FADD CASP8
8 immunoglobulin mediated immune response GO:0016064 9.88 PRKCD INPP5D CD27
9 immune system process GO:0002376 9.87 SLAMF1 SH2D1A LY9 ITK INPP5D FADD
10 apoptotic signaling pathway GO:0097190 9.85 CASP10 CASP8 CD5 FADD FASLG
11 TRAIL-activated apoptotic signaling pathway GO:0036462 9.81 FADD CASP8
12 necroptotic signaling pathway GO:0097527 9.73 FASLG FAS FADD
13 extrinsic apoptotic signaling pathway GO:0097191 9.65 FASLG FAS FADD CD27 CASP8
14 apoptotic process GO:0006915 9.58 XIAP PRKCD PRF1 INPP5D FASLG FAS

Molecular functions related to Lymphoproliferative Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 tumor necrosis factor receptor binding GO:0005164 9.63 FASLG FADD CASP8
2 cysteine-type endopeptidase activity involved in apoptotic signaling pathway GO:0097199 9.56 CASP8 CASP10
3 death receptor binding GO:0005123 9.43 FASLG FADD CASP8
4 death effector domain binding GO:0035877 9.1 FADD CASP8 CASP10
Sources

Sources for Lymphoproliferative Syndrome

About this section
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
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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