Disease · MONDO_0010626
hyper-IgM syndrome type 1
6 sourcesdiseases-2026-09-18-0.1.0
Cite this record
ConductScience Library. hyper-IgM syndrome type 1. Diseases, record MONDO_0010626. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0010626
On this page
Disease
Names and IDs as Open Targets gives them. Its IDs in MeSH, Orphanet, the Disease Ontology and the NCI Thesaurus are those Open Targets states for it.
- Other names
HIGM1, Hyper IgM Syndromes, Hyper-IgM Immunodeficiency Syndrome, X-linked hyper-IgM syndrome, XHIGM, hyper-IgM syndrome due to CD40 ligand deficiency, hyper-IgM syndrome due to CD40L deficiency, hyper-IgM syndrome, X-linked, hyperimmunoglobulin M syndrome, hyperimmunoglobulin M syndrome (disorder), immunodeficiency, X-linked, with hyper-IgM, X-linked recessive
- Open Targets
Drugs6
The drugs Open Targets' clinical reports study for the disease, the highest stage first, then the most trial reports. The stage is Open Targets', the highest any of its sources reports for the pair, and each row names the sources that report it.
| Drug | ChEMBL ID | Type | Open Targets' stage | Stage reported by | Reports | Of them trials | Trials shown here | Source |
|---|---|---|---|---|---|---|---|---|
| ALEMTUZUMAB | CHEMBL1201587 | Antibody | Phase 2 | ClinicalTrials.gov | 2 | 2 | 2 | Drugs and targets |
| CD40 LIGAND | CHEMBL4297718 | Phase 2 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets | |
| FLUDARABINE | CHEMBL1568 | Small molecule | Phase 2 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
| KEYHOLE LIMPET HEMOCYANIN | CHEMBL5314988 | Phase 2 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets | |
| MELPHALAN | CHEMBL852 | Small molecule | Phase 2 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
| BUSULFAN | CHEMBL820 | Small molecule | Phase 1/2 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
Targets2
The targets ChEMBL's mechanism records give for the disease's drugs, through Open Targets, the most drugs first. A mechanism record says what a drug acts on, not that the target causes the disease.
| Target | Protein | Its drugs that act on it | Mechanisms | Highest stage of those drugs | Gene in Genes | Source |
|---|---|---|---|---|---|---|
| CD40 | Tumor necrosis factor receptor superfamily member 5 | 1 | Tumor necrosis factor receptor superfamily member 5 agonist | Phase 2 | https://conductscience.org/library/genes/958 | Drugs and targets |
| CD52 | CAMPATH-1 antigen | 1 | CAMPATH-1 antigen inhibitor | Phase 2 | https://conductscience.org/library/genes/1043 | Drugs and targets |
Trials3
Trials an Open Targets clinical report links to the disease, posted results first, then the largest. A trial is shown when the release's checks confirm its link to the disease and Drugs and targets shows its link to one of the disease's drugs.
| Trial | Title | Phase | Participants | Results first posted | Conditions | Drugs | Linked by | Source |
|---|---|---|---|---|---|---|---|---|
| NCT01998633 | Reduced Intensity Conditioning for Hemophagocytic Syndromes or Selected Primary Immune Deficiencies (BMT CTN 1204) | Phase 2 | 47 | 2018-06-01 | Hemophagocytic Lymphohistiocytosis, Chronic Active Epstein-Barr Virus Infection, Chronic Granulomatous Disease, HIGM-1, Leukocyte Adhesion Deficiency, IPEX | ALEMTUZUMAB, FLUDARABINE, MELPHALAN | Its record names the disease | ClinicalTrials.gov |
| NCT00001145 | Study of Immune Responses and Safety of Recombinant Human CD40 Ligand in Patients With X-Linked Hyper-IgM Syndrome | Phase 2 | 5 | Immunoproliferative Disorder | CD40 LIGAND, KEYHOLE LIMPET HEMOCYANIN | Its record names the disease | ClinicalTrials.gov | |
| NCT06959771 | Base Editing Hematopoietic Stem Cell and T Cell Gene Therapy for CD40L-HyperIgM Syndrome: Single Patient Study | Phase 1/2 | 1 | CD40L-HyperIgM Syndrome | ALEMTUZUMAB, BUSULFAN | Its record names the disease | ClinicalTrials.gov |
Comparisons in its trials0
Comparisons Trial comparisons calculated in the trials shown here, most participants first. A comparison in one of these trials does not always compare a treatment for the disease, so read the two groups.
None in this release.
Papers from its trials1
The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.
| PMID | Listed as | Trials listing it |
|---|---|---|
| 37042921 | DERIVED | 1 |
Source PubMed
Data dates
The date of each source this release read.
Sources 6
- ClinicalTrials.gov's posted results, through CTTI's AACTFreely available. Acknowledgement requested
- ChEMBL, through Open TargetsCC BY-SA 3.0
- ClinicalTrials.govNo license stated
- NLM's MeSHNLM's terms
- Open TargetsCC0 1.0
- PubMedNLM's terms
Each source's terms
- ClinicalTrials.gov's posted results, through CTTI's AACTClinicalTrials.gov data via Aggregate Analysis of ClinicalTrials.gov (AACT), CTTI. CTTI makes its materials freely available and requests acknowledgement. https://aact.ctti-clinicaltrials.org/documentation/299
- ChEMBL, through Open TargetsCC BY-SA 3.0, for the parts from ChEMBL. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
- ClinicalTrials.govnone stated
- NLM's MeSHNLM's
- Open TargetsCC0 1.0. Open Targets asks that users cite its latest paper: Buniello et al. 2025, Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery, Nucleic Acids Research 53(D1):D1467, PMID 39657122. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
- PubMedNLM's terms
ClinicalTrials.gov's data carry an international copyright outside the United States: https://clinicaltrials.gov/about-site/terms-conditions.
For software
Read this disease from software.
The record and each of its lists are JSON, twenty rows a page. No key.
GET https://conductscience.org/api/diseases/v1/MONDO_0010626
GET https://conductscience.org/api/diseases/v1/MONDO_0010626/drugs