[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"lymphohistiocytosis\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:lymphohistiocytosis":28},{"pageToken":4,"total":5,"offset":6,"count":5,"results":7},null,2,0,[8,47],{"id":9,"slug":10,"hasResults":11,"nctId":12,"briefTitle":13,"officialTitle":14,"acronym":15,"eligibilityCriteria":16,"healthyVolunteers":11,"sex":17,"minAge":4,"maxAge":18,"enrollmentInfo":19,"targetDuration":4,"studyType":22,"phases":23,"briefSummary":25,"conditions":26,"keywords":29,"overallStatus":34,"whyStopped":4,"lastUpdateSubmitDate":35,"lastUpdatePostDateStruct":36,"startDateStruct":39,"completionDateStruct":41,"leadSponsor":43,"locationsCount":46},"100650029","impact-of-ultra-fast-genetic-diagnosis-of-familial-lymphohistiocytosis-on-the-time-to-bone-marrow-transplantation-and-overall-survival-100650029",false,"NCT07741747","Impact of Ultra-fast Genetic Diagnosis of Familial Lymphohistiocytosis on the Time to Bone Marrow Transplantation and Overall Survival","Impact of Ultra-rapid Genetic Diagnosis of Primary Haemophagocytic Lymphohistiocytosis on the Time to Haematopoietic Stem Cell Transplantation","LongRead-HLH","Inclusion Criteria:\n\n* Children under 18 years old\n* Confirmed or suspected diagnosis of FHL or a related genetic syndrome predisposing to HLH (e.g. Griscelli Syndrome, Chédiak-Higashi Syndrome, XLP1, XLP2) or a family history of lymphohistiocytic activation syndrome\n* Presence of at least 5 of the 8 following criteria (diagnostic criteria according to the definition of the \"Histiocyte Society\" (1)):\n\n  1. Fever\n  2. Splenomegaly\n  3. Hypertriglyceridemia ≥ 3 mmol\u002Fl and\u002For hypofibrinogenemia≤ 1.5g\u002Fl\n  4. Hemophagocytosis found in a histological sample\n  5. Decreased or absent NK function (\\\u003C10% of the laboratory normal)\n  6. Ferritin ≥ 500μg\u002Fl\n  7. Soluble CD25 ≥ 2,400U\u002Fml or presence of activated T cells in phenotyping\n  8. Cytopenia (affecting at least two blood cell lines): Haemoglobin \\\u003C 9.0 g\u002Fdl, Platelets \\\u003C100 G\u002FL, Neutrophils \\\u003C1,0 G\u002FL\n* Patient benefiting from social security coverage\n* The legal guardian(s) who have signed the informed consent form\n\nExclusion Criteria:\n\n* Age ≥ 18 years\n* Solid tumor, leukemia, lymphoma\n* Subjects covered by articles L1121-5 to 1121-8 of the public health code (patients under guardianship or curatorship, patient deprived of liberty, pregnant or breadtfeeding woman)\n* Persons who do not understand the French language\n* Patient in the exclusion period of another research protocol at the time of signing the consent form","ALL","18 Years",{"count":20,"type":21},240,"ESTIMATED","INTERVENTIONAL",[24],"NA","Familial lymphohistiocytosis (FHL) is a group of rare genetic diseases (around fifteen cases per year in France). The defect in T lymphocyte cytotoxicity resulting from this disease is responsible for hemophagocytic lymphohistiocytosis (HLH). Promptly treatment of HLH is essential for prognosis. These diseases are fatal without a bone marrow transplant, with an overall 5-year survival rate of no more than 80% for FHL. The genetic or acquired nature of HLH is not easy to determine. An infectious trigger can be confounding when it occurs in an FHL. But above all, functional biological tests demonstrating a T lymphocyte cytotoxicity defects are difficult to interpret. Genetic diagnosis is therefore essential for confirming the primary nature of HLH, and for initiating targeted treatments (first stage: putting HLH into remission with chemotherapy or immunotherapy; second stage: bone marrow transplant). Genetic diagnosis of FHL is therefore a matter of emergency, and is currently based on targeted gene panel exploration (fragmentation sequencing) requiring 6 to 8 weeks. Recently, the development of third-generation sequencing (TGS) has revolutionized genomic medicine, enabling unitary sequencing in real time. As a result of this innovation, certain private molecular diagnostic specialties can now access this new emergency genomic medicine.\n\nAim: the main aim of this study is to demonstrate the feasibility of a national circuit for ultra-rapid genetic diagnosis of pediatric HLH revealing familial lymphohistiocytosis. The secondary objective is to evaluate the impact of this early genetic diagnosis on the delay to remission of HLH and the delay to transplantation.\n\nMethods: This prospective, multicenter study measures the time required for genetic diagnosis of FHL in pediatric HLH, using innovative TGS sequencing technology.\n\nPerspectives: Fast genomic diagnosis of FHL will considerably shorten the time to confirm the diagnosis, to obtain HLH remission and, finally, to reach transplantation faster.",[27,28],"Familial Lymphohistiocytosis","Lymphohistiocytosis",[28,30,31,32,33],"Rare disease","Fast-genomic","Precision medecin","Bone marrow transplant","NOT_YET_RECRUITING","2026-07-30",{"date":37,"type":38},"2026-08-03","ACTUAL",{"date":40,"type":21},"2026-10",{"date":42,"type":21},"2030-10",{"name":44,"class":45},"Assistance Publique Hopitaux De Marseille","OTHER",1,{"id":48,"slug":49,"hasResults":11,"nctId":50,"briefTitle":51,"officialTitle":52,"acronym":4,"eligibilityCriteria":53,"healthyVolunteers":11,"sex":17,"minAge":18,"maxAge":54,"enrollmentInfo":55,"targetDuration":4,"studyType":22,"phases":57,"briefSummary":60,"conditions":61,"keywords":4,"overallStatus":64,"whyStopped":4,"lastUpdateSubmitDate":65,"lastUpdatePostDateStruct":66,"startDateStruct":68,"completionDateStruct":70,"leadSponsor":72,"locationsCount":46},"100621370","phase-2-golidocitinib-combined-with-selinexor-for-caebvd-100621370","NCT07369739","Golidocitinib Combined With Selinexor for CAEBVD","Golidocitinib Combined With Selinexor for the Treatment of Chronic Active Epstein-Barr Virus Disease (CAEBVD): A Multicenter, Prospective, Single-arm Clinical Study","Inclusion Criteria:\n\n1. CAEBVD diagnosed in accordance with the Consensus on the Diagnosis and Treatment of Chronic Active Epstein-Barr Virus Disease (2025 Edition).\n2. Aged ≥ 18 years and ≤ 70 years, regardless of gender.\n3. Eastern Cooperative Oncology Group (ECOG) performance status score of 0 or 1.\n4. Before the initiation of the study, aspartate aminotransferase (AST) and alanine aminotransferase (ALT) ≤ 3 × upper limit of normal (ULN); total bilirubin ≤ 2 × ULN; serum creatinine ≤ 1.5 × ULN.\n5. Routine blood test: absolute neutrophil count ≥ 1 × 10#\u002FL; platelet count ≥ 50 × 10#\u002FL; hemoglobin ≥ 60 g\u002FL.\n6. Coagulation function test requirements: international normalized ratio (INR) ≤ 2.0; prothrombin time (PT) ≤ 1.5 × ULN.\n7. Women of childbearing potential must have a negative pregnancy test result, and be willing to take effective contraceptive measures during the trial period and for ≥ 12 months after the last dose; all male subjects must take contraceptive measures during the trial period and for ≥ 6 months after the last dose.\n8. Signed informed consent form.\n\nExclusion Criteria:\n\n1. Evidence of EBV-associated hematological diseases or malignancies, such as hemophagocytic lymphohistiocytosis, lymphomatoid granulomatosis, post-transplant lymphoproliferative disorder, non-Hodgkin's lymphoma, Burkitt lymphoma, nasopharyngeal carcinoma, and gastric cancer.\n2. Having received any of the following treatments: prior treatment with any JAK inhibitor; administration of any investigational drug within 12 weeks prior to the first dose of the study drug; concurrent enrollment in another clinical study.\n3. A history of other primary malignancies within 5 years prior to the first dose of the study drug, excluding locally curable malignancies that have received curative treatment (e.g., basal or squamous cell carcinoma of the skin, superficial bladder cancer, or carcinoma in situ of the prostate, cervix, or breast).\n4. A history of organ transplantation (e.g., liver transplantation, kidney transplantation).\n5. Planned hematopoietic stem cell transplantation during the study period.\n6. Active hepatitis B (defined as positive hepatitis B surface antigen \\[HBsAg\\] at screening, or a peripheral blood hepatitis B virus DNA titer \\> 1×10³ copies\u002FmL); active hepatitis C (defined as positive anti-hepatitis C virus antibody \\[HCV-Ab\\] and HCV-RNA at screening); positive serum HIV antigen or antibody; a history of syphilis.\n7. Having undergone major surgery within 4 weeks prior to the first dose, or anticipating the need for major surgery during the study period.\n8. Pregnant or lactating women.\n9. A history of severe mental illness or drug abuse.\n10. Uncontrolled infections (including pulmonary infection, intestinal infection); active major visceral hemorrhage (including gastrointestinal bleeding, alveolar hemorrhage, intracranial hemorrhage).\n11. Hypersensitivity to the components of the study drug, or a history of severe allergic diathesis.\n12. Patients who are unable to comply with the requirements during the trial and\u002For follow-up phase.","70 Years",{"count":56,"type":21},28,[58,59],"PHASE2","PHASE3","This study is a multicenter, prospective, single-arm clinical investigation, with patients with CAEBVD as the main research subjects, to evaluate the effectiveness of the combined treatment regimen of golidocitinib and selinexor.",[28,62,63],"EBV","CAEBV","RECRUITING","2026-01-17",{"date":67,"type":38},"2026-01-27",{"date":69,"type":38},"2026-01-01",{"date":71,"type":21},"2028-01-01",{"name":73,"class":45},"Beijing Friendship Hospital"]